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Common Pediatric Solid
Tumors
Abdullatiff Sami Al-Rashed
College of Medicine, King Faisal University
Al-Ahsa, Saudi Arabia
Objectives
• Definition.
• Epidemiology.
• Etiology.
• Types.
• Symptoms &
Signs.
• Staging.
• Diagnosis.
• Treatment.
• Prognosis.
• Definition.
• Epidemiology.
• Etiology.
• Types.
• Symptoms &
Signs.
• Staging.
• Diagnosis.
• Treatment.
• Prognosis.
• Definition.
• Treatment.
Lymphoma
(Hodgkin's & Non-Hodgkin's)
Lymphoma
(Hodgkin's & Non-Hodgkin's)
Definition
• Neoplasia of the lymphatic system and its precursor cells
with genetically distrusted regulation of proliferation,
differentiation and apoptosis.
• Characterized by painless, progressive enlargement of
the lymph nodes with continues extension between
lymph node regions.
Epidemiology
• Lymphoma accounts 10% of all pediatric neoplasia.
Hodgkin's lymphoma Non-Hodgkin's lymphoma
7 in 1 million children < age
of 16 newly diagnosed with
HL annually
Ratio of boys to girls 2:1
Boys > Girls , but during
adolescence the incidence
is the same
Peak incidence between 5
to 15 years old
Peak incidence between 15
to 35 years old
Etiology
• Unknown etiology in humans.
• It has been found that there is
correlation with infection “e.g
Epstein-barr Virus”.
• Correlation with
Socioeconomic status: the
higher SES the more
frequently HL occur.
• Genetic causes.
• Unknown etiology in humans.
• Predisposing factors include:
– Autoimmune disorders.
– Infections ( HIV, EBV, HTLV-1 )
– Congenital B-Cell defect.
– Congenital T-Cell defect.
– Drug induced
(immunosuppressive treatment)
HL NHL
Types
Lymphoma
Hodgkin's
lymphoma
Classical
Hodgkin’s
lymphoma (90-
95%).
Nodular
lymphocyte
predominant HL
Non Hodgkin's
lymphoma
Diffuse large B-
cell lymphoma
(30%)
Peripheral T-call
lymphoma
Burkitt
Lymphoma
(Highly
aggressive)
Other types
Symptoms & Signs
Painless enlarged
lymph nodes
(commonest
cervical)
Symptoms & Signs
Staging
Diagnosis
Treatment
• Treatment plan is assigned based on
the type and stage of lymphoma.
• Traditionally, management consists of
a combination of chemotherapy
drugs.
• Combination of radiotherapy and
chemotherapy can be used in
Hodgkin's lymphoma.
Prognosis
Nephroblastoma
(Wilms tumor)
Nephroblastoma
(Wilms tumor)
Definition
• Malignant embryonal tumor of renal tissue.
• First described in 1899 by max wilms.
Epidemiology
Etiology
• Wilms tumor has been associated with loss of function mutations of
a number of tumor suppressor and transcription genes.
• These include mutations of the WT1, p53, FWT1, and FWT2 genes,
and at the 11p15.5 locus.
• Associated with some congenital syndromes. These syndromes
include:
– WAGR syndrome.
– Denys-Drash syndrome.
– Beckwith-Wiedemann syndrome.
Types
• Histologically, the classic favorable histology Wilms
tumor is comprised of three cell types:
Symptoms & Signs
Staging
Diagnosis
Diagnosis
THE DEFINITIVE DIAGNOSIS OF WILMS
TUMOR IS MADE BY HISTOLOGIC
CONFIRMATION AT THE TIME OF EITHER
SURGICAL EXCISION OR BIOPSY.
Treatment
• Treatment plan is assigned based on results of the initial
staging, histological, and molecular studies.
• In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy
Prognosis
NeuroblastomaNeuroblastoma
Definition
• Malignant embyronal tumor of precursor cells of sympathetic
ganglia and adrenal medulla.
• Commonly used to refer to a spectrum of neuroblastic tumors
(including neuroblastomas, ganglioneuroblastomas, and
ganglioneuromas)
Epidemiology
Etiology
• Etiology is unknown.
