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Seminar on
CHILD-WITH ONCOLOGICAL DISORDERS
Presented by
Mr. Abhijit Bhoyar
Anatomy and physiology
Function of Blood
 It acts as a transport system
 Conveys oxygen to the tissues
 Phagocytes action
 Distributes proteins needed for
tissue formation
 Produce hormones and enzyme
to organs.
LEUKEMIA
 Introduction:-Cancer account for the major cause
of death in India children; next only to infection and
malnutrition. Leukaemia is the most common form of
childhood malignancy.
Leukaemia
Definition
 Leukaemia is group of malignant disease of abnormal
proliferation and maturation of bone marrow which interfere
with the production of normal RBC, WBC and platelet.
 It is mostly characterised by persistent and uncontrolled
production of immature and abnormal white blood cells
(WBCs).
Incidence
 The annual incidence is 3-4 cases per 100,000
children globally.
 It occurs frequently in boys than in girls after age 1
year and peak onset is between 2-6 years of age.
 About 95-98% of childhood leukaemia are acute type.
Etiology
 Congenital or hereditary
 Environmental factors
 Drugs and chemicals
 Viral pathogenesis
 Chromosomal abnormalities or aberrations.
Classification of Leukemia
• Acute lymphocytes
leukemia(ALL)
• Acute non-lymphocyte leukemia
(ANLL) or Acute myelocytic
leukemia.
ACUTE
LEUKEMIA
• Chronic myeloid leukemia (CML)
• Chronic lymphocyte leukemia
(CLL)
CHRONIC
LEUKEMIA
1
2
Acute lymphocytes leukaemia
(ALL)
 It is the primary disorder of
the bone marrow in which
the normal bone marrow
elements are replaced by
immature lymphoblasts
from the lymphoid stem
cell.
FAB classification of acute lymphoblastic leukemia
Cytologic features L1 (80-85%) L2(15%) L3(1-2%)
Cell size Small cells
predominate,homogenous
Large heteroneous
in size
Large,
homogenous
Amount of
cytoplasm
Scanty Variable;often
moderately
abundant
Moderately
abundant
Nucleoli Small, incospicuous One or more, often
large
One or more,
prominent
Nuclear chromatin Homogenous Variable,
hetrogenous
Stippled,
homogenous
Nuclear shape Regular, occasional clefts Irregular
clefts,indentation
Regular, oval to
round
Cytoplasmic
basophilia
Variable Variable Intensely
basophlic
Cytoplasmic Variable Variable Prominent
2. Acute non-lymphocyte
leukemia (ANLL)-
 In this there is progressive proliferation of immature
monocytes and myelocytes from the bone marrow that
invade the blood and other tissues.
 15-20% of childhood leukemias.
Blood Smear of a
patient with Leukemia
A : Picture of bone marrow smear (control); Normal granulocytes and erythroblasts
are evident.
B : Acute lymphoid leukemia (ALL); There is a marked proliferation of small
lymphoblasts.
C : Acute myeloid leukemia (AML); There is a marked proliferation of large
myeloblasts.
D : Chronic myeloid leukemia (CML); There is a marked proliferation of granulocytes
at various stages of maturation.
A
D
C
B
1. Chronic myeloid leukemia (CML)
 Chronic myelocytic leukemia (CML) it arises from the
mutation in the myeloid stem cell
 It is rare in children i.e. 2-3% of all leukemias.
 It presents in child as adult and juvenile type. Juvenile type
occurs below 5 years of age.
Pathopysiology
Due to any etiological factors
Growth of abnormal fragile WBC in bone marrow spleen and lymph nodes
Normal bone is replaced by leukemic cells
RBC and Platelet formation decreased Leukemic cells infiltrated to
spleen and liver, bone
Anemia , bleeding petechiae Hepatospleenomegaly and lymphgland
enlargement , joint and bone pain.
Clinical Manifestation
 Fever
 Pallor and fatigue
 Anorexia,
 Weight loss
 Haemorrhage
 Petechiae
 Bone and joint pain
Cont..
 Infection
 Anemia
 Hepatosleenomegaly
 Thrombocytopenia
 Malaise and weakness
 Hematemesis ,
 Malena,
 Hematuria
 Abdominal mass.
Diagnostic Evaluation
 Blood count
 Bone marrow study
 Chest radiography
 CSF Study
 Blood chemistry
 Coagulation profile
CYTOGENIC ANALYSIS
Management
CHEMOTHERAPY
1) Remission induction phase
2) Maintenance or continuation therapy
3) Intensification or consolidation
4) CNS prophylactic therapy
1) Remission induction phase
 Immediately after confirming diagnosis, induction
therapy is began and lasts for 4-6 weeks.
 Drugs used are corticosteroids (prednisolone or
dexamethasone), vincristine and L- asparginase with
or without doxorubicin.
 These drugs give 95% remisson.
2) Maintenance or continuation
therapy
 It is to maintain the disease control and prevent there
relapse.
 It is to preserve remission and further reduce number of
leukemic cells.
 Drugs include daily dose of oral 6- mercaptopurine and
weekly dose of methotrexate and intermitten pulses of
vincristine and steroids which are standard in most
treatment regimen.
3) Intensification or consolidation
 It is used to further decrease the
number of leukemic cells in Childs body.
 The drug regimen include L-Asprginase
methotreaxate with vincristine,
cytarabine.
 It consist of pulses of these agents
given periodically during the 1st 6months
of treatment.
 It is given every 4 weeks.
4) CNS prophylactic therapy
 Children receive triple intrathecal
therapy of methotrexate,
 cytarabine and hydrocortisone
and is used during induction,
intensification and maintenance
therapy i.e. once a week during
induction and then every 8 weeks
for 2years.
Surgical Management
 Bone Marrow transplantation
Nursing Management
 Assessment
 Psychosocial care
Nursing Diagnosis
1.Risk of infection and haemorrhage related to bone marrow
suppression caused by chemotherapy and disease.
2. Disturbed body image related to alopecia associated with
chemotherapy.
3. Activity intolerance related to fatigue that results from the
disease and treatment.
4. Imbalanced nutrition less than body requirement related to
anaemia, anorexia, vomiting and mucosal ulceration
secondary to chemotherapy or radiation.
5. Anxiety of parents related to hospitalization and diagnosis.
Complication
 Bleeding
 Heart block
 Infection (GI, lungs, skin)
 CNS and skeletal involment
Prognosis
 Overall cure rate is 65-75%.
 Prognosis after transplantation varies with the
leukaemia.
 Higher WBC count initially has worse prognosis.
LYMPHOMA
 Lymphomas are the third most common malignancy
in children and adolescents, after leukemia and brain
tumors
 About 60% are non-Hodgkin’s lymphoma and 40%
are Hodgkin’s disease.
 Lymphomas are uncommon below the age of 5 years
and the incidence increases with age
Lymphoma is cancer that begins in infection-fighting cells of the immune
system, called lymphocytes. These cells are in the lymph nodes, spleen,
thymus, bone marrow, and other parts of the body. When you
have lymphoma, lymphocytes change and grow out of control.
Hodgkin’s disease
Definition
 Hodgkin’s lymphoma is
characterised by progressive
enlargement of lymph nodes.
 The disease is considered
unicentric in origin and has a
predictable pattern of spread
by extension to contiguous
nodes.
Incidence
 Hodgkin’s disease occur in 5 to 7 per 1,00,000
population.
 The incidence is highest in late childhood and early
adulthood (15-35 years).
 The sex ratio progresses from male preponderance of
10:1 under the age of 7 years falling to 1.1:1 after the
age of 12 years.
Etiology
 Ethnic groups and association with human
leukocyte antigen.
 Inherited susceptibility plays an important role in
the pathogenesis.
 Environmental factors
 Higher incidence in twins
Pathology
Hodgkin Lymphoma divided as
1. Nodular Lymphocyte-
Predominant Hodgkin Lymphoma
( NLPHL)
2. Classical Hodgkin Lymphoma
Nodular Lymphocyte-Predominant
Hodgkin Lymphoma ( NLPHL)
 Characterized by large cells with
multibed nuclei, referred to as
popcorn cells.
 NLPHL is most common in males
younger than 10 years.
Classical Hodgkin Lymphoma
 The hallmark of classic Hodgkin Lymphoma is
the R-S cell.
 This is a binucleated or multinucleated giant
cell that is often characterised by a bilobed
nucleus, with two large nucleoli, giving an
owl’s eye appearance to the cells.
 The classical subtype are defined according to
the number of Reed- Sternberg (R-S) cells,
characteristics of the inflammatory milieu, and
the presence or absence of fibrosis .
Staging
 A- No symptoms
 B- Fever, night sweats, or weight loss of more than
10% of body weight in the previous 6 months.
 X- Bulky disease (greater than 10 cm in maximum
dimension; greater than 1/3rd of the internal transverse
diameter of the thorax at the level T5/T6.
Anatomic definition of
lymph node regions used
for staging purposes
Ann Arbor staging classification for Hodgkin lymphoma
Stage Definition
I Involment of single lymph node region (I) or of a single extra lymphatic organ
or site (IE) by direct extension
II Involment of two or more lymph node regions on the same side of the
diaphragm (II) or localized involment of an extralyphatic organ or site and one
or more lymph node regions on the same side of the diaphragm (IIE)
III Involvement of lymph node regions on both sides of the diaphragm (III),
which may be accompanied by involvement of the spleen (IIIS), or by
localized involment of an extralymphatic organ or site (IIIE) or both III (E+S)
IV Diffuse or disseminated (multifocal) involvement of one or more
extralymnphatic organ or tissues with or without associated lymph node
involvement.
