LWBK1006-29 LWW-Govindan-Review December 9, 2011 15:36
404 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology ReviewANSWERS
Answer 29.1. The answer is C.
Pediatric malignancies generally cannot be traced to long-term exposure
to known carcinogens because they occur in the young, without time for
prolonged exposure and transition to a malignant change.Answer 29.2. The answer is D.
The mechanisms listed above are some of the known pathways of car-
cinogenesis in the pediatric age group and predispose to specific groups
of malignant disorders.Answer 29.3. The answer is C.
Many hereditary premalignant disorders, such as Li-Fraumeni, hereditary
retinoblastoma, and familial adenomatous polyposis, are devoid of phys-
ical stigmata making them difficult to diagnose without a detailed family
history.Answer 29.4. The answer is B.
Xeroderma pigmentosum predisposes to melanoma and leukemia and is
caused by multiple gene mutations.Answer 29.5. The answer is A.
Ewing sarcoma is associated witht(11;22) and synovial sarcoma is associ-
ated witht(x;11). Cytogenetic abnormalities seen in Wilm’s tumor include
del11p13 andt(3;17), and retinoblastoma is associated with del13q14.Answer 29.6. The answer is C.
EWS is not implicated in retinoblastoma formation but is involved in
the development of Ewing sarcoma. Amplification of MDMX results in
retinoblastoma progression.Answer 29.7. The answer is D.
Retinoblastoma is associated with deletions or mutations of Rb1. In neu-
roblastoma, there are deletions or mutations in the short arm of chromo-
some 1, and rarely chromosomes 10, 14, 17, and 19; neuroblastoma is
also associated with amplification of the N-myc oncogene. Ewing sarcoma
is characterized by a reciprocalt(11;22) translocation, fusing a transcrip-
tion factor FLI-1 with oncogene EWS.Answer 29.8. The answer is A.
Von Hippel-Lindau syndrome is associated with predisposition for devel-
oping renal cell carcinoma, whereas Beckwith-Wiedemann, WAGR, and