 Risk factors:
• Maternal factors: These include the following:
– Opiate consumption
– Folate deficiency
– Toxic exposures
– Congenital abnormalities
– Gestational diabetes mellitus
• Genetic factors:
– A higher incidence of neuroblastoma has been suggested in girls with Turner
syndrome, Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1
(NF1)
Types
• Neuroblastomas can arise anywhere throughout the
sympathetic nervous system.
• The common primary sites:
Adrenal gland
(40%)
Abdominal
(25%)
Thoracic
(15%)
Types
Symptoms & Signs
Staging
Diagnosis
Diagnosis
• Diagnostic criteria — Minimum criteria for establishing a diagnosis
of neuroblastoma have been agreed upon by an international
consensus panel. A definitive diagnosis of neuroblastoma requires
one of the following:
– An unequivocal histologic diagnosis from tumor tissue by light microscopy, with
or without immunohistochemistry, electron microscopy, or increased urine (or
serum) catecholamines or their metabolites.
– Evidence of metastases to bone marrow on an aspirate or trephine
biopsy with concomitant elevation of urinary or serum catecholamines or their
metabolites.
Treatment
• Treatment plan is assigned based on:
– Stage of the disease.
– Patient age.
– Histologic appearance of the tumor.
– Presence or absence of amplification of the MYCN oncogene.
– Quantitative DNA content of the tumor (DNA index or ploidy).
Treatment
•In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy
Prognosis
hepatoblastomahepatoblastoma
Definition
• Hepatoblastoma is the most common primary
hepatic malignancy in early childhood.
• The majority of hepatoblastomas occur in the
first two years of life and rarely in children older
than five years.
Epidemiology
• One percent of all pediatric neoplasias
• The incidence of hepatoblastoma in boys is
twice that in girls.
Etiology
• Exact etiology is unknown.
• Syndromes with an increased incidence of
hepatoblastoma include:
– Beckwith Wiedmann syndrome.
– Trisomy 18 & 21.
– Acardia syndrome.
– Li-Fraumeni syndrome.
– Goldenhar syndrome (a type of craniofacial microsomia).
– Type 1a glycogen storage disease (von Gierke’s disease).
– Familial adenomatous polyposis (FAP).
Types
Symptoms & Signs
Staging
Stage Characteristics
Stage I • The tumor is completely resectable via
wedge resection or lobectomy.
• The tumor has PFH results.
• The AFP level is within reference range
within 4 weeks of surgery.
Stage IIA • The tumor is completely resectable.
• The tumor has histologic results other than
PFH (UH).
Stage IIB • The tumor is completely resectable.
• AFP findings are negative at time of diagnosis
(ie, no marker to follow).
Stage III (any of the following) • The tumor is initially unresectable but is
confined to one lobe of liver.
• Gross residual disease is present after
surgery.
• Tumor ruptures or spills preoperatively or
intraoperatively.
• Regional lymph nodes are involved.
Stage IV Distant bone or lung metastasis is present.
Diagnosis
Treatment
• Treatment plan is assigned based on results of the initial
staging, histological, and molecular studies.
• In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy
Prognosis
HemangiomaHemangioma
Definition
• Infantile hemangiomas are benign vascular neoplasms
that have a characteristic clinical course marked by early
proliferation and followed by spontaneous involution.
• Hemangiomas are the most common tumors of infancy
and usually are medically insignificant.
Treatment
• The vast majority of infantile hemangiomas do not
require any medical or surgical intervention.
• Intervention may be required for lesions with potential to
interfere with a vital structure or function. These include,
but are not limited to:
– Lesions in the airway, liver, or gastrointestinal tract.
– Lesions in the periorbital region.
– Very large, rapidly growing cutaneous hemangiomas.
Treatment
• Treatment choices are:
– Drugs: Steroids and Beta-Blocker
– Surgery: laser surgery or surgical excesion
References
Common Pediatric Solid Tumors

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Common Pediatric Solid Tumors

  • 1. Common Pediatric Solid Tumors Abdullatiff Sami Al-Rashed College of Medicine, King Faisal University Al-Ahsa, Saudi Arabia
  • 2.
