This document provides information on the types of cancers that affect children, including hematological malignancies such as leukemia and lymphomas, as well as solid tumors like brain tumors and abdominal masses. It discusses the signs, symptoms, diagnostic testing, staging, and treatment options for common childhood cancers.

1. DR ESTHER MAJALIWA
KCMC ONCOLOGY
DEPARTMENT

8. TYPES OF CANCERS IN CHILDREN
CHILDH00D
CANCERS
HAEMATOLOGICAL
MALIGNANCY
SOLID TUMORS BRAIN TUMORS

11. Hematologic malignancies
•
Form of cancer that begin in the cells of blood-forming tissue,
such as the bone marrow, or in the cells of the immune system
ie: lymphatic system.

12. Types
•Leukemia's
• Onset: acute or chronic
• Lineage: myeloid or lymphoid
•Lymphomas
• Hodgkins Lymphoma
• Non Hodgkins Lymphoma

13. What’s the difference
Leukemia- from the bone marrow producing
abnormal cells- Blasts
Lymphoma- starts from the immune system
affects lymph nodes and lymphocytes B and T
cells.

14. Bone Marrow
• Present in the soft inner part of some bones such as
the skull, shoulder, blade, ribs, pelvis, and
backbones. (Occupies central cavity of bone)
• The bone marrow is made up of blood-forming stem
cells, lymphoid tissue, fat cells, and supporting
tissues that aid the growth of blood forming cells.

20. Diagnosis of leukemia
Peripheral blood smears
AML ALL

21. Bone marrow Aspirate and trephine
Confirmatory test
Flow cytometer

23. LYMPHOMA

24. What is Lymphoma
• Lymphomas are cancers that begin by the
“malignant transformation” of a lymphocyte
in the lymphatic system
• Many lymphomas are known to be due to
specific genetic mutations

25. What is the Lymphatic System?
• Made up of organs, such as the tonsils, spleen,
liver, bone marrow and a network of lymphatic
vessels that connect glands, called lymph nodes
• Lymph nodes located throughout the body
• Lymph nodes filter foreign particles out of the
lymphatic fluid
• Contain B and T lymphocytes

26. Lymphatic System
• Lymph nodes act as a filter to remove
bacteria, viruses, and foreign particles
• Most people will have had “swollen
glands” at some time as a response to
infection

29. Lymphocytes
• Most lymphocytes are in lymph nodes, spleen,
bone marrow and lymphatic vessels
• 20% of white blood cells in blood are lymphocytes
• T cells, B cells, natural killer cells
• B cells produce antibodies that help fight
infectious agents
• T cells help B cells produce antibodies and they
fight viruses

30. T-Cells and B-Cells
Immature lymphocytes that travel to the
thymus differentiate into T-Cells
– “T” is for thymus
Immature lymphocytes that travel to the
spleen or lymph nodes differentiate into B
cells
– "B" stands for the bursa of Fabricius, which is
an organ unique to birds, where B cells
mature.

31. ALL MM
CLL Lymphomas
Hematopoietic
stem cell
Neutrophils
Eosinophils
Basophils
Monocytes
Platelets
Red cells
Myeloid
progenitor
Myeloproliferative disorders
AML
Lymphoid
progenitor T-lymphocytes
Plasma
cells
B-lymphocytes
naïve

32. B-cell development
stem
cell
lymphoid
progenitor
progenitor-B
pre-B
immature
B-cell
memory
B-cell
plasma cell
DLBCL,
FL, HL
ALL
CLL
MM
germinal
center
B-cell
mature
naive
B-cell

33. Hodgkin
Lymphoma
Non Hodgkin Lymphoma
Highly
Aggressive
Aggressive
Indolent
B cell
Follicular
SLL/CLL
Marginal zone
LP (WM)
T/NK cell
Mycosis fungoides
Sezary syndrome
Primary cut ALCL
B cell
Pre-B
lymphoblastic
Burkitt
T/NK cell
Pre-T
lymphoblastic
B cell
DLBCL
FLg3 and tFL
Mantle cell
Primary effusion
T/NK cell
ALCL
Angioimmunoblastic
Classical
HL
Nodular
lymphocyte
Predominant
(NLPHL)

