Devita, Hellman, and Rosenberg's Cancer

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

406 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology Review

Answer 29.16. The answer is B.

Except for MGF, all other statements regarding alveolar RMS are true.

Additional mutations on p53 are also described. Interestingly, the mouse

model with overexpression of PAX-3-FKHR develops no tumors, but

mice expressing an hepatocyte growth factor transgene develop embry-

onal RMS.

Answer 29.17. The answer is D.

Some 60% to 80% of Li-Fraumeni families have detectable germ line

mutations in p53. The others may be associated with modifier genes,

promoter defects, or involvement of other as yet unknown candidate genes

involved in the p53 tumor suppressor pathway. hCHK2, a checkpoint

kinase, is a potential candidate.

Answer 29.18. The answer is D.

Identification of various altered molecular signaling pathways (e.g.,

growth factor, tumor suppressor, and tyrosine kinase) have shown that

novel and more effective treatment approaches may lie within reach when

these pathways are targeted against tumors.

Answer 29.19. The answer is B.

The presentation is classic for Wilms’ tumor. This often silent tumor can

metastasize or invade all the structures listed and can perforate through

the capsule if very large. It is, however, unlikely to invade muscle tissue.

The common pathologic variety is termed “favorable histology Wilms’

tumor.”

Answer 29.20. The answer is C.

Pulmonary metastasis is the most common site of distant dissemination.

The need for a chest CT in addition to a chest radiograph is controversial

because small nodules identified by CT scans are often negative on biopsy.

Pulmonary nodules should be biopsied to prove tumor dissemination.

Brain and bone imaging are only necessary if the diagnosis includes rare

renal tumors, such as clear cell sarcoma or rhabdoid tumor.

Answer 29.21. The answer is D.

The National Wilms Tumor Study Group (NWTSG) supports tumor

resection. There is a 19.8% operative complication rate that includes

intestinal obstruction and hemorrhage. Although the SIOP has promoted

the use of preoperative chemotherapy before biopsy, there is a 7.6% to

9.9% rate of benign or other malignant disorders involving the kidney

in children. The risk of rupture of the tumor during surgery is 14% and

may be associated with an increased risk of local recurrence. Preopera-

tive chemotherapy after a biopsy is recommended for solitary kidneys,

bilateral renal tumors, tumor in a horseshoe kidney, or patients who are

at anesthesia risks because of extensive lung involvement. Exploring the

opposite kidney is also recommended by the NWTSG because there is a