Devita, Hellman, and Rosenberg's Cancer

LWBK1006-25 LWW-Govindan-Review December 12, 2011 19:21

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

Answer 25.24. The answer is A.

The gene MEN2A is the RET proto-oncogene, located at the centromeric

region of chromosome 10 (10q11-2). Data are accumulating regarding

genotype-phenotype correlations and mutations in various RET codons.

A negative family history is not reliable because of the variable age of

clinical presentation that can affect the identification of clinical cases.

Thus, family members should be screened.

Answer 25.25. The answer is B.

The MENIN gene was recently identified, and it is localized on the long

arm of chromosome 11.

Answer 25.26. The answer is D.

The RET proto-oncogene mutation is associated with MEN2 and

Hirschsprung disease.

Answer 25.27. The answer is B.

The development of amenorrhea, acne, hirsutism, and rapidly progres-

sive Cushing’s syndrome in a woman is a typical presentation of adrenal

carcinoma. Complete metabolic workup is indicated before any inter-

vention. Approximately 50% of adrenocortical neoplasms have features

of hormonal hypersecretion. Surgical resection represents the standard

treatment in localized tumors. Mitotane is given in metastatic disease

and as adjuvant therapy. Hormonal hypersecretion can be medically con-

trolled: Effective agents include ketoconazole, aminoglutethimide, and

metyrapone.

Answer 25.28. The answer is D.

The cyclophosphamide, vincristine, and doxorubicin regimen has been the

most used chemotherapy regimen in patients with metastatic pheochro-

mocytoma. External beam radiation can palliate metastatic bone lesions.

I131 meta-iodo-benzyl-guanidine leads to a response rate of approxi-

mately 40%. Mitotane is used to treat metastatic adrenocortical carci-

noma and not pheochromocytoma.

Answer 25.29. The answer is E.

Carcinoid tumors are responsive to higher doses of external beam radia-

tion, with a response rate of 40% to 50%.

Answer 25.30. The answer is A.

Size more than 6 cm should raise the possibility of adrenocortical carci-

noma. The other features are suggestive of benign adrenal adenoma.

Answer 25.31. The answer is A.

Pheochromocytoma arises from the adrenal medulla in 90% of cases,

where most are not malignant. Extra-adrenal lesions have increased risk