34. Mechanisms of lymphomagenesis
• Genetic alterations
• Infection
• Antigen stimulation
• Immunosuppression

35. Epidemiology of lymphomas
• males > females
• incidence
– NHL increasing
– Hodgkin lymphoma stable
• in NHL: 3rd most frequently diagnosed cancer
in males and 4th in females
• in HL: 5th most frequently diagnosed cancer in
males and 10th in females

36. Risk factors for NHL
• immunosuppression or immunodeficiency
• connective tissue disease
• family history of lymphoma
• infectious agents
• ionizing radiation

37. Burkitt’s Lymphoma
• Very Aggressive
• Curable with standard-dose therapy but
requires very extensive chemotherapy
protocol
• Translocation t(8,14)
• Specific Hematopathology Finding
– Starry, Starry Night

38. Burkitt’s Lymhoma
Starry, Starry Night

39. Clinical manifestations
• Variable
• severity: asymptomatic to extremely ill
• time course: evolution over weeks, months, or years
• Systemic manifestations
• fever, night sweats, weight loss, anorexia, pruritis
• Local manifestations
• lymphadenopathy, splenomegaly most common
• any tissue potentially can be infiltrated

41. Other complications of lymphoma
• bone marrow failure (infiltration)
• CNS infiltration
• immune hemolysis or thrombocytopenia
• compression of structures (eg spinal cord,
ureters)
• pleural/pericardial effusions, ascites

42. Non-Hodgkin’s Lymphoma
Staging
• Stage is the term used to describe the extent of
tumor that has spread through the body ( I and
II are localized where as III and IV are advanced.
• Each stage is then divided into categories A, B,
and E
–A: No systemic symptoms
–B: Systemic Symptoms such as fever, night sweats
and weight loss
–E: Spreading of disease from lymph node to another
organ

43. Stage I Stage II Stage III Stage IV
Staging of lymphoma
A: absence of B symptoms
B: fever, night sweats, weight loss

44. Symptoms
• Painful Swelling of lymph nodes located in the
neck, underarm and groin.
• Unexplained Fever
• Night Sweats
• Constant Fatigue
• Unexplained Weight loss
• Itchy Skin
Cancer Sourcebook

45. Causes and Risk Factors
• The Exact causes are still unknown
– Higher risk for individuals who:
• Exposed to chemicals such as pesticides or solvents
• Infected w/ Epstein-Barr Virus
• Family history of NHL (although no hereditary pattern
has been established)
• Infected w/ Human Immunodeficiency Virus (HIV)
Lymphoma.org

46. Diagnosis
Staging Studies
• Bone marrow aspiration and biopsy
• Radionuclide scans:
• GI x-rays
• Spinal fluid analysis
• CT scans
• Magnetic Resonance Imaging (MRI)
• Biopsy

47. Treatment Options
• Chemotherapy
• Radiation
• Bone Marrow Transplantation
• Surgery
• Immunotherapy
• Using the bodies own immune system combined with
material made in a lab.

48. Hodgkin lymphoma
Thomas Hodgkin
(1798-1866)

49. Hodgkin lymphoma
• cell of origin: germinal centre B-cell
• Reed-Sternberg cells (or RS variants) in the
affected tissues
• most cells in affected lymph node are
polyclonal reactive lymphoid cells, not
neoplastic cells

50. Reed-Sternberg cell

51. RS cell and variants
popcorn cell
lacunar cell
classic RS cell
(mixed cellularity) (nodular sclerosis) (lymphocyte
predominance)

52. A possible model of pathogenesis
germinal
centre
B cell
transforming
event(s)
loss of apoptosis
RS cell
inflammatory
response
EBV?
cytokines

