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26 Cards in this Set

  • Front
  • Back
The majority of childhood cancers have what cell type of origin?
embryonic cells such as neuroblastoma or retinoblastoma
What congenital syndromes predispose a child to cancer?
Down's
Beckwith-Wiedemann
ataxia-telangiectasia
neurofibromatosis
Li-Fraumeni syndrome
familial polyposis coli
Bloom syndrome
What are the most common cancer types in children? (3)
Leukemia (25%)

CNS tumors (17%)

Lymphomas (16%)
What age group(s) of children has the highest incidence of cancer?
0-4yrs -- usually attrib to genetic component

15-19yrs -- puberty begins; reason for ↑ incidence at this age is largely unknown
Which cancer age group has the poorest outcome?
adolescents and young adults have a poorer outcome than younger children and older adults
Common signs and symptoms of leukemia?
pallor, fatigue, fever
bruises, bleeding
lymphadenopathy
bone pain, limping
hepatomegaly
splenomegaly
Common signs and symptoms of brain tumor?
headache
morning vomiting

May have CSF blockage, ataxia, focal neural deficits ± hemiparesis, papilledema
Common signs and symptoms of lymphoma?
First presentation usually cervical lymphadenopathy
hepatomegaly
splenomegaly
bone pain, limping (bone lymphoma)

may have painless mass, symptoms of compression, fever, night sweats, wt loss
Childhood cancer predisposition due to chromosome abnormalities?
Trisomy 21:
-- ANLL M7 (<2yrs)
-- ALL (x20)

Turner's & Klinefelter's Syndrome
-- gonadoblastoma
-- dysgerminoma
-- extragonidal
Childhood cancer predisposition due to autosomal dominant conditions?
Rb gene mutation:
-- retinoblastoma
-- osteosarcoma
-- melanoma

p53 gene mutation:
-- adrenal carcinoma
-- brain tumors
-- sarcomas
-- breast cancer
-- leukemias

Multiple endocrine neoplasia:
-- thyroid carcinomas
-- pheochromocytomas
What is the key to early detection of childhood cancer?
"high index of suspicion"
-- educate parents
-- thorough exam
ex: red reflex in infant for retinoblastoma
Indicators of rhabdomyosarcoma?
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
List common problems a childhood cancer survivor might encounter.
excess long-term mortality
second neoplasms
endocrine dysfxn and infertility
--hypothyroidism
--gonadal dysfxn
growth retardation
neuropsychological and neuro fxn difficulties
Childhood cancer and long-term mortality rates?
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
What is the likelihood of second neoplasms in a child in remission from cancer?
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
ANLL is a second malignant neoplasm (SMN) common to which primary malignancies?
Hodgkin's
-- alkylating agents

Leukemias
Solid tumors
-- epipodophyllotoxin
Skin SMNs are common to which primary malignancies?
any malignancies requiring radiation
Breast carcinoma is a SMN common to which primary malignancies?
Hodgkin's
-- mantle radiation

Wilms tumor
-- whole lung radiation
Thyroid carcinoma is a SMN common to which primary malignancies?
Hodgkin's
-- mantle, neck radiation

All CNS prophylaxis
-- CNS radiation

BMT
-- total body radiation
CNS glial tumors are a SMN common to which primary malignancies?
ALL
-- TBI
-- CNS radiation
Soft tissue and bone sarcomas are a SMN common to which primary malignancies?
all solid tumors requiring radiation therapy
-- local radiation
What is the most common nonmalignant late effect?
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
Describe gonadal dysfxn in male childhood cancer survivors.
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
Describe gonadal dysfxn in female childhood cancer survivors.
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
Growth retardation in childhood cancer survivors?
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
Neuro sequelae in childhood cancer survivors?
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