Stage Definition
Systemic
Symptoms
Each stage is subdivided into A and B subcategories, B for those with
defined systemic symptoms and A for those without any.
The B designation is given to patients with:
(i) unexplained loss of >10% of body weight in 6 months before
diagnosis
(ii) unexplained fever with temperature above 380c for 3 consecutive
days
(iii) drenching night sweats. Pruritis alone does not qualify for B
classification, nor does a short febrile illness associated with an
infection.
Clinical manifestation
 Painless enlargement of lymph nodes
 Firm, nontender, moveable nodes in the
supraclavicular or cervical area.
 In children the sentinel node located near
the left clavical
 Enlargement of axillary and inguinal lymph
nodes.
How do they present?
Other
 Mediastinal lymphadenopathy
 Enlarged retroperitoneal nodes
 Systemic symptoms include low-grade or intermittent
fever (Pel-Ebstein disease)
 Anorexia
 Nausea
 Weight Loss
 Night Sweats
 Pruritus
Diagnostic Evaluation
 History and Physical
examination
 Complete blood count
 Liver and renal function tests,
serum LDH, serum albumin
 Lymph node biopsy
 Bone marrow aspiration and
biopsy
Cont..
 Chest X-ray,
 CT scan of neck, chest
and abdomen and pelvis
 Positron emission
tomography (of the PET)
scan whole body
Treatments
 Chemotherapy
 Radiation therapy
Prognostic factors=70-90% patients with
Hodgkin lymphoma are cured.
CHEMOTHERAPY
Cont..
 Early stage (I,II) Hodgkin lymphoma
are given combination chemotherapy
(e.g. 2-4 cycles of ABVD comprising
adriamycin, bleomycin, vinblastine,
dacarbazine) with or without,
radiotherapy.
Cont..
 Advanced stage of Hodgkin lymphoma are given 4-6
cycles of chemotherapy(e.g. ABVD).
 Other combination chemotherapy include 6-8 cycles of
MOPP ( nitrogen mustard, vincristine, procarbazine
and prednisolone)
 COPP regime in which nitrogen mustard is replaced of
ABVD and COPP ( 4 cycles of each).
Nursing diagnosis
 Impaired tissue integrity related to high-dose
radiation therapy.
 Impaired oral mucous membrane related to
high-dose radiation therapy.
NON-HODGKIN’S LYMPHOMA
 Definition
Non- Hodgkin's lymphoma are a group of
malignancies of lymphoid tissue arising from T or B
lymphocytes or their precursors; include both indolent and
aggressive.
Epidemiology
 Male to female ratio of 3:1.
 In equatorial Africa, 50 % of all cancers are
lymphomas (Burkitt lymphoma being predominant).
 In India, lymphoblastic lymphoma or more common.
 Infection with malaria and EB virus are considered
risk factors for Burkitt lymphoma.
Staging system for Non- Hodgkin lymphoma
Stage I A single tumor or nodal area is involved, excluding the abdomen and
mediastinum.
Stage II Disease extent limited to a single tumor with regional node involvement,
2 or more tumors or nodal areas involved on one side of the diaphragm,
or a primary gastrointestinal tract tumour (completely resected) with or
without regional node involvement.
Stage III Tumours or involved lymph node areas; occur on both sides of the
diaphragm. Also includes any primary intrathoracic (Mediastinal, pleural
or thymic0 Disease, extensive primary intra-abdominal disease, or any
paraspinal or epidural tumors.
Stage IV Bone marrow and/or central nervous system disease, regardless of other
sites of involvement.
Etiology
 Immune System
 Other Risk Factors
 Male Gender
 White Ethnicity
 History Of Helicobacter
Gastritis
 History Of Hodgkin’s
Lymphoma
 History Of Radiation
Therapy
 Diet High In Meats And Fat
 Exposure To Certain
Pesticides.
Clinical manifestation
 Extranodal disease involving the mediastrnium, abdomen or
head and neck region.
 Intrathoracic NHL, most often T-cell lymphoma
 Cough
 Dyspnoea
 Dysphagia
 Chest pain or the superior vena cava syndrome.
 Pleural and pericardianl effusion.
Cont..
 Cervical Lymphadenopathy
 Abdominal Pain
 Ascities
 Palpable Abdominal Mass
 Intestinal Obstruction
 Intussusceptions(typical B-
cell Disease)
 Cranial Nerve Palsy
 Bone Involvement
 Jaw Swelling (Burkitt
Lymphoma) And
Pancytopenia Due To Bone
Marrow Involvement May
Occur.
Diagnostic Evaluation
 History and physical examination
 Complete blood count, peripheral
smear
 Renal function, uric acid, liver function,
serum albumin, lactate dehydrogenase
 Examination of cerebrospinal,
Peritoneal, pericardial or pleural fluid-
cytomorphology and
immuophenotyping
Cont..
 Surgical biopsy
 Chest radiograph or CT scan of the chest
 Ultrasonography /CT- scan of the abdomen
with contrast
 Bone marrow aspiration and biopsy
 Plain radiogram/ Ct- scan of other affected
sites
 PET scan of whole body (optional)
Management
 Chemotherapy and supportive care.
 Surgery
 Radiotherapy
e.g. superior vena cava syndrome or spinal cord
compression due to paraspinal disease.
 Chemotherapeutic regimens
 The regimens for lymphoblastic lymphoma are usually
based on protocols for ALL.
Cont…
 Most successful protocols are the German BFM (Burlin,
Frankurt, Munster)protocol and a modified version of LS A2 L2
protocol.
 Cranial irradiation or prophylactic intrathecal chemotherapy.
 90% patients with limited disease and 75-85% in patients with
bone marrow disease.
 The use of antiCD20 monoclonal antibodies
Cont..
 Most protocols consisits of short duration (6 months),
intensive alkylating agent therapy (cyclophosphamde or
ifosphamie) with high dose methotrexate, vincristine,
anthracyclines, etoposide and cytarabine;
 CNS prophylaxis is provided with intrathecal
chemotheraphy.
STEM CELLS
Nursing management
Nursing diagnosis
 Risk for infection related to altered immune
response because of lymphoma and leukopenia
caused by chemotherapy or radiation therapy
WILM’S TUMOR
Wilms' tumor is a
rare
kidney cancer that
primarily affects
children. Also
known as
nephroblastoma, it's
the most
common cancer of
the kidneys in
children.
Definition-
 Wilm’s tumor is a
malignant renal tumor
and is the most common
renal neoplasm in
children.
Incidence
 The incidence is 7.6 cases per million for
children younger than age 15% of cases
occur before the child is age 5.
 Most commonly a unilateral disease, but
in 5% to 10%, both kidneys are involved.
Causes
 Wilm;s tumor is not know.
 Genetic inheritance
Pathopysiology-
Due to etiology
Develop in the renal parenchyma in either the
central or polar location.
Entire renal parenchyma may appear replaced
by tumor.
The majority of the tumor present as a single,
expanding mass surrounded by a pseudo
capsule of connective tissue that appears to
separate kidney and tumor .
Cont..
The tumor is encapsulated, the membrane may
be very thin and easily torn.
Because of their rapid growth, the lesions are
often vascular, soft, gelatinous, and necrotic in
the centre
Lesions are composed of mesenchymal and
epithelial elements in various ratios and various
stages of maturity.
Some tumor are composed mainly of sheets of
anaplastic cells with little evidence of
differentiation.
Clinical manifestation-
 A firm, nontender upper quadrant abdominal
mass
 Abdominal pain,
 Less common are hypertension, fever, hematuria,
and anemia
Associated anomalies
a) Hemihypertrophy
b) Aniridia (without the iris)
c) Genito urinary anomalies
d) Over growth syndrome (i.e. beck with wiedemann
syndrome)
ABDOMINAL MASS
Diagnostic evaluation
 Abdominal ultrasound
 Radiography
 Complete blood count
(CBC) and peripheral
smear
 Urinalysis
 Blood chemistry
 Urinary VMA and HVA
 MRI or CT scan
 Ultrasonography
Staging of wilm’s tumor
 Stage I-
 Tumor is limited to the kidney and is
completely resected.
 Surface of the renal capsule is intact.
 Tumor is not ruptured before or during
removal, and no residual tumor is
apparent beyond the margins of
resection.
Stage II
 Tumor extend
beyond the kidney
but is completely
excised.
Stage III
 Residual non haematogenous tumor
confined to the abdomen.
Stage IV
 Haematogenous metastasis with deposits
beyond stage III (i.e. lung, liver, bone and
brain)
Stage V
 Bilateral renal involment at diagnosis
Staging of wilm’s tumor
Management
 Histological findings clinical staging and
metastasis.
 Stage I and II wilm’s tumor usually managed
with nephrectomy and chemotherapy for 18
weeks.
 Stage III, IV and V tumours are treated with
nephrectomy, abdominal radiotherapy and
chemotherapy for 24 weeks.
