Pediatric tumors By Maya Al-Homouz
➢ Types of tumors ?! Malignant, Nonmalignant
➢ In what does the pediatric & adult tumors differ from each other ?! prognosis , distribution by histology and
tumor site
➢ What is the Most common pediatric malignancy?! Acute leukemia
Leukemia:
➢ Definition ?! Group of malignant diseases where genetic abnormalities in a hematopoietic cells resulting in
unregulated clonal proliferation of cells.
➢ Types of leukemia?!
- Acute lymphoblastic leukemia (ALL) "MC"
- Acute myelogenous leukemia (AML)
- Chronic myelogenous leukemia (CML)
- Juvenile myelomonocytic leukemia
Acute lymphoblastic leukemia (ALL) :
• Peak age?! 2-3 years
• Common in ?! M > F, Chromosomal abnormalities (Down ,Bloom Ataxia, Telangiectasia, Fanconi syndromes)
• Etiology?! Unknown, several genetics & environmental factors(chemical, drugs, radiation)
• Classified into:
- Pre-B ALL "MC"
- T-Cell ALL 15%
- Mature B (Burkitt) ALL
• What are the Clinical Manifestation of ALL?!
Initial symptoms are nonspecific and relatively brief:
• Anorexia, fatigue, malaise, irritability(anemia)
• low-grade fever
• Sever bone Or joint pain "mainly in the lower extremities"(thrombocytopenia)
## Hx of an upper respiratory tract infection in the preceding 1-2 month(leukopenia)
Later signs and symptoms of bone marrow failure:
• Organ infiltration( lymphadenopathy, hepatosplenomegaly, testicular enlargement, CNS
involvement
• Respiratory distress (due to?! severe anemia OR mediastinal node comparison of the airways)
• What are the PE findings in ALL?!
- Pallor, restlessness
- Purpuric and petechial skin lesions, mucous membrane
- Lymphadenopathy, splenomegaly, Less commonly hepatomegaly.
- Tenderness over the bone (In Pt with bone pain)
- Papilledema (in CNS involvement )
- Testicular mass "rare"
- Respiratory distress (in T-cell with large anterior mediastinal mass)
• How to Diagnose ALL?
CBC (severe anemia and thrombocytopenia & High WBC), Blood film( peripheral blasts "enough to diagnose
leukemia "), Bone marrow aspiration /biopsy, flow cytometry(to differentiate if t-cell/b-cell/AML), cytogenetics,
molecular studies, LP and CSF examination for blasts, CXR( mediastinal mass), High lactate dehydrogenase (LDH)
*(High LDH +Anemia +Thrombocytopenia= "clue for malignancy")
• Mx of ALL?!
## Treatment protocol according to risk stratification (low risk, intermediate vs high risk)
- Several chemotherapeutic agents
- Corticosteroids(1st one week of ttt) , vincristine(SE?! peripheral neuropathy), methotrexate (SE?! pancreatitis),
daunomycin(SE?! decrease ejection fraction), Mercaptopurine(SE?! elevates liver enzyme) and methotrexate oral
(maintenance therapy)
## Several phases= Remission induction(clear bone marrow from blast cells by intrathecal chemotherapy),
consolidation and intensification, maintenance phase
- What are the Good prognostic factors?!
- Age 1-10 year.
- WBC < 50,000
- Chromosomal Abnormalities: presence of TEL/AML1 gene, Philadelphia negative , hyperdiploidy, absence of MLL
rearrangement
- Immunophenotype: B-cell ALL
- No CNS involvement
- Early Response to Chemotherapy
Pediatrics Page 1
➢ Types of tumors ?! Malignant, Nonmalignant
➢ In what does the pediatric & adult tumors differ from each other ?! prognosis , distribution by histology and
tumor site
➢ What is the Most common pediatric malignancy?! Acute leukemia
Leukemia:
➢ Definition ?! Group of malignant diseases where genetic abnormalities in a hematopoietic cells resulting in
unregulated clonal proliferation of cells.
➢ Types of leukemia?!
- Acute lymphoblastic leukemia (ALL) "MC"
- Acute myelogenous leukemia (AML)
- Chronic myelogenous leukemia (CML)
- Juvenile myelomonocytic leukemia
Acute lymphoblastic leukemia (ALL) :
• Peak age?! 2-3 years
• Common in ?! M > F, Chromosomal abnormalities (Down ,Bloom Ataxia, Telangiectasia, Fanconi syndromes)
• Etiology?! Unknown, several genetics & environmental factors(chemical, drugs, radiation)
• Classified into:
- Pre-B ALL "MC"
- T-Cell ALL 15%
- Mature B (Burkitt) ALL
• What are the Clinical Manifestation of ALL?!
Initial symptoms are nonspecific and relatively brief:
• Anorexia, fatigue, malaise, irritability(anemia)
• low-grade fever
• Sever bone Or joint pain "mainly in the lower extremities"(thrombocytopenia)
## Hx of an upper respiratory tract infection in the preceding 1-2 month(leukopenia)
Later signs and symptoms of bone marrow failure:
• Organ infiltration( lymphadenopathy, hepatosplenomegaly, testicular enlargement, CNS
involvement
• Respiratory distress (due to?! severe anemia OR mediastinal node comparison of the airways)
• What are the PE findings in ALL?!
- Pallor, restlessness
- Purpuric and petechial skin lesions, mucous membrane
- Lymphadenopathy, splenomegaly, Less commonly hepatomegaly.
- Tenderness over the bone (In Pt with bone pain)
- Papilledema (in CNS involvement )
- Testicular mass "rare"
- Respiratory distress (in T-cell with large anterior mediastinal mass)
• How to Diagnose ALL?
CBC (severe anemia and thrombocytopenia & High WBC), Blood film( peripheral blasts "enough to diagnose
leukemia "), Bone marrow aspiration /biopsy, flow cytometry(to differentiate if t-cell/b-cell/AML), cytogenetics,
molecular studies, LP and CSF examination for blasts, CXR( mediastinal mass), High lactate dehydrogenase (LDH)
*(High LDH +Anemia +Thrombocytopenia= "clue for malignancy")
• Mx of ALL?!
## Treatment protocol according to risk stratification (low risk, intermediate vs high risk)
- Several chemotherapeutic agents
- Corticosteroids(1st one week of ttt) , vincristine(SE?! peripheral neuropathy), methotrexate (SE?! pancreatitis),
daunomycin(SE?! decrease ejection fraction), Mercaptopurine(SE?! elevates liver enzyme) and methotrexate oral
(maintenance therapy)
## Several phases= Remission induction(clear bone marrow from blast cells by intrathecal chemotherapy),
consolidation and intensification, maintenance phase
- What are the Good prognostic factors?!
- Age 1-10 year.
- WBC < 50,000
- Chromosomal Abnormalities: presence of TEL/AML1 gene, Philadelphia negative , hyperdiploidy, absence of MLL
rearrangement
- Immunophenotype: B-cell ALL
- No CNS involvement
- Early Response to Chemotherapy
Pediatrics Page 1