  • 3. Objectives • Definition. • Epidemiology. • Etiology. • Types. • Symptoms & Signs. • Staging. • Diagnosis. • Treatment. • Prognosis. • Definition. • Epidemiology. • Etiology. • Types. • Symptoms & Signs. • Staging. • Diagnosis. • Treatment. • Prognosis. • Definition. • Treatment.
  • 5. Definition • Neoplasia of the lymphatic system and its precursor cells with genetically distrusted regulation of proliferation, differentiation and apoptosis. • Characterized by painless, progressive enlargement of the lymph nodes with continues extension between lymph node regions.
  • 6. Epidemiology • Lymphoma accounts 10% of all pediatric neoplasia. Hodgkin's lymphoma Non-Hodgkin's lymphoma 7 in 1 million children < age of 16 newly diagnosed with HL annually Ratio of boys to girls 2:1 Boys > Girls , but during adolescence the incidence is the same Peak incidence between 5 to 15 years old Peak incidence between 15 to 35 years old
  • 7. Etiology • Unknown etiology in humans. • It has been found that there is correlation with infection “e.g Epstein-barr Virus”. • Correlation with Socioeconomic status: the higher SES the more frequently HL occur. • Genetic causes. • Unknown etiology in humans. • Predisposing factors include: – Autoimmune disorders. – Infections ( HIV, EBV, HTLV-1 ) – Congenital B-Cell defect. – Congenital T-Cell defect. – Drug induced (immunosuppressive treatment) HL NHL
  • 8. Types Lymphoma Hodgkin's lymphoma Classical Hodgkin’s lymphoma (90- 95%). Nodular lymphocyte predominant HL Non Hodgkin's lymphoma Diffuse large B- cell lymphoma (30%) Peripheral T-call lymphoma Burkitt Lymphoma (Highly aggressive) Other types
  • 9. Symptoms & Signs Painless enlarged lymph nodes (commonest cervical)
  • 13. Treatment • Treatment plan is assigned based on the type and stage of lymphoma. • Traditionally, management consists of a combination of chemotherapy drugs. • Combination of radiotherapy and chemotherapy can be used in Hodgkin's lymphoma.
  • 16. Definition • Malignant embryonal tumor of renal tissue. • First described in 1899 by max wilms.
  • 18. Etiology • Wilms tumor has been associated with loss of function mutations of a number of tumor suppressor and transcription genes. • These include mutations of the WT1, p53, FWT1, and FWT2 genes, and at the 11p15.5 locus. • Associated with some congenital syndromes. These syndromes include: – WAGR syndrome. – Denys-Drash syndrome. – Beckwith-Wiedemann syndrome.
  • 19. Types • Histologically, the classic favorable histology Wilms tumor is comprised of three cell types:
  • 23. Diagnosis THE DEFINITIVE DIAGNOSIS OF WILMS TUMOR IS MADE BY HISTOLOGIC CONFIRMATION AT THE TIME OF EITHER SURGICAL EXCISION OR BIOPSY.
  • 24. Treatment • Treatment plan is assigned based on results of the initial staging, histological, and molecular studies. • In General , lines of treatment include: Surgery Chemotherapy Radiotherapy
  • 27. Definition • Malignant embyronal tumor of precursor cells of sympathetic ganglia and adrenal medulla. • Commonly used to refer to a spectrum of neuroblastic tumors (including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas)
  • 29. Etiology • Etiology is unknown.  Risk factors: • Maternal factors: These include the following: – Opiate consumption – Folate deficiency – Toxic exposures – Congenital abnormalities – Gestational diabetes mellitus • Genetic factors: – A higher incidence of neuroblastoma has been suggested in girls with Turner syndrome, Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1 (NF1)
  • 30. Types • Neuroblastomas can arise anywhere throughout the sympathetic nervous system. • The common primary sites: Adrenal gland (40%) Abdominal (25%) Thoracic (15%)
  • 31. Types
  • 35. Diagnosis • Diagnostic criteria — Minimum criteria for establishing a diagnosis of neuroblastoma have been agreed upon by an international consensus panel. A definitive diagnosis of neuroblastoma requires one of the following: – An unequivocal histologic diagnosis from tumor tissue by light microscopy, with or without immunohistochemistry, electron microscopy, or increased urine (or serum) catecholamines or their metabolites. – Evidence of metastases to bone marrow on an aspirate or trephine biopsy with concomitant elevation of urinary or serum catecholamines or their metabolites.
