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26 Cards in this Set
- Front
- Back
The majority of childhood cancers have what cell type of origin?
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embryonic cells such as neuroblastoma or retinoblastoma
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What congenital syndromes predispose a child to cancer?
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Down's
Beckwith-Wiedemann ataxia-telangiectasia neurofibromatosis Li-Fraumeni syndrome familial polyposis coli Bloom syndrome |
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What are the most common cancer types in children? (3)
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Leukemia (25%)
CNS tumors (17%) Lymphomas (16%) |
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What age group(s) of children has the highest incidence of cancer?
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0-4yrs -- usually attrib to genetic component
15-19yrs -- puberty begins; reason for ↑ incidence at this age is largely unknown |
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Which cancer age group has the poorest outcome?
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adolescents and young adults have a poorer outcome than younger children and older adults
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Common signs and symptoms of leukemia?
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pallor, fatigue, fever
bruises, bleeding lymphadenopathy bone pain, limping hepatomegaly splenomegaly |
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Common signs and symptoms of brain tumor?
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headache
morning vomiting May have CSF blockage, ataxia, focal neural deficits ± hemiparesis, papilledema |
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Common signs and symptoms of lymphoma?
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First presentation usually cervical lymphadenopathy
hepatomegaly splenomegaly bone pain, limping (bone lymphoma) may have painless mass, symptoms of compression, fever, night sweats, wt loss |
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Childhood cancer predisposition due to chromosome abnormalities?
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Trisomy 21:
-- ANLL M7 (<2yrs) -- ALL (x20) Turner's & Klinefelter's Syndrome -- gonadoblastoma -- dysgerminoma -- extragonidal |
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Childhood cancer predisposition due to autosomal dominant conditions?
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Rb gene mutation:
-- retinoblastoma -- osteosarcoma -- melanoma p53 gene mutation: -- adrenal carcinoma -- brain tumors -- sarcomas -- breast cancer -- leukemias Multiple endocrine neoplasia: -- thyroid carcinomas -- pheochromocytomas |
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What is the key to early detection of childhood cancer?
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"high index of suspicion"
-- educate parents -- thorough exam ex: red reflex in infant for retinoblastoma |
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Indicators of rhabdomyosarcoma?
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loss of heterozygosity at 11p15 (paternal)
OR PAX3 chr 2 -- FKHE chr13 (transl) -- older children -- young adults -- seen in extremities **Use FISH to visualize the translocation |
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List common problems a childhood cancer survivor might encounter.
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excess long-term mortality
second neoplasms endocrine dysfxn and infertility --hypothyroidism --gonadal dysfxn growth retardation neuropsychological and neuro fxn difficulties |
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Childhood cancer and long-term mortality rates?
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5-year survival is not equal to cure
risk of recurrence of primary cancer is still 4 to 5-fold at 20 years relative risk of death due to other causes is still signif increased after 25yrs follow-up |
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What is the likelihood of second neoplasms in a child in remission from cancer?
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10-20 time higher lifetime risk of a second malignant neoplasm (SMN)
Risk factors include: 1. Dx of Hodgkin's, retinoblastoma, and Wilms tumor 2. Tx w/ radiation therapy, alkylating agents, or epipodophyllotoxins 3. underlying susceptibility to cancer like neurofibromatosis, p53 germ cell mutation |
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ANLL is a second malignant neoplasm (SMN) common to which primary malignancies?
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Hodgkin's
-- alkylating agents Leukemias Solid tumors -- epipodophyllotoxin |
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Skin SMNs are common to which primary malignancies?
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any malignancies requiring radiation
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Breast carcinoma is a SMN common to which primary malignancies?
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Hodgkin's
-- mantle radiation Wilms tumor -- whole lung radiation |
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Thyroid carcinoma is a SMN common to which primary malignancies?
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Hodgkin's
-- mantle, neck radiation All CNS prophylaxis -- CNS radiation BMT -- total body radiation |
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CNS glial tumors are a SMN common to which primary malignancies?
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ALL
-- TBI -- CNS radiation |
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Soft tissue and bone sarcomas are a SMN common to which primary malignancies?
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all solid tumors requiring radiation therapy
-- local radiation |
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What is the most common nonmalignant late effect?
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hypothyroidism -- always due to neck radiation
Seen 1.5-16yrs after doses of radiation ranging from 1500-7000cGy Adolescents, females, hemithyroidectomy and use of iodine-containing contrast seems to contribute to increased incidence |
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Describe gonadal dysfxn in male childhood cancer survivors.
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Germ cell depletion and abnormalities of gonadal endocrine fxn
-- testicular, para-aortic node, and ipsilateral pelvic irradiation after unilateral orchiectomy assoc w/ irreversible oligo- and azospermia -- delayed sexual maturation or failure to enter puberty reported in both peripubertal and prepubertal boys treated w/ 2400cGy for testicular leukemia or treated w/ alkylating agents for other malignancies |
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Describe gonadal dysfxn in female childhood cancer survivors.
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radiation damage --> germ cell failure and loss of endocrine fxn; dose and age dependent
Prepubescent overies --> relatively resistant to radiation Peripubertal and fxnal ovaries --> ovarian failure w/ radiation in range of 90-1000cGy and early menopause Ovarian failure at all ages --> assoc w/ myeloablative doses of alkylating agents Fertile female pts -- intensive chemo before pregnancy is compatible w/ normal offspring Hodgkin's and Wilms treated w/ abdom radiation have higher incidence of perinatal death, prematurity, and low birth weight |
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Growth retardation in childhood cancer survivors?
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brain tumors -- 30-35%
leukemia -- 15% **effect on hypothalamus and pituitary is dose and age dependent: >3000cGy <5yrs results in severe growth retardation spine radiation -- early closure of growth plates w/ loss of sitting and standing height **chemo induced is TEMPORARY; changes in weight gain more prominent will obesity reported in pts w/ ALL treated w/ conventional tx or BMT --radiation and corticosteroids are contrib factors |
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Neuro sequelae in childhood cancer survivors?
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CNS tumors and Acute Leukemias have been most intensively studied
-- 8-50% have disabilities requiring special education or intervention Learning disabilities attrib to cranial irradiation -- cumulative dose -- size of indiv fraction -- age of trtmt ** impairment usually irreversible but not progressive after first 3-5yrs after tx CNS tumors -- more severe and global ALL -- more subtle -- attential capacities -- nonverbal cognitive skills |