Abstract
BackgroundCREST (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias) syndrome has been rarely associated with other malignancies (lung, esophagus).This is the first report of a primary adenocarcinoma of the third portion of the duodenum in a patient with CREST syndrome.Case presentationA 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.ConclusionCREST 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.
Highlights
CREST (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias) syndrome has been rarely associated with other malignancies.This is the first report of a primary adenocarcinoma of the third portion of the duodenum in a patient with CREST syndrome.Case presentation: 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
CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias) has been rarely associated with other malignancies, whereas duodenal adenocarcinoma has never been reported to be associated with this subtype of systemic sclerosis [1,2,3]
This is the first report of a primary adenocarcinoma of the third portion of the duodenum in an adult male patient with CREST syndrome
Summary
A relationship between duodenal adenocarcinoma and limited cutaneous scleroderma has not yet been described and in our case is considered a matter of coincidence. Primary duodenal adenocarcinoma is an aggressive tumor with an overall 5-year survival rate of about 25% [7], which can be significantly improved up to 54% after curative resection [8]. A case of an early duodenal adenocarcinoma from a Brunner's gland has been reported [12]. Limited cutaneous scleroderma or CREST syndrome consists one of the two main subsets of systemic sclerosis, which is a multisystemic disorder of unclear pathogenesis, characterized by inflammatory, vascular and fibrotic changes of the skin and various internal organ systems (GI tract, lungs, heart and kidneys) and involves immunologic mechanisms leading to vascular endothelial damage and fibroblast activation. A copy of the written consent is available for review by the Editor-in-Chief of this journal
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