  • 36. Treatment • Treatment plan is assigned based on: – Stage of the disease. – Patient age. – Histologic appearance of the tumor. – Presence or absence of amplification of the MYCN oncogene. – Quantitative DNA content of the tumor (DNA index or ploidy).
  • 37. Treatment •In General , lines of treatment include: Surgery Chemotherapy Radiotherapy
  • 40. Definition • Hepatoblastoma is the most common primary hepatic malignancy in early childhood. • The majority of hepatoblastomas occur in the first two years of life and rarely in children older than five years.
  • 41. Epidemiology • One percent of all pediatric neoplasias • The incidence of hepatoblastoma in boys is twice that in girls.
  • 42. Etiology • Exact etiology is unknown. • Syndromes with an increased incidence of hepatoblastoma include: – Beckwith Wiedmann syndrome. – Trisomy 18 & 21. – Acardia syndrome. – Li-Fraumeni syndrome. – Goldenhar syndrome (a type of craniofacial microsomia). – Type 1a glycogen storage disease (von Gierke’s disease). – Familial adenomatous polyposis (FAP).
  • 43. Types
  • 45. Staging Stage Characteristics Stage I • The tumor is completely resectable via wedge resection or lobectomy. • The tumor has PFH results. • The AFP level is within reference range within 4 weeks of surgery. Stage IIA • The tumor is completely resectable. • The tumor has histologic results other than PFH (UH). Stage IIB • The tumor is completely resectable. • AFP findings are negative at time of diagnosis (ie, no marker to follow). Stage III (any of the following) • The tumor is initially unresectable but is confined to one lobe of liver. • Gross residual disease is present after surgery. • Tumor ruptures or spills preoperatively or intraoperatively. • Regional lymph nodes are involved. Stage IV Distant bone or lung metastasis is present.
  • 47. Treatment • Treatment plan is assigned based on results of the initial staging, histological, and molecular studies. • In General , lines of treatment include: Surgery Chemotherapy Radiotherapy
  • 50. Definition • Infantile hemangiomas are benign vascular neoplasms that have a characteristic clinical course marked by early proliferation and followed by spontaneous involution. • Hemangiomas are the most common tumors of infancy and usually are medically insignificant.
  • 51. Treatment • The vast majority of infantile hemangiomas do not require any medical or surgical intervention. • Intervention may be required for lesions with potential to interfere with a vital structure or function. These include, but are not limited to: – Lesions in the airway, liver, or gastrointestinal tract. – Lesions in the periorbital region. – Very large, rapidly growing cutaneous hemangiomas.
  • 52. Treatment • Treatment choices are: – Drugs: Steroids and Beta-Blocker – Surgery: laser surgery or surgical excesion

Editor's Notes

  1. The adrenal gland is the most common primary site (40 percent), followed by abdominal (25 percent), thoracic (15 percent), cervical (5 percent), and pelvic sympathetic ganglia (5 percent) 
  2. stage I The tumor is completely resectable via wedge resection or lobectomy. The tumor has PFH results. The AFP level is within reference range within 4 weeks of surgery. Stage IIA The tumor is completely resectable. The tumor has histologic results other than PFH (UH). Stage IIB The tumor is completely resectable. AFP findings are negative at time of diagnosis (ie, no marker to follow). Stage IIC The tumor is completely resected or rendered completely resectable by initial radiotherapy or chemotherapy or microscopic residual disease is present. The AFP level is elevated 4 weeks after resection. Stage III (any of the following) The tumor is initially unresectable but is confined to one lobe of liver. Gross residual disease is present after surgery. Tumor ruptures or spills preoperatively or intraoperatively. Regional lymph nodes are involved. Stage IV: Distant bone or lung metastasis is present.