Devita, Hellman, and Rosenberg's Cancer

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LWBK1006-29 LWW-Govindan-Review December 9, 2011 15:36


Chapter 29•Cancers of Childhood 405

Simpson-Golabi-Behmel syndromes are all associated with predisposition
for developing Wilm’s tumor.

Answer 29.9. The answer is C.
MEN1 syndrome results from an autosomal-dominant mutation in the
MEN1 gene. Patients with MEN1 syndrome are predisposed to develop-
ing multiple endocrine tumors. These include pancreatic islet cell tumors,
pituitary adenomas, and parathyroid adenomas. Pheochromocytomas are
associated with MEN2 syndrome, but not MEN1 syndrome.

Answer 29.10. The answer is A.
Three hereditary syndromes genetically predispose to Wilms’ tumor.
WAGR manifests with aniridia, GU abnormalities, and mental retar-
dation. Denys-Drash syndrome manifests with intersex disorders and
progressive renal failure. Beckwith-Wiedemann syndrome, the most com-
mon, is a hereditary overgrowth syndrome characterized by macroglossia,
visceromegaly, and hyperinsulinemia.

Answer 29.11. The answer is D.
Wilms’ tumor and neurofibromatosis are often associated with syndromic
manifestations that are easy to recognize. No association is described
between neurologic abnormalities and neuroblastoma.

Answer 29.12. The answer is D.
Loss of neurofibromin activity (on both alleles) because of mutations in
NF1 results in elevated levels of GTP-bound ras oncoprotein that trans-
duces signals for cell division. This is one of the mechanisms of tumor
development in neurofibromatosis type 1. The gene located on chromo-
some 22 has been implicated in tumor genesis due to interference of the
function of the protein it encodes, called “merlin.”

Answer 29.13. The answer is B.
Nerve growth factor receptor trkA gene amplification shows ganglionic
differentiation and has a favorable prognosis. All other markers, including
trkB receptor expression, herald poor prognosis neuroblastoma.

Answer 29.14. The answer is A.
EWS-ETS fusion transcripts or related variants can be found in greater
than 90% of peripheral PNET group of tumors by reverse transcriptase
polymerase chain reaction or fluorescence in situ hybridization.

Answer 29.15. The answer is C.
Paternal gene duplication or loss of imprinting of the normally transcrip-
tionally silent maternal allele results in overexpression of IGF-2 in embry-
onal RMS.
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