Obstructive jejunal adenocarcinoma in the Muir-Torre syndrome.
A young male patient was referred for endoscopic resection of a recto-sigmoid polyp and abdominal complaints. His medical history revealed a sebaceous adenoma resection from his back and a tubular adenoma excision from his right upper eyelid. After disclosure of a jejunal tumour on a small bowel enema and given the remarkable family history characterized by the frequent occurrence of bowel cancer, the diagnosis of Muir-Torre syndrome was established. This syndrome pertains to the combination of sebaceous gland tumours/adenomas in combination with gastrointestinal or genitourinary tract tumours. Inheritance takes place in an autosomal dominant manner. It usually occurs in males at an early age. Given its low malignancy potential, early detection of the syndrome renders a favourable prognosis. Careful examination and follow-up also involving the small intestine are mandatory for patients and relatives.
Panday
S C
SC
Department of Internal Medicine, Canisius Wilhelmina Hospital, Nijmegen, Netherlands.
Go
I H
IH
Mravunac
M
M
de Koning
R W
RW
D000230:Adenocarcinoma / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery; D000236:Adenoma / Q000235:genetics / Q000601:surgery; D000328:Adult; D001466:Barium Sulfate; D003113:Colonoscopy; D004733:Enema; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology*; D007580:Jejunal Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery; D011859:Radiography; D012626:Sebaceous Gland Neoplasms / Q000235:genetics / Q000601:surgery; D013577:Syndrome
D001466:Barium Sulfate