 Radiotherapy
Therapeutic management
 Surgical management
 Radiation therapy
 Chemotherapy
Nursing Management-
Goal include
 Assessing for the presence of wilm’s
tumor
 Emotional support
 Diagnostic procedure and treatment
management
 Planning for discharge.
Nursing Diagnosis-
 Ineffective thermoregulation related to infection
 Imbalanced nutritional status less than body
requirement related to family nutritional pattern
and secondary to disease condition.
 Risk of infection related to the disease condition
and secondary to immunity.
 Disturbed sleeping pattern due to hospitalization
and secondary to disease condition.
Complication
 Metastasis to lung , lymph nodes, liver, bone and
brain.
 Complications from radiation therapy include-
 bowel obstruction
 hepatic damage
 nephritis
 sterility in girls and interstitial pneumonia.
NEUROBLASTOMA
 Neuroblastoma is the
most common
extracranial solid
tumor in children and
the most common
tumor in infancy.
Neuroblastoma most
commonly occurs in:-
 Either one of the two adrenal glands
situated in the abdomen (tummy)
 Nerve tissue that runs alongside the
spinal cord, in the neck, chest,
abdomen or pelvis.
Neuroblastoma is a cancer of
specialised nerve cells called
neural crest cells. These cells
are involved in the development
of the nervous system and other
Incidence
 Fewer than 100 children in the UK are diagnosed
each year.
 Most children who get this cancer are younger than
five years old.
 Neuroblastoma is the second most common solid
tumour in childhood, and it makes up 8% of the total
number of children's cancers
Epidemiology
 Neuroblastoma is predominantly a tumor of
early childhood, with two thirds of the cases
presenting in children younger than 5 years.
It accounts for 7-10% of all childhood
cancers.
Causes
 Congenital
 Unknown
Sign and symptoms
 First symptoms are vague, such as tiredness, loss
of appetite and pain in the bones.
 More specific symptoms will depend on where the
neuroblastoma starts:-
 Tumour is in the abdomen----------
 Chest area---------------
 Neck-------------
Figure 2: A) Infant with a large, distended abdomen and a right-
sided abdomen mass. B) Infant with stage 4-S neuroblastoma with
multiple skin nodules, sometimes called the blueberry muffin
syndrome. C) This skin tumor on the scalp is found in another
infant with stage 4-S neuroblastoma.
CONT…..
 Occasionally, there are deposits of neuroblastoma in the
skin that appear as small, blue-coloured lumps.
 Tumour is pressing on the spinal cord, children may have
weakness in the legs and walk unsteadily. urine.
 Very rarely, children may have jerky eye and muscle
movements, and general unsteadiness associated with
the neuroblastoma.
 High blood pressure.
Diagnostic criteria
 General investigation
 Biopsy
 Blood and bone marrow tests
 X-rays
 CT or MRI scans,
CONT…
 Vanillylmandelic acid (VMA) or
homovanillic acid (HVA) tests
 MIBG scans (metaiodobenzyl
guanidine)
 Biopsy
Types of neuroblastoma
Staging system for neuroblastoma
 Stage 1
The cancer is contained
within one area of the body
(localised) and there's no
evidence of it having
spread.
CONT………
 Stage 2A
The cancer is localised and has not
begun to spread, but it cannot be completely
removed by surgery.
 Stage 2B
The cancer is localised and has begun to
spread into nearby lymph nodes.
 Stage 3
The cancer has spread into surrounding
organs and structures, but has not spread to distant
areas of the body.
CONT…….
 Stage 4
The cancer has spread to
distant lymph nodes, bone, bone marrow,
the liver, the skin or other organs.
 Stage 4S (also called special
neuroblastoma)
This is found in children under
one year old. The cancer is localised (as
in stage 1, 2A or 2B) but has begun to
spread to the liver, skin or bone marrow.
OTHER STAGES
 Stage L1
The tumour is localised and has not
spread into important areas (vital structures)
nearby. It can be removed by surgery.
 Stage L2
The tumour is localised but has 'image-
defined risk factors' and can't be safely
removed by surgery.
CONT….
 Stage M
The tumour has spread to other
parts of the body.
 Stage MS
The tumour has spread to the
skin, liver and/or the bone marrow in
children younger than 18 months old.
CONT…
 If the cancer has spread to distant parts of
the body, this is known as secondary or
metastatic cancer.
 If the cancer comes back after initial
treatment, this is known as recurrent or
relapsed cancer.
Management
 Surgery
 Chemotherapy
 High-dose chemotherapy with stem
cell support
 Monoclonal antibody treatment
 Radiotherapy
Monoclonal antibody treatment
Side effect of treatment
 Feeling sick (nausea) and being sick
(vomiting)
 Diarrhoea
 Hair loss
 Increased risk of infection
 Bruising and bleeding
 Tiredness
Late side effect
 Change in the way the heart and kidneys
work
 Hearing problems
 Fertility problems
 A possible reduction in bone growth
 And a slightly increased risk of developing
another cancer in later life.
Complication
 Metastasis to the liver, soft tissue, bones,
lymph nodes, bone marrow and skin.
 Neurologic deficits due to nerve
compression.
NURSING MANAGEMENT
 Nursing Assessment
Nursing Diagnoses
 Anxiety of parents related to learning of diagnosis.
 Fear of child related to diagnostic procedures and surgery or biopsy.
 Activity intolerance related to fatigue from tumor growth and bone
marrow suppression.
 Constipation or bowel and bladder incontinence related to pressure of
tumor.
 Risk for infection related to bone marrow suppression from chemotherapy
and radiation.
 Acute pain related to tumor, surgery or progression of disease.
 Disturbed body image related to hair loss.
 Incidence
 Approximately 350 new cases are reported per year in the united state.
 Rhabdomyosarcoma accounts for 3.5% of all malignant disease in children
younger than age 14. Most common are the head and neck, genitourinary
(GU) tract, and extremities.
Rhabdomyosarcoma
A Rhabdomyosarcoma,
commonly referred to as RMS, is
a type of cancer, specifically
a sarcoma (cancer of connective
tissues), in which the cancer cells
are thought to arise from skeletal
muscle progenitors.
Etiology
 Unknown
 Certain genetic and environmental factors
 Embryonal and alveolar.
 Tumor spreads either by local extension or by
metastasis via the venous and lymphatic
system.
 The lung is the most common site of
metastasis.
Tumor staging is based on the
extent of the disease:-
 Stage/group I: localized tumor completely resected.
 Stage/ group II: local tumor resected, microscopic
residual disease.
 Stage/group III: localized disease, with gross
residual disease after resection.
 Stage/group IV: metastatic disease at diagnosis.
Clinical Manifestations
 Asymptomatic lump
 Orbit - ptosis, ocular
paralysis,exophthalmaos.
 Nasopharynx- Epistaxis, pain,
dysphagia, nasal voice, airway
obstruction.
 Sinuses – swelling, pain , discharge,
sinusitis..
 Middle ear- pain, chronic otitis, facial
nerve palsy
 Neck- hoarseness, dysphagia
 Truncal, extremities, testicular areas-
enlarging soft tissue masses
 Prostate, bladder – urinary tract
symptoms
 Retroperitoneal tumours- GI and
urinary obstruction, weakness,
paresthesia, pain
 Vaginal- abnormal vaginal bleeding or
mass
Diagnostic evaluation
 Open biopsy of the primary
tumor- definitive diagnostic
procedure
 CT scan of the chest node
primary lesion
 MRI
 Bone marrow aspiration and
biopsy
 Bone scan or skeletal survey
 Ultrasound
 Chest X-ray
 CBC, liver and renal function
tests, electrolytes, serum calcium
and phosphorus, uric acid
 Monoclonal antibody assays
 Urinalysis
 Lumbar puncture
Management
1. Surgery- to biopsy the lesion, determine the stage of the disease, and completely
remove or reduce the primary tumor.
 Increasingly, chemotherapy and radiation therapy are used before surgery for
selected anatomic sites (head, neck and pelvis).
2. Radiation
 3. Chemotherapy-
 Dactinomycin (Actinomycin D), vincristine (oncovin), cyclophosphamide
(cytoxan), doxorubicin (Adriamycinn), cisplatin (Platinol), etoposide(VP_ 16
VePesid), Ifosfamide (IFEX), and melphalan(Alkeran
Prognosis
 Survival rates have improved considerably in recent
years, and overall survival is approximately 70%.
 Survival for Stage I favourable histology is greater than
85%, stage 4 is less than 30%.
 Prognosis is related to the stage of the disease at
diagnosis, the location of the primary tumor, and the age
at diagnosis.
Complication
 Direct tumor extension to CNS with cranial nerve palsy,
brain stem compromise with bradypnea and
bradycardia.
 Metastasis to bone, bone marrow, lung.
NURSING MANAGEMENT
 Nursing Assessment
Nursing Diagnoses
 Anxiety of parents related to learning of diagnosis .
 Anxiety of child related to diagnostic procedures and surgery or
biopsy.
 Imbalanced nutrition less then body requirements related to
anaemia, anorexia , nausea, vomiting and mucosal ulceration
secondary to chemotherapy or radiation.
 Acute pain related to surgery or possible progression of disease.
 Disturbed body image related to alopecia associated with
chemotherapy.
