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AAA-PEDIATRICS Y3S2 > CHILDHOOD MALIGNANCIES 2 (SB) > Flashcards

CHILDHOOD MALIGNANCIES 2 (SB) Flashcards

1
Q

What percentage of childhood cancers is leukemia responsible for according to DOH and India statistics?

A

47.8%

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2
Q

How are leukemias categorized based on cell maturity?

A

Acute (immature/blastic cells) and Chronic (mature differentiated cells)

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3
Q

What defines leukemia as a disease?

A

A group of malignant diseases where genetic abnormalities in hematopoietic cells cause unregulated clonal proliferation, leading to marrow failure.

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4
Q

What symptoms may indicate leukemia?

A

Pallor, easy bruisability, malaise, anorexia, intermittent fever, bone pains, abdominal pain, or bleeding

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5
Q

What causes the overgrowth in acute leukemia?

A

Overgrowth of blood cell precursors and maturational arrest at a specific stage of hematopoiesis

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6
Q

What is the most common childhood malignancy under 15 years old?

A

Acute leukemia

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7
Q

According to DOH PhilHealth (2005), what rank is leukemia in causes of mortality?

A

5th overall

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8
Q

What does GM-CSF promote?

A

Myeloid cell development, maturation, and dendritic cell differentiation

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9
Q

What cells arise from the myeloid series?

A

Erythrocytes, megakaryocytes, eosinophils, basophils, neutrophils

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10
Q

What cells arise from the lymphoid series?

A

B cells, T cells, and natural killer cells

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11
Q

What is seen in leukemic marrow?

A

Abnormal proliferation of immature cells, leading to decreased RBCs and platelets

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12
Q

What replaces marrow in ALL?

A

Undifferentiated lymphoblasts

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13
Q

How much blast presence in peripheral smear suggests ALL?

A

> 5% nucleated marrow cells

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14
Q

Which group classified ALL immunophenotypes?

A

French-American-British (FAB) Cooperative Working Group

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15
Q

What percentage of ALL cases are B-lymphoblastic leukemia?

A

0.85

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16
Q

What percentage of ALL cases are T-lymphoblastic leukemia?

A

0.15

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17
Q

What is Burkitt leukemia?

A

A rare, fast-growing mature B-cell leukemia requiring a different therapeutic approach

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18
Q

What are common constitutional symptoms of leukemia?

A

Pallor, bruising, petechiae, bleeding, fever, pain, lymphadenopathy, hepatosplenomegaly

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19
Q

What causes bone pain in leukemia?

A

Stretching of periosteum or joint capsule by leukemic infiltration

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20
Q

What blood abnormalities are seen in leukemia?

A

Anemia, leukocytosis, thrombocytopenia, low reticulocyte count

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21
Q

What is seen in peripheral smear in leukemia?

A

Normocytic, hypochromic RBCs, normal platelet size, presence of blast cells

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22
Q

What is the gold standard for leukemia diagnosis?

A

Bone marrow aspiration biopsy

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23
Q

What indicates leukemia in bone marrow?

A

Hypercellularity with >25% blast cells

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24
Q

When is lumbar puncture done in leukemia?