53. Hodgkin lymphoma
Histologic subtypes
• Classical Hodgkin lymphoma
– nodular sclerosis (most common subtype)
– mixed cellularity
– lymphocyte-rich
– lymphocyte depleted

54. Epidemiology
• less frequent than non-Hodgkin lymphoma
• overall M>F
• peak incidence in 3rd decade

55. Age distribution of new Hodgkin lymphoma
cases
Age (years)
0-1
1-4
5-9
10-14
15-19
20-24
25-29
30-34
35-39
40-44
45-49
50-54
55-59
60-64
65-69
70-74
75-79
80-84
85+
incidence/100,000/annum
0
1
2
3
4
5
6

56. Associated (etiological?) factors
• EBV infection
• smaller family size
• higher socio-economic status
• caucasian > non-caucasian
• possible genetic predisposition
• other: HIV? occupation? herbicides?

57. Clinical manifestations
• lymphadenopathy
• contiguous spread
• extranodal sites relatively uncommon except
in advanced disease
• “B” symptoms

58. Lab Diagnostic Studies
• Lymph node biopsy
• Bone marrow aspiration and biopsy
• Immunohistochemistry
• Flow cytometry
• Molecular Genetic studies
• FISH
• Cytogenetics

59. Treatment and Prognosis
Stage Treatment Failure-
free
survival
Overall 5
year
survival
I,II ABVD x 4
& radiation
70-80% 80-90%
III,IV ABVD x 6 60-70% 70-80%

60. Long term complications of treatment
• infertility
– MOPP > ABVD; males > females
– sperm banking should be discussed
– premature menopause
• secondary malignancy
– skin, AML, lung, MDS, NHL, thyroid, breast...
• cardiac disease

61. Solid Tumors and Abdominal Masses in
Children

62. Introduction
• Abdominal masses are most common in
children younger than 5 years of age.
• In < 5 year group, the older the child
presenting with the mass, the higher the
likelihood of malignancy.
• Often an incidental finding.

63. Most Common Abdominal Solid Tumors
in Childhood
 Lymphomas
 Wilms Tumour
 Neuroblastoma

64.  Pallor plus Bleeding
 Fever / Apathy / Weight Loss (exclude TB, HIV and UTIs)
 Bone Pain (wakes at night, toddler who stops walking, backache)
 Adenopathy (cervical > 2cm, supraclavicular, no regional infection,
negative TB work up, not responding to antibiotics)
 Unexplained Neurological Signs (headache > 2/52, early morning
vomiting, cranial nerve palsies, ataxia, hemiplegia)
 Unexplained Mass (abdominal mass under the age of 5 years is a
tumour until proven otherwise)
 Eye Changes (proptosis, leucocoria, acute onset squint, loss of vision)
What Are The Warning Signs ?

65. Influence of Age on differential Diagnosis
NEONATES INFANTS / TODDLERS
Congenital
Abnormalities
NEONATES INFANTS / TODDLERS
RENAL Mesoblastic nephroma Wilms Tumour
LIVER Mesenchymal Hamartoma
Hepatoblastoma (HBS)
HBS
HCC
ADRENAL Neuroblastoma (NBS) NBS
UROGENITAL Teratomas Germ cell tumors

66. GENERAL EXAMINATION
DYSMORPHOLOGY BWS, Overgrowth syndromes (WT, Hepatoblastoma)
ANTHROPOMETRY Malnourished, immunocompromised, syndromic
PALLOR, BRUISING, PETECHIAE Haematological malignancy, Stage IV neuroblastomas
JAUNDICE Portal tract obstruction by nodes,
NHL
LYMPHADENOPATHY Haematological malignancies
PERI-ORBITAL ECCHYMOSIS Neuroblastoma (NBS)
PROPTOSIS AML or NBS (bilateral), rhabdomyosarcoma or
retinoblastoma (unilateral)
HORNER’S SYNDROME NBS
VARICOCOELE WT
BLOOD PRESSURE WT, NBS, syndromic