 Risk for infection related to bone marrow suppression from
chemotherapy or radiation.
 Definition
 Retinoblastoma (Rb) is a rapidly developing cancer that develops
from the immature cells of a retina, the light detecting tissue of the
eye and is the most common malignant tumor of the eye in children.
RETINOBLASTOMA
Incidence
 Retinoblastoma presents with cumulative
lifetime incidence rate of 18000 to 30000 live
births worldwide.
 In the developed world, retinoblastoma has
one of the best cure rates of all childhood
cancers (95-98%), with more than nine out of
every ten sufferers surviving into adulthood.
Classification
 Heritable form
 Non-heritable form
Cause of retinoblastoma
 Heridity or chromosomal abnormality
 The genetic codes found in chromosomes control the way in which
cells grow and develop within the body.
 If a portion of the code is missing or altered (mutation) a cancer may
develop.
 Inherited forms of retinoblastomas are more likely to be bilateral; in
addition, they may be associated with pinealoblastoma (also known as
trilateral retinoblastoma) with a dismal outcome
Signs and symptoms
 “Cat’s eye reflex” – whitish appearance of the pupil (leukocoria),
represents visualization of the tumor through the lens as light falls on
the tumor mass- most common sign. Absent red reflex on photographs
is another sign.
 Strabisumus – second most common presenting sign.
An abnormal appearance of the
pupil, leukocoria, also known as amaurotic
cat's eye reflex.
Other occasional presenting signs
 Orbital inflammation
 Hyphema
 Fixed pupil
 Heterochromia iridis- different colours of each
iris or in the same iris.
CONT……….
 Vision loss
 Symptoms of distant metastasis – anorexia weight loss,
vomiting, headache, bone pain.
 Some children with retinoblastoma can develop a squint
 commonly referred to as "cross-eyed" or "wall-eyed"
(strabismus).
 Eye enlargement
Strabismus
 Esotropia - affected
eye turns inwards
 Exotropia - affected
eye turns outwards
 Hypertropia -
affected eye turns
upwards
 Hyoptropia - affected
eye turns downwards
CONT..
 The presence of the
photographic fault red eye in
only one eye and not in the
other may be a sign of
retinoblastoma.
 A clearer sign is "white eye"
or "cat's eye" (leukocoria)
Diagnostic Evaluation
 Bilateral indirect opthalmoscopy under
general anaesthesia.
 Ultrasonography and CT scan or MRI of head
and eyes to visualize tumor.
 Bone marrow aspiration and lumbar puncture
under anaesthesia to determine metastasis.
CONT……..
 The red reflex:
checking for a normal
reddish-orange
reflection from the
eye's retina with an
ophthalmoscope or
retinoscope from
approximately 30
cm / 1 foot, usually
done in a dimly lit
or dark room.
CONT………
 The corneal light reflex
/ Hirschberg test: checking
for symmetrical reflection of
beam of light in the same
spot on each eye when a
light is shined into each
cornea, to help determine
whether the eyes are crossed.
Management
 Depends on the stage
 Unilateral tumor in stage I,II, or III are usually treated with
external beam irradiation.
 Goal of treatment is to eradicate the tumor and to preserve
useful vision.
 Radiation is usually administered over the course of 3 to 4
weeks.
 Surgery (enucleation) is the treatment of choice for advanced
tumor growth , especially with optic nerve involvement
when no hope exists for useful vision.
CONT…….
 Radioactive applicators', light coagulation, and cryotherapy
are sometimes used to treat small, localized tumours.
 Intraocular penetration of systemic drugs is poor, so
chemotherapy is used for cases of extra ocular disease,
regional or distant metastasis, Generally, chemotherapy
response has been of short duration;
 however , a phase II trial of etoposide (Toposar, vePesid) and
carboplatin (paraplatin) has provide an 85% response rate for
children with extraocular disease.
CONT…..
 Overall survival rate is high (90%).
 Heritable retinoblastoma and bilateral
retinoblastoma are associated with a high
incidence of spontaneous and radiation-
related new tumor, particularly sarcomas.
Complications
 Spread to brain and other eye.
 Metastasis to bone, bone marrow, liver, and
lymph nodes.
NURSING MANAGEMENT
 Nursing Assessment
Nursing diagnosis
 Impaired tissue integrity related to skin changes, loss of
lashes, fat atrophy, impaired bone growth, and dryness caused
by radiation.
 Disturbed body image and ineffective coping related to
enucleation and need for an eye prosthesis.
 Impaired sensory perception (visual) related to disease process
or enucleation.
 Fear of child related to hospitalization and to diagnostic and
treatment procedures.
 Anxiety of parents related to the diagnosis, treatment and
genetic implication of the diagnosis.
HEPATOBLASTOMA
Hepatoblastoma is an uncommon
malignant liver neoplasm occurring in
infants and children and composed of
tissue resembling fetal liver cells,
mature liver cells, or bile duct cells.
Alpha-fetoprotein (AFP) commonly is
elevated, but when AFP is not elevated
at diagnosis the prognosis is poor.
EPIDEMIOLOGY
 Hepatoblastoma occurs predominantly in children <3 yr of age.
 Hepatoblastoma also is associated with Beckwith-Wiedemann
syndrome, which can show a similar loss of genomic imprinting
of the insulin-like growth factor-2 gene.
 Low birth weight is associated with increased incidence of
hepatoblastoma, with the risk increasing as birth weight decreases.
PATHOGENESIS
 Hepatoblastoma can be epithelial type, containing fetal or
embryonal malignant cells (either as a mixture or as pure
elements), or the mixed type, containing mesenchymal and
epithelial elements.
 Pure fetal histology predicts a more favourable outcome.
CLINICAL MANIFESTATIONS
 Large, asymptomatic abdominal mass.
 Right lobe 3 times more often than the left and usually is
unifocal.
 Weight loss,
 Anorexia,
 Vomiting,
 Abdominal pain
 Most commonly involves regional lymph nodes and the
lungs.
DIAGNOSTIC EVALUATION
 A valuable serum tumor marker, α-fetoprotein (AFP)
 AFP level is elevated in almost all hepatoblastomas.
 Bilirubin and liver enzymes usually are normal.
 Anemia is common, and thrombocytosis occurs in about ⅓ of
patients.
 Hepatitis B and C serology should be obtained but usually are
negative in hepatoblastoma.
CONT……
 Plain radiographs and ultrasonography of the
abdomen
 Ultrasonography can differentiate malignant
hepatic masses from benign vascular lesions.
 Either ECT or MRI
 CT of the chest and bone scan.
TREATMENT
Child with liver mass
Cross sectional imaging of the abdomen and chest using CT or MRI serum level
of AFP
No Resectable by standard lobectomy Yes
Biopsy to confirm diagnosis Resect
Chemotherapy Histology of lesion
Restudy every 2 cycles CT scan and AFP Chemotherapy unless pure fetal
histology
CONT……..
Restudy every 2 cycles CT scan and AFP
No Yes
List for liver transplantation Resects
Transplant Complete chemotherapy
Chemotherapy- 2 cycles
Nursing Care
 Follow-up care:
 Multidisciplinary evaluation
 Liver transplantation
 Diet
 Activity
 A bone tumor, (also spelled bone tumour), is
a neoplastic growth of tissue in bone. Abnormal growths
found in the bone can be either benign(noncancerous)
or malignant (cancerous).
Bone Tumor
INCIDENCE
 Bone cancer is caused by a problem
with the cells that form bone.
 More than 2,000 people are
diagnosed in the U.S. each year with a
bone tumor.
C
L
A
S
S
I
F
I
C
A
T
I
O
N
• Which originate in
bone or from bone-
derived cells and tissue.
Primary
bone
tumors
• Which originate in
other sites and
spread (metastasize)
to the skeleton.
Secondary
bone
tumors
C
L
A
S
S
I
F
I
C
A
T
I
O
N
 3 levels of malignant tumor stages
• When a low degree of
malignancy
Stage I
• Meaning the tumor has a
high degree of malignancy
Stage II
• Which means the tumor
has spread
Stage III
Causes
 Genetics
 Radiation treatment
 Injuries to the bone
 Bone tumors occur most commonly in children and
adolescents
 less common in the older adults.
 Cancer involving the bone in adults older are most
commonly the result of metastatic spread from another
tumor.
Symptoms
 Pain (Pain increases with the growth of the tumor)
 Fever,
 Weight loss,
 Anemia,
 Unexplained bone fractures.
 Many patients will not experience any symptoms,
except for a painless mass.
 Some bone tumors may weaken the structure of the
bone, causing pathologic fractures.
Treatment
 Chemotherapy and radiotherapy
 Medication
 Surgical treatment
Surgical treatment
 Amputation
Types of amputation
Leg
 Below knee
 Above knee
 Symes
 Hip disarticulation
 Hemipelvectomy or hindquarter, in which the whole
leg is removed with one half of the pelvis
Arm
 Below elbow
 Above elbow
 Shoulder disarticulation
 Forequarter (amputation of the whole arm,
along with the shoulder blade and the
clavicle)
Prognosis
 The outcome is expected to be good for people with
noncancerous (benign) tumors, although some types of
benign tumors may eventually become cancerous
(malignant).
 With malignant bone tumors that have not spread,
most patients achieve a cure, but the cure rate depends
on the type of cancer, location, size, and other factors.