A

With first dose of intrathecal chemo if diagnosis confirmed from bone marrow

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25
Which age group has better prognosis in leukemia?
1-10 years old
26
What leukocyte count is associated with poor prognosis?
Higher leukocyte count
27
What immunophenotype is linked to poor outcomes?
B-cell, T-cell, and natural killer cell lineage
28
What CNS condition at diagnosis indicates poor prognosis?
Overt CNS disease
29
What treatment response indicates good prognosis?
Early response to induction chemotherapy
30
What is the most important prognostic factor in ALL?
Effective treatment
31
What does risk-directed therapy in ALL consider?
Age, WBC count, immunophenotype, cytogenetics, early response, MRD
32
What is the goal of remission induction phase in ALL?
Reduce leukemic burden to undetectable level
33
What defines hematologic remission?
<5% blasts in marrow, no lymphoblasts in PS, granulocytes >500–1000/mm3, platelets >100,000/mm3
34
What does complete remission in ALL require?
Hematologic remission plus absence of extramedullary leukemia
35
What therapies are used for CNS-directed treatment in ALL?
Cranial irradiation, intrathecal chemo, oral meds
36
What is the purpose of intensification phase?
Further reduce disease burden, adjust treatment intensity
37
What is the duration and nature of maintenance phase?
Longest phase, less intensive chemo for 2–3 years
38
What is the 2nd most common childhood malignancy?
Brain tumors
39
What is the overall mortality of childhood brain tumors?
About 30%
40
What % of brain tumors are infratentorial in SEER data?
0.43200000000000005
41
What % of brain tumors are supratentorial in SEER data?
0.409
42
What are the common pediatric brain tumors?
Pilocytic astrocytomas and medulloblastomas/PNETs
43
What are the common adolescent brain tumors?
Pituitary and craniopharyngeal tumors, and PAs
44
Where do supratentorial tumors arise from?
Cerebral hemispheres, basal ganglia, thalamus, ventricles, hypothalamus, corpus callosum
45
Where do infratentorial tumors arise from?
Cerebellum, brainstem, fourth ventricle
46
What triad is associated with infratentorial tumors?
Headache, nausea/vomiting, papilledema
47
What symptoms suggest brainstem tumors?
Gaze palsy, cranial nerve palsies, hemiparesis, hyperreflexia, clonus
48
What is diencephalic syndrome of Russell?
Failure to thrive despite normal intake, emaciation, inappropriate affect
49
What imaging modality is preferred for brain tumors?
MRI with gadolinium contrast
50
When are neuroendocrine function tests indicated?
For midline tumors like pituitary, suprasellar, optic chiasmal
51
What serum/CSF markers suggest germ cell tumors?
Beta-HCG and alpha-fetoprotein (AFP)
52
What tumors require CSF cyto-analysis?
Medulloblastoma, PNET, ependymoma, germ cell tumors
53
What is the primary treatment for brain tumors?
Surgical resection
54
What is the role of radiation therapy in brain tumors?
Depends on tumor physiology, not all are radiosensitive
55
Why is supportive care important after brain tumor surgery?
To manage chronic neurologic deficits due to tissue damage
56
What is lymphoma?
A malignancy of the lymphatic system that presents as enlarged lymph nodes.
57
What is the most common malignancy in adolescence?
Lymphoma.
58
What percentage of newly diagnosed malignancies in 15-19 y/o is lymphoma?
More than 25%.
59
What are the two main categories of lymphoma?
Hodgkin’s lymphoma (HL) and Non-Hodgkin’s lymphoma (NHL).
60
What virus is associated with Reed-Sternberg cells?
Epstein-Barr virus (EBV).
61
What system does Hodgkin's lymphoma involve?
The lymphoreticular system.
62
What is the age distribution peak for HL?
15-35 years and after 50 years.
63
What infectious agents are associated with HL?
Epstein-Barr virus, Human herpes virus 6, Cytomegalovirus.
64
What is the pathognomonic feature of HL?
Reed-Sternberg cell.
65
Describe a Reed-Sternberg cell.
Large cell with multiple or multi-lobulated nuclei.
66
Where is the specimen taken from to diagnose HL?
The lymph node.
67
What are clinical manifestations of HL?
Painless, firm, rubbery enlarged lymph nodes.
68
What symptoms may HL cause if the mediastinum is affected?
Airway compression, chest pain, superior vena cava syndrome.
69
What are B symptoms in HL?
Unexplained fever, weight loss, and night sweats.
70
What causes the systemic symptoms in HL?
Substances (IL, TNF, TGF) secreted by the Reed-Sternberg cell.
71
What lymph nodes are usually affected in HL?
Cervical, mediastinal, abdominal.
72
How is HL diagnosed?
By excision or FNA biopsy of a lymph node, immunophenotyping, and imaging.
73
What staging system is used for HL?
Ann Arbor classification.
74
What are the treatment options for HL?
Chemotherapy, radiation therapy, or both.
75
What is Stage I in the Ann Arbor classification?
Involvement of a single lymph node or a single extralymphatic site.
76
What is Stage II in the Ann Arbor classification?
Involvement of 2+ lymph node regions on the same side of the diaphragm or with localized extralymphatic involvement.
77
What is Stage III in the Ann Arbor classification?
Lymph node regions on both sides of the diaphragm, possibly with spleen or organ involvement.
78
What is Stage IV in the Ann Arbor classification?
Diffuse/disseminated involvement of one or more extralymphatic organs with or without lymph node involvement.
79
What does the suffix A or B indicate in Ann Arbor staging?
A: absence of B symptoms; B: presence of fever, night sweats, or >10% weight loss.
80
What percentage of childhood lymphomas are NHL?
About 60%.
81
What is the 2nd most common malignancy in ages 15–35?
Non-Hodgkin's lymphoma.
82
Describe the nature of pediatric NHL.
Usually high-grade and aggressive but has a good prognosis.
83
What does de novo disease mean in NHL?
Genetic alteration not inherited but occurs spontaneously.
84
What are other etiologies of NHL?
Inherited immune deficiencies, HIV, EBV, genetic syndromes (e.g., Bloom syndrome, ataxia-telangiectasia).
85
Name pathologic subtypes of NHL.
Lymphoblastic lymphoma, Burkitt’s lymphoma, Diffuse large B-cell lymphoma, Anaplastic large cell lymphoma.
86
What staging system is used for pediatric NHL?
St. Jude staging system.
87
How is NHL treated?
Multi-agent systemic chemotherapy with intrathecal chemo, surgery (diagnosis), radiation (in select cases).
88
What is Stage I in St. Jude classification?
Single tumor or nodal area excluding mediastinum/abdomen.
89
What is Stage II in St. Jude classification?
Tumor with regional nodes, 2+ nodal areas on same diaphragm side, GI tumor grossly resected.
90
What is Stage III in St. Jude classification?
Tumors/nodes on opposite diaphragm sides, intrathoracic/intraabdominal disease.
91
What is Stage IV in St. Jude classification?
CNS or bone marrow involvement at diagnosis.

AAA-PEDIATRICS Y3S2 (55 decks)

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