67. Abdominal Examination
• Site Central / Flank Lymphoma vsWT
Filling loin/ not WT vs NBS
Unilateral / Bilateral WT
Crossing midline
• Characteristics Size
Consistency
Tender
Smooth / Nodular
Mobile / Fixed
• Associated Ascites / Pleural effusions NHL
findings

68. Initial Imaging for Diagnosis and Referral
How are plain film X rays useful?
AXR
• Confirm Obstruction - lymphoma
• Show calcification - NBS
CXR
• Metastasis
• Mediastinum (nodes and thymus)

69. Initial Imaging For Diagnosis And Referral
ULTRASOUND
 Cheap and readily available.
 No Sedation (usually)
 Good initial imaging
 Doppler studies to assess blood vessel involvement by
tumour – where there is concern regards tumour thrombus (WT, HBS) or
compression by mass (NBS).
Useful to remember:
• Neuroblastoma encases vessels / calcification
• WT displaces vessels, no calcification.
• Check the BP!

70. Further Imaging at Referral Centre
CT CHEST
 Essential to assess chest metastases
best modality.
CT ABDOMEN
 Sedation not such a problem / no
anaesthetic.
 Good imaging of blood vessels.
 Radiation: Implications for follow up
imaging.
 Need for “child friendly” imaging.
MRI
 Best imaging of abdominal
tumours in ideal situation
(abdomen and pelvis).
 Expensive
 Sedation/often anaesthetic
required.
 No radiation: Implications
for initial and follow up
scans.
NEVER let imaging delay the transfer of a child to a treatment centre.
EARLIER DIAGNOSIS = BETTER OUTCOMES!

71. Additional tests
• MIBG SCAN Neuroblastoma
• BONE SCAN Neuroblastoma, Clear cell
sarcoma
• PET / CT (PET ) Hodgkin’s Lymphoma
• BONE MARROW NBS, RMS, lymphomas

72. SOLID TUMORS

73. • RHABDOMYOSARCOMA
– Type of soft tissue sarcoma
– Arising from the primitive mesenchymal cells
Sites
Head and neck
GUT
Extremities

77. Diagnosis
• CT chest
• MRI
• BM
• Others
– FBC
– U&E

78. TREATMENT
• CHEMOTHERAPY
• SURGERY
• RADIOTHERAPY

79. • OSTEOSARCOMA
• Malignant bone tumor
– Environmental factors
• Radiation
– Genetic factors
• Diamond -Blackfan anaemia
• Retinoblastoma
• P53 mutation
– Li-fraumenisyndrome( breast, soft tissue, leukemia, brain and
bone)

82. • TX
• Surgery
• Chemotherapy
• Does not respond to Radiotherapy
• Can be considered for palliative intent for bone pain
management

83. BRAIN TUMORS

84. Classification of CNS tumors
• By location/site
– Supratentorial ( cerebrum)
– Infratentorial/Posterior fossa (cerebellum)
– Spinal cord
• By cell origin
– Astrocytes
– Oligondendrocytes
– Ependymal cell
– Radial glial
– Schwan cells
– Satellite cells
– Enteric glial cells

87. Common childhood CNS tumors
• Pilocytic Astrocytoma
• Medulloblastoma
• Ependymoma
• Pineoblastoma
• ATRT (Atypical Teratoid Rhabdoid Tumor)
• CNS germ cell tumor
• Choroid plexus tumor

94. Approach to management
• Supportive management
– Shunting for hydrocephalus
• Meds Acetazolamide
– Anti epileptic medication
– Anti emetics
Steroids- Dexamethasone
to reduce inflammation and vasogenic edema

95. Approach to management
• Surgery- Main stay of treatment
– Biopsy if the tumor is on important structures
– Complete resection
– Incomplete resection or De-bulking reduction of
the tumor volume,
• More than 50 % advice
Less than 50% high chance of relapse.

