LWBK1006-14 LWW-Govindan-Review November 24, 2011 11:28
160 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology Reviewbut can also develop in the colon or the stomach. Recurrent obstruction
and intussusception may occur. Individuals with Peutz-Jeghers syndrome
are at increased risk for various tumors, including breast, colorectal, ovar-
ian, gastric, and pancreatic cancers. Affected females are also at increased
risk for adenoma malignum of the cervix and a benign ovarian tumor,
sex cord tumors with annular tubules (SCTAT). Peutz-Jeghers syndrome
results from mutations in theSTK11gene found on chromosome 19.
The Peutz-Jeghers syndrome serves as an example of the importance of
a detailed physical examination, in combination with the family can-
cer history, to identify individuals with a hereditary cancer syndrome.
Other physical features that provide important diagnostic clues include
the mucosal neuromas of the lips and tongue in the multiple endocrine
neoplasia syndrome type 2B, trichilemmomas or papillomatous lesions
in the Cowden syndrome, and sebaceous adenomas in the Muir-Torre
syndrome.