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Variant of Gardener's Syndrome

TO THE EDITOR: Recently, I came across a 54-yr-old white woman with a history of abdominal pain and weight loss. Evaluation included EGD (Fig. 1), colonoscopy, and CT scan of the abdomen. She was found to have multiple benign gastric polyps. Her records revealed a past history of multiple intradermal and junctional nevus. Later, she developed obstructive jaundice, and ERCP detected pancreatic cancer. There are many well-known syndromes reported with an association between gastrointestinal polyps, ectodermal neoplasia and pancreatic cancer. Examples of such syndromes (1) are Peutz-Jegher syndrome (mucocutaneous pigmentation and intestinal polyposis) and Cronkhite-Cananda syndrome (ectodermal defects such as alopecia, excessive skin pigmentation, and generalized gastrointestinal popyposis) and Gardener’s syndrome (soft tissue tumors such as sebaceous cysts and fibromas, bony tumors such as osteoma, adenomatous polyps of the colon and rectum associated with development of periampullary tumors). The case reported here had a somewhat different configuration: pancreatic cancer, multiple benign gastric polyps, multiple intradermal and junctional nevus, and no colonic or rectal polyposis. The purpose of this letter is to find out whether any similar cases have been noticed by readers. Is this reported combination just a coincidence, or could here be any relationship that should be explored further?