Nursing Diagnosis
1. Anxiety related to change in health status.
2. Chronic Pain related to pathologic processes.
3. Imbalanced Nutrition Less Than Body Requirements related to
hypermetabolic status with regard to cancer, the consequences of
chemotherapy, and radiation effects
4. Risk for Fluid Volume Excess related to damage to fluid intake.
5. Risk for Infection related to the inadequate immunosuppression,
malnutrition and invasive procedures.
6. Risk for Impaired skin integrity related to radiation effects and
changes in nutritional status.
Quiz Time
Oncology disorder

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Oncology disorder

  • 1. Seminar on CHILD-WITH ONCOLOGICAL DISORDERS Presented by Mr. Abhijit Bhoyar
  • 2. Anatomy and physiology Function of Blood  It acts as a transport system  Conveys oxygen to the tissues  Phagocytes action  Distributes proteins needed for tissue formation  Produce hormones and enzyme to organs.
  • 3. LEUKEMIA  Introduction:-Cancer account for the major cause of death in India children; next only to infection and malnutrition. Leukaemia is the most common form of childhood malignancy.
  • 4. Leukaemia Definition  Leukaemia is group of malignant disease of abnormal proliferation and maturation of bone marrow which interfere with the production of normal RBC, WBC and platelet.  It is mostly characterised by persistent and uncontrolled production of immature and abnormal white blood cells (WBCs).
  • 5. Incidence  The annual incidence is 3-4 cases per 100,000 children globally.  It occurs frequently in boys than in girls after age 1 year and peak onset is between 2-6 years of age.  About 95-98% of childhood leukaemia are acute type.
  • 6. Etiology  Congenital or hereditary  Environmental factors  Drugs and chemicals  Viral pathogenesis  Chromosomal abnormalities or aberrations.
  • 7. Classification of Leukemia • Acute lymphocytes leukemia(ALL) • Acute non-lymphocyte leukemia (ANLL) or Acute myelocytic leukemia. ACUTE LEUKEMIA • Chronic myeloid leukemia (CML) • Chronic lymphocyte leukemia (CLL) CHRONIC LEUKEMIA 1 2
  • 8. Acute lymphocytes leukaemia (ALL)  It is the primary disorder of the bone marrow in which the normal bone marrow elements are replaced by immature lymphoblasts from the lymphoid stem cell.
  • 9. FAB classification of acute lymphoblastic leukemia Cytologic features L1 (80-85%) L2(15%) L3(1-2%) Cell size Small cells predominate,homogenous Large heteroneous in size Large, homogenous Amount of cytoplasm Scanty Variable;often moderately abundant Moderately abundant Nucleoli Small, incospicuous One or more, often large One or more, prominent Nuclear chromatin Homogenous Variable, hetrogenous Stippled, homogenous Nuclear shape Regular, occasional clefts Irregular clefts,indentation Regular, oval to round Cytoplasmic basophilia Variable Variable Intensely basophlic Cytoplasmic Variable Variable Prominent
  • 10. 2. Acute non-lymphocyte leukemia (ANLL)-  In this there is progressive proliferation of immature monocytes and myelocytes from the bone marrow that invade the blood and other tissues.  15-20% of childhood leukemias. Blood Smear of a patient with Leukemia
  • 11. A : Picture of bone marrow smear (control); Normal granulocytes and erythroblasts are evident. B : Acute lymphoid leukemia (ALL); There is a marked proliferation of small lymphoblasts. C : Acute myeloid leukemia (AML); There is a marked proliferation of large myeloblasts. D : Chronic myeloid leukemia (CML); There is a marked proliferation of granulocytes at various stages of maturation. A D C B
  • 12. 1. Chronic myeloid leukemia (CML)  Chronic myelocytic leukemia (CML) it arises from the mutation in the myeloid stem cell  It is rare in children i.e. 2-3% of all leukemias.  It presents in child as adult and juvenile type. Juvenile type occurs below 5 years of age.
  • 13. Pathopysiology Due to any etiological factors Growth of abnormal fragile WBC in bone marrow spleen and lymph nodes Normal bone is replaced by leukemic cells RBC and Platelet formation decreased Leukemic cells infiltrated to spleen and liver, bone Anemia , bleeding petechiae Hepatospleenomegaly and lymphgland enlargement , joint and bone pain.
  • 14. Clinical Manifestation  Fever  Pallor and fatigue  Anorexia,  Weight loss  Haemorrhage  Petechiae  Bone and joint pain
  • 15. Cont..  Infection  Anemia  Hepatosleenomegaly  Thrombocytopenia  Malaise and weakness  Hematemesis ,  Malena,  Hematuria  Abdominal mass.
  • 16.
  • 17. Diagnostic Evaluation  Blood count  Bone marrow study  Chest radiography  CSF Study  Blood chemistry  Coagulation profile CYTOGENIC ANALYSIS
  • 18. Management CHEMOTHERAPY 1) Remission induction phase 2) Maintenance or continuation therapy 3) Intensification or consolidation 4) CNS prophylactic therapy
  • 19.
  • 20. 1) Remission induction phase  Immediately after confirming diagnosis, induction therapy is began and lasts for 4-6 weeks.  Drugs used are corticosteroids (prednisolone or dexamethasone), vincristine and L- asparginase with or without doxorubicin.  These drugs give 95% remisson.
  • 21. 2) Maintenance or continuation therapy  It is to maintain the disease control and prevent there relapse.  It is to preserve remission and further reduce number of leukemic cells.  Drugs include daily dose of oral 6- mercaptopurine and weekly dose of methotrexate and intermitten pulses of vincristine and steroids which are standard in most treatment regimen.
  • 22. 3) Intensification or consolidation  It is used to further decrease the number of leukemic cells in Childs body.  The drug regimen include L-Asprginase methotreaxate with vincristine, cytarabine.  It consist of pulses of these agents given periodically during the 1st 6months of treatment.  It is given every 4 weeks.
  • 23. 4) CNS prophylactic therapy  Children receive triple intrathecal therapy of methotrexate,  cytarabine and hydrocortisone and is used during induction, intensification and maintenance therapy i.e. once a week during induction and then every 8 weeks for 2years.
  • 24. Surgical Management  Bone Marrow transplantation
  • 26. Nursing Diagnosis 1.Risk of infection and haemorrhage related to bone marrow suppression caused by chemotherapy and disease. 2. Disturbed body image related to alopecia associated with chemotherapy. 3. Activity intolerance related to fatigue that results from the disease and treatment. 4. Imbalanced nutrition less than body requirement related to anaemia, anorexia, vomiting and mucosal ulceration secondary to chemotherapy or radiation. 5. Anxiety of parents related to hospitalization and diagnosis.
  • 27. Complication  Bleeding  Heart block  Infection (GI, lungs, skin)  CNS and skeletal involment
  • 28. Prognosis  Overall cure rate is 65-75%.  Prognosis after transplantation varies with the leukaemia.  Higher WBC count initially has worse prognosis.
  • 29. LYMPHOMA  Lymphomas are the third most common malignancy in children and adolescents, after leukemia and brain tumors  About 60% are non-Hodgkin’s lymphoma and 40% are Hodgkin’s disease.  Lymphomas are uncommon below the age of 5 years and the incidence increases with age Lymphoma is cancer that begins in infection-fighting cells of the immune system, called lymphocytes. These cells are in the lymph nodes, spleen, thymus, bone marrow, and other parts of the body. When you have lymphoma, lymphocytes change and grow out of control.
  • 30. Hodgkin’s disease Definition  Hodgkin’s lymphoma is characterised by progressive enlargement of lymph nodes.  The disease is considered unicentric in origin and has a predictable pattern of spread by extension to contiguous nodes.
  • 31. Incidence  Hodgkin’s disease occur in 5 to 7 per 1,00,000 population.  The incidence is highest in late childhood and early adulthood (15-35 years).  The sex ratio progresses from male preponderance of 10:1 under the age of 7 years falling to 1.1:1 after the age of 12 years.
  • 32. Etiology  Ethnic groups and association with human leukocyte antigen.  Inherited susceptibility plays an important role in the pathogenesis.  Environmental factors  Higher incidence in twins
  • 33. Pathology Hodgkin Lymphoma divided as 1. Nodular Lymphocyte- Predominant Hodgkin Lymphoma ( NLPHL) 2. Classical Hodgkin Lymphoma
  • 34. Nodular Lymphocyte-Predominant Hodgkin Lymphoma ( NLPHL)  Characterized by large cells with multibed nuclei, referred to as popcorn cells.  NLPHL is most common in males younger than 10 years.
  • 35. Classical Hodgkin Lymphoma  The hallmark of classic Hodgkin Lymphoma is the R-S cell.  This is a binucleated or multinucleated giant cell that is often characterised by a bilobed nucleus, with two large nucleoli, giving an owl’s eye appearance to the cells.  The classical subtype are defined according to the number of Reed- Sternberg (R-S) cells, characteristics of the inflammatory milieu, and the presence or absence of fibrosis .
  • 36. Staging  A- No symptoms  B- Fever, night sweats, or weight loss of more than 10% of body weight in the previous 6 months.  X- Bulky disease (greater than 10 cm in maximum dimension; greater than 1/3rd of the internal transverse diameter of the thorax at the level T5/T6.
