LWBK1006-20 LWW-Govindan-Review December 12, 2011 19:4
Chapter 20•Cancer of the Gastrointestinal Tract 267Answer 20.4.6. The answer is A.
Carney triad, which encompasses multifocal GIST with pulmonary chon-
droma and extra-adrenal paraganglioma, familial paraganglioma, and
neurofibromatosis type 1 are associated with GIST. These syndromes,
along with pediatric GIST, can be characterized by the wild-type KIT
gene. It is unclear whether GISTs associated with these syndromes have a
similar response to imatinib therapy as sporadic GIST.Answer 20.4.7. The answer is E.
All of the conditions listed are associated with increased risk for the devel-
opment of cancer in the small intestine. Other conditions include AIDS,
neurofibromatosis, Gardner syndrome, Peutz-Jeghers, and a history of
other primary tumors, such as uterine, ovarian, prostate, thyroid, skin,
and soft tissue sarcomas.Answer 20.4.8. The answer is C.
The most common tumors of the small bowel are carcinoids, followed by
adenocarcinomas and lymphomas. Sarcomas are less likely and schwan-
nomas are rare.Answer 20.4.9. The answer is D.
MALT lymphomas are most commonly seen in men, with a peak in the
sixth decade. These malignancies may be associated with chronic inflam-
matory disorders such as Hashimoto’s thyroiditis and the majority of
patients present with stages I or II. The translocationt(11;14) causing
overexpression of cyclin D1 is characteristic of mantle cell lymphoma.Answer 20.4.10. The answer is B.
Small bowel tumors are slightly more common in men. Duodenum rep-
resents with most common location, accounting for approximately half
of the cases. Compared to more distal lesions, tumors of the duodenum
tend to occur in older patients and are associated with worse outcomes.Answer 20.4.11. The answer is D.
Although the patient is at high risk for developing recurrent or metastatic
disease, there is currently no clear role for the use of adjuvant octreotide
therapy or chemotherapy. Close follow-up is necessary, and if tumor
markers are elevated, repeat CT or octreotide scans may be done to deter-
mine metastatic disease.Answer 20.4.12. The answer is D.
The main criteria to distinguish between primary intestinal lym-
phomas and secondary involvement include no superficial palpable lym-
phadenopathy, no mediastinal adenopathy, no evidence of peripheral
blood or bone marrow involvement, no involvement of the liver or spleen
unless by direct extension of the primary tumor, and disease confined to
the affected small bowel and regional draining mesenteric lymph nodes.