Adenocarcinoma of the third portion of the duodenum in a man with CREST syndrome

A 54-year-old male patient with CREST syndrome presented with colicky postprandial pain of the upper abdomen, diminished food uptake and a 6-Kg-body weight loss during the previous 2 months. An ulcerative lesion in the third portion of the duodenum was revealed during duodenoscopy, with a diagnosis of adenocarcinoma on biopsy specimen histology. The patient underwent a partial pancreatoduodenectomy. No adjuvant therapy was instituted and follow-up is negative for local recurrence or metastases 21 months postoperatively.CREST syndrome has been associated with colon cancer, gastric polyps, familial adenomatous polyposis (FAP) syndrome and Crohn's disease; however, this is the first report of a primary adenocarcinoma of the duodenum in a patient with CREST syndrome. However, any etiologic relationship remains to be further investigated.CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias) has been rarely associated with other malignancies (lung, esophagus), whereas duodenal adenocarcinoma has never been reported to be associated with this subtype of systemic sclerosis [1-3]. This is the first report of a primary adenocarcinoma of the third portion of the duodenum in an adult male patient with CREST syndrome.A 54-year-old man was referred to our clinic because of colicky postprandial pain of the upper abdomen during the last month, with aggravation 5 days before admission. The patient had diminished the amount of food uptake and had also a 6-Kg-body weight loss during the previous 2 months. His past medical history included CREST syndrome under cortisone therapy during the last year and primary repair of the left ureter and the small intestine due to traumatic perforation in a car accident 17 years ago. Physical examination revealed a pale, malnourished patient and an unremarkable abdomen without tenderness or distension. Besides an anemia, the other blood investigations, as well as tumor markers, were within normal