  • 37.
  • 38. Anatomic definition of lymph node regions used for staging purposes
  • 39. Ann Arbor staging classification for Hodgkin lymphoma Stage Definition I Involment of single lymph node region (I) or of a single extra lymphatic organ or site (IE) by direct extension II Involment of two or more lymph node regions on the same side of the diaphragm (II) or localized involment of an extralyphatic organ or site and one or more lymph node regions on the same side of the diaphragm (IIE) III Involvement of lymph node regions on both sides of the diaphragm (III), which may be accompanied by involvement of the spleen (IIIS), or by localized involment of an extralymphatic organ or site (IIIE) or both III (E+S) IV Diffuse or disseminated (multifocal) involvement of one or more extralymnphatic organ or tissues with or without associated lymph node involvement.
  • 40. Stage Definition Systemic Symptoms Each stage is subdivided into A and B subcategories, B for those with defined systemic symptoms and A for those without any. The B designation is given to patients with: (i) unexplained loss of >10% of body weight in 6 months before diagnosis (ii) unexplained fever with temperature above 380c for 3 consecutive days (iii) drenching night sweats. Pruritis alone does not qualify for B classification, nor does a short febrile illness associated with an infection.
  • 41. Clinical manifestation  Painless enlargement of lymph nodes  Firm, nontender, moveable nodes in the supraclavicular or cervical area.  In children the sentinel node located near the left clavical  Enlargement of axillary and inguinal lymph nodes.
  • 42. How do they present?
  • 43. Other  Mediastinal lymphadenopathy  Enlarged retroperitoneal nodes  Systemic symptoms include low-grade or intermittent fever (Pel-Ebstein disease)  Anorexia  Nausea  Weight Loss  Night Sweats  Pruritus
  • 44. Diagnostic Evaluation  History and Physical examination  Complete blood count  Liver and renal function tests, serum LDH, serum albumin  Lymph node biopsy  Bone marrow aspiration and biopsy
  • 45. Cont..  Chest X-ray,  CT scan of neck, chest and abdomen and pelvis  Positron emission tomography (of the PET) scan whole body
  • 46. Treatments  Chemotherapy  Radiation therapy Prognostic factors=70-90% patients with Hodgkin lymphoma are cured.
  • 48. Cont..  Early stage (I,II) Hodgkin lymphoma are given combination chemotherapy (e.g. 2-4 cycles of ABVD comprising adriamycin, bleomycin, vinblastine, dacarbazine) with or without, radiotherapy.
  • 49.
  • 50. Cont..  Advanced stage of Hodgkin lymphoma are given 4-6 cycles of chemotherapy(e.g. ABVD).  Other combination chemotherapy include 6-8 cycles of MOPP ( nitrogen mustard, vincristine, procarbazine and prednisolone)  COPP regime in which nitrogen mustard is replaced of ABVD and COPP ( 4 cycles of each).
  • 51. Nursing diagnosis  Impaired tissue integrity related to high-dose radiation therapy.  Impaired oral mucous membrane related to high-dose radiation therapy.
  • 52. NON-HODGKIN’S LYMPHOMA  Definition Non- Hodgkin's lymphoma are a group of malignancies of lymphoid tissue arising from T or B lymphocytes or their precursors; include both indolent and aggressive.
  • 53. Epidemiology  Male to female ratio of 3:1.  In equatorial Africa, 50 % of all cancers are lymphomas (Burkitt lymphoma being predominant).  In India, lymphoblastic lymphoma or more common.  Infection with malaria and EB virus are considered risk factors for Burkitt lymphoma.
  • 54. Staging system for Non- Hodgkin lymphoma Stage I A single tumor or nodal area is involved, excluding the abdomen and mediastinum. Stage II Disease extent limited to a single tumor with regional node involvement, 2 or more tumors or nodal areas involved on one side of the diaphragm, or a primary gastrointestinal tract tumour (completely resected) with or without regional node involvement. Stage III Tumours or involved lymph node areas; occur on both sides of the diaphragm. Also includes any primary intrathoracic (Mediastinal, pleural or thymic0 Disease, extensive primary intra-abdominal disease, or any paraspinal or epidural tumors. Stage IV Bone marrow and/or central nervous system disease, regardless of other sites of involvement.
  • 55. Etiology  Immune System  Other Risk Factors  Male Gender  White Ethnicity  History Of Helicobacter Gastritis  History Of Hodgkin’s Lymphoma  History Of Radiation Therapy  Diet High In Meats And Fat  Exposure To Certain Pesticides.
  • 56. Clinical manifestation  Extranodal disease involving the mediastrnium, abdomen or head and neck region.  Intrathoracic NHL, most often T-cell lymphoma  Cough  Dyspnoea  Dysphagia  Chest pain or the superior vena cava syndrome.  Pleural and pericardianl effusion.
  • 57.
  • 58. Cont..  Cervical Lymphadenopathy  Abdominal Pain  Ascities  Palpable Abdominal Mass  Intestinal Obstruction  Intussusceptions(typical B- cell Disease)  Cranial Nerve Palsy  Bone Involvement  Jaw Swelling (Burkitt Lymphoma) And Pancytopenia Due To Bone Marrow Involvement May Occur.
  • 59. Diagnostic Evaluation  History and physical examination  Complete blood count, peripheral smear  Renal function, uric acid, liver function, serum albumin, lactate dehydrogenase  Examination of cerebrospinal, Peritoneal, pericardial or pleural fluid- cytomorphology and immuophenotyping
  • 60. Cont..  Surgical biopsy  Chest radiograph or CT scan of the chest  Ultrasonography /CT- scan of the abdomen with contrast  Bone marrow aspiration and biopsy  Plain radiogram/ Ct- scan of other affected sites  PET scan of whole body (optional)
  • 61.
  • 62. Management  Chemotherapy and supportive care.  Surgery  Radiotherapy e.g. superior vena cava syndrome or spinal cord compression due to paraspinal disease.  Chemotherapeutic regimens  The regimens for lymphoblastic lymphoma are usually based on protocols for ALL.
  • 63. Cont…  Most successful protocols are the German BFM (Burlin, Frankurt, Munster)protocol and a modified version of LS A2 L2 protocol.  Cranial irradiation or prophylactic intrathecal chemotherapy.  90% patients with limited disease and 75-85% in patients with bone marrow disease.  The use of antiCD20 monoclonal antibodies
  • 64.
  • 65. Cont..  Most protocols consisits of short duration (6 months), intensive alkylating agent therapy (cyclophosphamde or ifosphamie) with high dose methotrexate, vincristine, anthracyclines, etoposide and cytarabine;  CNS prophylaxis is provided with intrathecal chemotheraphy.
  • 67. Nursing management Nursing diagnosis  Risk for infection related to altered immune response because of lymphoma and leukopenia caused by chemotherapy or radiation therapy
  • 68. WILM’S TUMOR Wilms' tumor is a rare kidney cancer that primarily affects children. Also known as nephroblastoma, it's the most common cancer of the kidneys in children.
  • 69. Definition-  Wilm’s tumor is a malignant renal tumor and is the most common renal neoplasm in children.
  • 70. Incidence  The incidence is 7.6 cases per million for children younger than age 15% of cases occur before the child is age 5.  Most commonly a unilateral disease, but in 5% to 10%, both kidneys are involved.
  • 71. Causes  Wilm;s tumor is not know.  Genetic inheritance
  • 72. Pathopysiology- Due to etiology Develop in the renal parenchyma in either the central or polar location. Entire renal parenchyma may appear replaced by tumor. The majority of the tumor present as a single, expanding mass surrounded by a pseudo capsule of connective tissue that appears to separate kidney and tumor .
  • 73. Cont.. The tumor is encapsulated, the membrane may be very thin and easily torn. Because of their rapid growth, the lesions are often vascular, soft, gelatinous, and necrotic in the centre Lesions are composed of mesenchymal and epithelial elements in various ratios and various stages of maturity. Some tumor are composed mainly of sheets of anaplastic cells with little evidence of differentiation.
  • 74. Clinical manifestation-  A firm, nontender upper quadrant abdominal mass  Abdominal pain,  Less common are hypertension, fever, hematuria, and anemia
  • 75. Associated anomalies a) Hemihypertrophy b) Aniridia (without the iris) c) Genito urinary anomalies d) Over growth syndrome (i.e. beck with wiedemann syndrome)
  • 77. Diagnostic evaluation  Abdominal ultrasound  Radiography  Complete blood count (CBC) and peripheral smear  Urinalysis  Blood chemistry  Urinary VMA and HVA  MRI or CT scan  Ultrasonography
  • 78. Staging of wilm’s tumor  Stage I-  Tumor is limited to the kidney and is completely resected.  Surface of the renal capsule is intact.  Tumor is not ruptured before or during removal, and no residual tumor is apparent beyond the margins of resection.
  • 79.
  • 80. Stage II  Tumor extend beyond the kidney but is completely excised.
  • 81. Stage III  Residual non haematogenous tumor confined to the abdomen.
