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Abstract
"The number of
described cancer susceptibility syndromes continues to grow, as does our
knowledge on how to manage these syndromes with the aim of early
detection and cancer prevention. Oncologists now have greater
responsibility to recognize patterns of cancer that warrant referral for
a genetics consultation. While some patterns of common cancers are easy
to recognize as related to hereditary cancer syndromes, there are a
number of rare tumors that are highly associated with cancer syndromes
yet are often overlooked given their infrequency. We present a review of
ten rare tumors that are strongly associated with hereditary cancer
predisposition syndromes: adrenocortical carcinoma, carcinoid tumors,
diffuse gastric cancer, fallopian tube/primary peritoneal cancer,
leiomyosarcoma, medullary thyroid cancer,
paraganglioma/pheochromocytoma, renal cell carcinoma of chromophobe,
hybrid oncocytoic, or oncocytoma histology, sebaceous carcinoma, and sex
cord tumors with annular tubules. This review will serve as a guide for
oncologists to assist in the recognition of rare tumors that warrant
referral for a genetic consultation."
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