Devita, Hellman, and Rosenberg's Cancer

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

Chapter 29•Cancers of Childhood 395

Question 29.11. Strong correlations are present between all of the following, EXCEPT:

A. Wilms’ tumor, intersex disorders, and progressive renal failure

B. Macroglossia, hyperinsulinemia, and Wilms’ tumor

C. Neurofibromas, cafe au lait spots, and optic glioma ́

D. Mental retardation, spinal abnormalities, and neuroblastoma

Question 29.12. The NF1 gene on chromosome 17 has which of the following functions:

A. Encoding a ubiquitously expressed protein neurofibromin

B. Facilitating the hydrolysis of GTP to GDP by ras oncoprotein

C. Sending inhibitory signals to cell division

D. All of the above

Question 29.13. Each of the following is associated with poor prognosis neuroblastoma,

EXCEPT:

A. N-myc amplification

B. trkA gene activation

C. LOH at 1p36 and 11q23

D. Telomerase expression and increased telomere length

Question 29.14. The fusion transcript associated with t(11;22), EWS-ETS, or

EWS-FLI-1, and loss of p53 and/or p16 signaling are associated with

all of the following tumors, EXCEPT:

A. Osteosarcoma

B. Ewing sarcoma

C. Askin’s tumor

D. Peripheral primitive neuroectodermal tumor (pPNET)

Question 29.15. Which of the following statements regarding the molecular basis for the

development of embryonal rhabdomyosarcoma (RMS) is FALSE?

A. They are characterized by LOH at the 11p15 locus.

B. Insulin-like growth factor (IGF)-2 gene activity is increased twofold.

C. IGF-2 tumor suppressor gene activity is lost with the LOH.

D. There is loss of paternal imprinting of IGF-2.

Question 29.16. Alveolar RMS has all of the following molecular characteristics, EXCEPT:

A. t(2;13) ort(1;13) translocation (PAX-3-FKHR or PAX-7-FKHR

fusion)

B. Increased expression of myoblast growth factor (MGF)

C. Increased c-met expression

D. Increased hepatocyte growth factor expression

Question 29.17. The Li-Fraumeni syndrome is characterized by the following:

A. Germ line mutations in the tumor suppressor gene p53

B. Expression of a stabilized mutant protein in affected cells

C. Mutation or deletion of the second wild-type p53 allele in tumors

D. All of the above