  • 82. Stage IV  Haematogenous metastasis with deposits beyond stage III (i.e. lung, liver, bone and brain)
  • 83. Stage V  Bilateral renal involment at diagnosis
  • 85. Management  Histological findings clinical staging and metastasis.  Stage I and II wilm’s tumor usually managed with nephrectomy and chemotherapy for 18 weeks.  Stage III, IV and V tumours are treated with nephrectomy, abdominal radiotherapy and chemotherapy for 24 weeks.  Radiotherapy
  • 86. Therapeutic management  Surgical management  Radiation therapy  Chemotherapy
  • 87. Nursing Management- Goal include  Assessing for the presence of wilm’s tumor  Emotional support  Diagnostic procedure and treatment management  Planning for discharge.
  • 88. Nursing Diagnosis-  Ineffective thermoregulation related to infection  Imbalanced nutritional status less than body requirement related to family nutritional pattern and secondary to disease condition.  Risk of infection related to the disease condition and secondary to immunity.  Disturbed sleeping pattern due to hospitalization and secondary to disease condition.
  • 89. Complication  Metastasis to lung , lymph nodes, liver, bone and brain.  Complications from radiation therapy include-  bowel obstruction  hepatic damage  nephritis  sterility in girls and interstitial pneumonia.
  • 90. NEUROBLASTOMA  Neuroblastoma is the most common extracranial solid tumor in children and the most common tumor in infancy.
  • 91. Neuroblastoma most commonly occurs in:-  Either one of the two adrenal glands situated in the abdomen (tummy)  Nerve tissue that runs alongside the spinal cord, in the neck, chest, abdomen or pelvis. Neuroblastoma is a cancer of specialised nerve cells called neural crest cells. These cells are involved in the development of the nervous system and other
  • 92. Incidence  Fewer than 100 children in the UK are diagnosed each year.  Most children who get this cancer are younger than five years old.  Neuroblastoma is the second most common solid tumour in childhood, and it makes up 8% of the total number of children's cancers
  • 93. Epidemiology  Neuroblastoma is predominantly a tumor of early childhood, with two thirds of the cases presenting in children younger than 5 years. It accounts for 7-10% of all childhood cancers.
  • 95. Sign and symptoms  First symptoms are vague, such as tiredness, loss of appetite and pain in the bones.  More specific symptoms will depend on where the neuroblastoma starts:-  Tumour is in the abdomen----------  Chest area---------------  Neck-------------
  • 96. Figure 2: A) Infant with a large, distended abdomen and a right- sided abdomen mass. B) Infant with stage 4-S neuroblastoma with multiple skin nodules, sometimes called the blueberry muffin syndrome. C) This skin tumor on the scalp is found in another infant with stage 4-S neuroblastoma.
  • 97. CONT…..  Occasionally, there are deposits of neuroblastoma in the skin that appear as small, blue-coloured lumps.  Tumour is pressing on the spinal cord, children may have weakness in the legs and walk unsteadily. urine.  Very rarely, children may have jerky eye and muscle movements, and general unsteadiness associated with the neuroblastoma.  High blood pressure.
  • 98. Diagnostic criteria  General investigation  Biopsy  Blood and bone marrow tests  X-rays  CT or MRI scans,
  • 99. CONT…  Vanillylmandelic acid (VMA) or homovanillic acid (HVA) tests  MIBG scans (metaiodobenzyl guanidine)  Biopsy
  • 100.
  • 102. Staging system for neuroblastoma  Stage 1 The cancer is contained within one area of the body (localised) and there's no evidence of it having spread.
  • 103. CONT………  Stage 2A The cancer is localised and has not begun to spread, but it cannot be completely removed by surgery.  Stage 2B The cancer is localised and has begun to spread into nearby lymph nodes.  Stage 3 The cancer has spread into surrounding organs and structures, but has not spread to distant areas of the body.
  • 104. CONT…….  Stage 4 The cancer has spread to distant lymph nodes, bone, bone marrow, the liver, the skin or other organs.  Stage 4S (also called special neuroblastoma) This is found in children under one year old. The cancer is localised (as in stage 1, 2A or 2B) but has begun to spread to the liver, skin or bone marrow.
  • 105. OTHER STAGES  Stage L1 The tumour is localised and has not spread into important areas (vital structures) nearby. It can be removed by surgery.  Stage L2 The tumour is localised but has 'image- defined risk factors' and can't be safely removed by surgery.
  • 106. CONT….  Stage M The tumour has spread to other parts of the body.  Stage MS The tumour has spread to the skin, liver and/or the bone marrow in children younger than 18 months old.
  • 107. CONT…  If the cancer has spread to distant parts of the body, this is known as secondary or metastatic cancer.  If the cancer comes back after initial treatment, this is known as recurrent or relapsed cancer.
  • 108. Management  Surgery  Chemotherapy  High-dose chemotherapy with stem cell support  Monoclonal antibody treatment  Radiotherapy
  • 110. Side effect of treatment  Feeling sick (nausea) and being sick (vomiting)  Diarrhoea  Hair loss  Increased risk of infection  Bruising and bleeding  Tiredness
  • 111. Late side effect  Change in the way the heart and kidneys work  Hearing problems  Fertility problems  A possible reduction in bone growth  And a slightly increased risk of developing another cancer in later life.
  • 112. Complication  Metastasis to the liver, soft tissue, bones, lymph nodes, bone marrow and skin.  Neurologic deficits due to nerve compression.
  • 114. Nursing Diagnoses  Anxiety of parents related to learning of diagnosis.  Fear of child related to diagnostic procedures and surgery or biopsy.  Activity intolerance related to fatigue from tumor growth and bone marrow suppression.  Constipation or bowel and bladder incontinence related to pressure of tumor.  Risk for infection related to bone marrow suppression from chemotherapy and radiation.  Acute pain related to tumor, surgery or progression of disease.  Disturbed body image related to hair loss.
  • 115.  Incidence  Approximately 350 new cases are reported per year in the united state.  Rhabdomyosarcoma accounts for 3.5% of all malignant disease in children younger than age 14. Most common are the head and neck, genitourinary (GU) tract, and extremities. Rhabdomyosarcoma A Rhabdomyosarcoma, commonly referred to as RMS, is a type of cancer, specifically a sarcoma (cancer of connective tissues), in which the cancer cells are thought to arise from skeletal muscle progenitors.
  • 116. Etiology  Unknown  Certain genetic and environmental factors  Embryonal and alveolar.  Tumor spreads either by local extension or by metastasis via the venous and lymphatic system.  The lung is the most common site of metastasis.
  • 117. Tumor staging is based on the extent of the disease:-  Stage/group I: localized tumor completely resected.  Stage/ group II: local tumor resected, microscopic residual disease.  Stage/group III: localized disease, with gross residual disease after resection.  Stage/group IV: metastatic disease at diagnosis.
  • 118. Clinical Manifestations  Asymptomatic lump  Orbit - ptosis, ocular paralysis,exophthalmaos.  Nasopharynx- Epistaxis, pain, dysphagia, nasal voice, airway obstruction.  Sinuses – swelling, pain , discharge, sinusitis..  Middle ear- pain, chronic otitis, facial nerve palsy  Neck- hoarseness, dysphagia  Truncal, extremities, testicular areas- enlarging soft tissue masses  Prostate, bladder – urinary tract symptoms  Retroperitoneal tumours- GI and urinary obstruction, weakness, paresthesia, pain  Vaginal- abnormal vaginal bleeding or mass
  • 119. Diagnostic evaluation  Open biopsy of the primary tumor- definitive diagnostic procedure  CT scan of the chest node primary lesion  MRI  Bone marrow aspiration and biopsy  Bone scan or skeletal survey  Ultrasound  Chest X-ray  CBC, liver and renal function tests, electrolytes, serum calcium and phosphorus, uric acid  Monoclonal antibody assays  Urinalysis  Lumbar puncture
  • 120. Management 1. Surgery- to biopsy the lesion, determine the stage of the disease, and completely remove or reduce the primary tumor.  Increasingly, chemotherapy and radiation therapy are used before surgery for selected anatomic sites (head, neck and pelvis). 2. Radiation  3. Chemotherapy-  Dactinomycin (Actinomycin D), vincristine (oncovin), cyclophosphamide (cytoxan), doxorubicin (Adriamycinn), cisplatin (Platinol), etoposide(VP_ 16 VePesid), Ifosfamide (IFEX), and melphalan(Alkeran
  • 121. Prognosis  Survival rates have improved considerably in recent years, and overall survival is approximately 70%.  Survival for Stage I favourable histology is greater than 85%, stage 4 is less than 30%.  Prognosis is related to the stage of the disease at diagnosis, the location of the primary tumor, and the age at diagnosis.
  • 122. Complication  Direct tumor extension to CNS with cranial nerve palsy, brain stem compromise with bradypnea and bradycardia.  Metastasis to bone, bone marrow, lung.
  • 124. Nursing Diagnoses  Anxiety of parents related to learning of diagnosis .  Anxiety of child related to diagnostic procedures and surgery or biopsy.  Imbalanced nutrition less then body requirements related to anaemia, anorexia , nausea, vomiting and mucosal ulceration secondary to chemotherapy or radiation.  Acute pain related to surgery or possible progression of disease.  Disturbed body image related to alopecia associated with chemotherapy.  Risk for infection related to bone marrow suppression from chemotherapy or radiation.