96. Approach to treatment
• Chemotherapy
– Not all brain tumors respond to chemotherapy
• Radiotherapy
– On the lesion itself
– Both brain and spine

97. complications
• Neurological concerns
– Seizures
– Cognitive issues
– Decrease school performance
– Loss of other motor skills
All patients with brain tumor require occupation
and physiotherapy and rehabilitation

98. CONCLUSION
• Childhood cancers are treatable
• Better outcome depends on early diagnosis
• Let’s continue to spread awareness of
childhood cancers in the community

99. Thank you
• Questions??

100. RETINOBLASTOMA

101. Retinoblastoma (RB) is the most common childhood intraocular
tumor. However, it is rare,
• accounting for 1% to 3% of all childhood cancers. The tumor
originates in one or both eyes,
• arising from embryonic retinal cells and growing into the
vitreous humor and the sub-retinalspace.
The tumor has a variable growth rate and may have a single or
multiple foci in one
(unilateral) or both eyes (bilateral).
RETINOBLASTOMA

102. 25%-40% percent of retinoblastomas are bilateral and hereditary
(familial)
• approximately 60-75 % are unilateral and occur as a (A – 2) non-
hereditary, spontaneous(sporadic) form.
• The term “familial” or “hereditary” is commonly used for bilateral
retinoblastoma.
However, only 25% of children with bilateral disease have family history
of retinoblastoma.
The other 75% usually acquire the mutation of the RB1 gene in utero, in
the absence of a family history of this cancer

103. Bilateral RB is often diagnosed at an early age (< 1 year old), and is
often hereditary, while unilateral RB is commonly diagnosed during
the toddler years.
In rare cases, RB can present as trilateral disease involving the pineal
gland.
• Trilateral retinoblastoma is a pineal tumor typically appearing
approximately 3-5 years after diagnosis of bilateral RB. It is often
associated with high mortality.

104. The retinoblastoma gene (RB1) is located on band 14 of chromosome
13.
• RB1 is a tumor suppressor gene (acts as a brake on the cell division
cycle to prevent uncontrolled cell division).
The loss of RB1 causes unregulated cell proliferation and tumor
development.
Abnormalities in the retinoblastoma gene not associated with RB are
very common and occur in many types of malignancies.
RISK FACTORS

108. Common clinical signs
Leukocoria:“cat’s eye reflex,” “white eyes,” white
pupil, the most common presentation
Strabismus: esotropia (eye turning in) and extropia
(eyes turning out)
Decreased vision: especially if only in one eye
(unilateral)
Painful eyes
Erythmatous conjunctivae

109. Diagnostic work up options
Complete history of illness including familial incidence of
retinoblastoma,
• ocular loss of unknown etiology,
• decreased vision in one eye, changes in the appearance
of eyes.
• The child may be bumping into things because he does
not see them.
Physical exam assesses visual acuity and tracking,
strabismus, esotropia, exotropia, and
leukocoria
Funduscopic exam (direct or indirect) is done under
anesthesia by a ophthalmologist

110. Treatment options
1.Surgical enucleation: Complete removal of the eye is recommended
when there is no vision
2. Systemic chemotherapy
3. Local therapy
• Cryotherapy: A freezing process that kills tumor cells is used
mostly for tumors in the anterior retina
• Thermotherapy: The use of heat from a laser to destroy the cancer
cells;
the heat can also improve the efficacy of chemotherapy or
radiotherapy.

111. Radiation therapy (RT):
• External Beam RT: Used in multifocal advanced disease, usually after
chemotherapy and focal treatments. Lateral or anterior fields are used.
• Brachytherapy (Radioactive applicators - Plaques): Used to treat
individual tumors that are too large for treatment with cryotherapy,
laser or thermotherapy, usually after chemotherapy