  • 125.  Definition  Retinoblastoma (Rb) is a rapidly developing cancer that develops from the immature cells of a retina, the light detecting tissue of the eye and is the most common malignant tumor of the eye in children. RETINOBLASTOMA
  • 126. Incidence  Retinoblastoma presents with cumulative lifetime incidence rate of 18000 to 30000 live births worldwide.  In the developed world, retinoblastoma has one of the best cure rates of all childhood cancers (95-98%), with more than nine out of every ten sufferers surviving into adulthood.
  • 128. Cause of retinoblastoma  Heridity or chromosomal abnormality  The genetic codes found in chromosomes control the way in which cells grow and develop within the body.  If a portion of the code is missing or altered (mutation) a cancer may develop.  Inherited forms of retinoblastomas are more likely to be bilateral; in addition, they may be associated with pinealoblastoma (also known as trilateral retinoblastoma) with a dismal outcome
  • 129. Signs and symptoms  “Cat’s eye reflex” – whitish appearance of the pupil (leukocoria), represents visualization of the tumor through the lens as light falls on the tumor mass- most common sign. Absent red reflex on photographs is another sign.  Strabisumus – second most common presenting sign. An abnormal appearance of the pupil, leukocoria, also known as amaurotic cat's eye reflex.
  • 130. Other occasional presenting signs  Orbital inflammation  Hyphema  Fixed pupil  Heterochromia iridis- different colours of each iris or in the same iris.
  • 131. CONT……….  Vision loss  Symptoms of distant metastasis – anorexia weight loss, vomiting, headache, bone pain.  Some children with retinoblastoma can develop a squint  commonly referred to as "cross-eyed" or "wall-eyed" (strabismus).  Eye enlargement
  • 132. Strabismus  Esotropia - affected eye turns inwards  Exotropia - affected eye turns outwards  Hypertropia - affected eye turns upwards  Hyoptropia - affected eye turns downwards
  • 133. CONT..  The presence of the photographic fault red eye in only one eye and not in the other may be a sign of retinoblastoma.  A clearer sign is "white eye" or "cat's eye" (leukocoria)
  • 134. Diagnostic Evaluation  Bilateral indirect opthalmoscopy under general anaesthesia.  Ultrasonography and CT scan or MRI of head and eyes to visualize tumor.  Bone marrow aspiration and lumbar puncture under anaesthesia to determine metastasis.
  • 135. CONT……..  The red reflex: checking for a normal reddish-orange reflection from the eye's retina with an ophthalmoscope or retinoscope from approximately 30 cm / 1 foot, usually done in a dimly lit or dark room.
  • 136. CONT………  The corneal light reflex / Hirschberg test: checking for symmetrical reflection of beam of light in the same spot on each eye when a light is shined into each cornea, to help determine whether the eyes are crossed.
  • 137. Management  Depends on the stage  Unilateral tumor in stage I,II, or III are usually treated with external beam irradiation.  Goal of treatment is to eradicate the tumor and to preserve useful vision.  Radiation is usually administered over the course of 3 to 4 weeks.  Surgery (enucleation) is the treatment of choice for advanced tumor growth , especially with optic nerve involvement when no hope exists for useful vision.
  • 138. CONT…….  Radioactive applicators', light coagulation, and cryotherapy are sometimes used to treat small, localized tumours.  Intraocular penetration of systemic drugs is poor, so chemotherapy is used for cases of extra ocular disease, regional or distant metastasis, Generally, chemotherapy response has been of short duration;  however , a phase II trial of etoposide (Toposar, vePesid) and carboplatin (paraplatin) has provide an 85% response rate for children with extraocular disease.
  • 139. CONT…..  Overall survival rate is high (90%).  Heritable retinoblastoma and bilateral retinoblastoma are associated with a high incidence of spontaneous and radiation- related new tumor, particularly sarcomas.
  • 140. Complications  Spread to brain and other eye.  Metastasis to bone, bone marrow, liver, and lymph nodes.
  • 142. Nursing diagnosis  Impaired tissue integrity related to skin changes, loss of lashes, fat atrophy, impaired bone growth, and dryness caused by radiation.  Disturbed body image and ineffective coping related to enucleation and need for an eye prosthesis.  Impaired sensory perception (visual) related to disease process or enucleation.  Fear of child related to hospitalization and to diagnostic and treatment procedures.  Anxiety of parents related to the diagnosis, treatment and genetic implication of the diagnosis.
  • 143. HEPATOBLASTOMA Hepatoblastoma is an uncommon malignant liver neoplasm occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. Alpha-fetoprotein (AFP) commonly is elevated, but when AFP is not elevated at diagnosis the prognosis is poor.
  • 144. EPIDEMIOLOGY  Hepatoblastoma occurs predominantly in children <3 yr of age.  Hepatoblastoma also is associated with Beckwith-Wiedemann syndrome, which can show a similar loss of genomic imprinting of the insulin-like growth factor-2 gene.  Low birth weight is associated with increased incidence of hepatoblastoma, with the risk increasing as birth weight decreases.
  • 145. PATHOGENESIS  Hepatoblastoma can be epithelial type, containing fetal or embryonal malignant cells (either as a mixture or as pure elements), or the mixed type, containing mesenchymal and epithelial elements.  Pure fetal histology predicts a more favourable outcome.
  • 146. CLINICAL MANIFESTATIONS  Large, asymptomatic abdominal mass.  Right lobe 3 times more often than the left and usually is unifocal.  Weight loss,  Anorexia,  Vomiting,  Abdominal pain  Most commonly involves regional lymph nodes and the lungs.
  • 147. DIAGNOSTIC EVALUATION  A valuable serum tumor marker, α-fetoprotein (AFP)  AFP level is elevated in almost all hepatoblastomas.  Bilirubin and liver enzymes usually are normal.  Anemia is common, and thrombocytosis occurs in about ⅓ of patients.  Hepatitis B and C serology should be obtained but usually are negative in hepatoblastoma.
  • 148. CONT……  Plain radiographs and ultrasonography of the abdomen  Ultrasonography can differentiate malignant hepatic masses from benign vascular lesions.  Either ECT or MRI  CT of the chest and bone scan.
  • 149. TREATMENT Child with liver mass Cross sectional imaging of the abdomen and chest using CT or MRI serum level of AFP No Resectable by standard lobectomy Yes Biopsy to confirm diagnosis Resect Chemotherapy Histology of lesion Restudy every 2 cycles CT scan and AFP Chemotherapy unless pure fetal histology
  • 150. CONT…….. Restudy every 2 cycles CT scan and AFP No Yes List for liver transplantation Resects Transplant Complete chemotherapy Chemotherapy- 2 cycles
  • 151. Nursing Care  Follow-up care:  Multidisciplinary evaluation  Liver transplantation  Diet  Activity
  • 152.  A bone tumor, (also spelled bone tumour), is a neoplastic growth of tissue in bone. Abnormal growths found in the bone can be either benign(noncancerous) or malignant (cancerous). Bone Tumor
  • 153.
  • 154. INCIDENCE  Bone cancer is caused by a problem with the cells that form bone.  More than 2,000 people are diagnosed in the U.S. each year with a bone tumor.
  • 155. C L A S S I F I C A T I O N • Which originate in bone or from bone- derived cells and tissue. Primary bone tumors • Which originate in other sites and spread (metastasize) to the skeleton. Secondary bone tumors
  • 156. C L A S S I F I C A T I O N  3 levels of malignant tumor stages • When a low degree of malignancy Stage I • Meaning the tumor has a high degree of malignancy Stage II • Which means the tumor has spread Stage III
  • 157. Causes  Genetics  Radiation treatment  Injuries to the bone  Bone tumors occur most commonly in children and adolescents  less common in the older adults.  Cancer involving the bone in adults older are most commonly the result of metastatic spread from another tumor.
  • 158. Symptoms  Pain (Pain increases with the growth of the tumor)  Fever,  Weight loss,  Anemia,  Unexplained bone fractures.  Many patients will not experience any symptoms, except for a painless mass.  Some bone tumors may weaken the structure of the bone, causing pathologic fractures.
  • 159.
  • 160. Treatment  Chemotherapy and radiotherapy  Medication  Surgical treatment
  • 161. Surgical treatment  Amputation Types of amputation Leg  Below knee  Above knee  Symes  Hip disarticulation  Hemipelvectomy or hindquarter, in which the whole leg is removed with one half of the pelvis
  • 162. Arm  Below elbow  Above elbow  Shoulder disarticulation  Forequarter (amputation of the whole arm, along with the shoulder blade and the clavicle)
  • 163. Prognosis  The outcome is expected to be good for people with noncancerous (benign) tumors, although some types of benign tumors may eventually become cancerous (malignant).  With malignant bone tumors that have not spread, most patients achieve a cure, but the cure rate depends on the type of cancer, location, size, and other factors.
  • 164. Nursing Diagnosis 1. Anxiety related to change in health status. 2. Chronic Pain related to pathologic processes. 3. Imbalanced Nutrition Less Than Body Requirements related to hypermetabolic status with regard to cancer, the consequences of chemotherapy, and radiation effects 4. Risk for Fluid Volume Excess related to damage to fluid intake. 5. Risk for Infection related to the inadequate immunosuppression, malnutrition and invasive procedures. 6. Risk for Impaired skin integrity related to radiation effects and changes in nutritional status.