A primary extra-ampullary duodenal neuroendocrine carcinoma was found in a 40-year-old man who presented with upper abdominal pain and weight loss. Duodenoscopy and hypotonic duodenography revealed a protruding fungating mass with luminal occlusion at the third part of the duodenum (D3). Although the metastatic work-up was normal, the tumor was inoperable intraoperatively, hence a palliative bypass was carried out followed by chemotherapy with 5-fluorouracil and leucovorin. Examination of the biopsy by immunohistochemistry and ultrastructural study revealed it to be neuroendocrine in nature, expressing synaptophysin, chromogranin and cytokeratin and containing dense core cytoplasmic granules. However, there was no evidence of clinical endocrinopathy. The present case emphasizes the need for better detection, further analysis and evaluation of such rare cases to identify their clinical course and effective treatment modalities.

Rapunzel syndrome is a rare condition of gastric trichobezoar with contiguous extension in the intestines found commonly in pediatric and teenage patients, mostly females, with underlying psychological disorders. We report the case of a 37-yearold widow who presented with epigastric pain, fullness and early satiety of 6 months duration. Investigations revealed a trichobezoar extending from the stomach through the duodenum into the jejunum. Psychiatric analysis revealed depressive symptoms. She was posted for elective surgery; however, 2 days prior to the surgery she presented with acute abdomen with plain X-ray of the chest and abdomen showing free gas under the diaphragm. Urgent laparotomy was carried out which confirmed the diagnosis of gastric perforation due to Rapunzel syndrome. The perforation was closed primarily along with removal of the trichobezoar en masse by anterior gastrotomy. This is the first case of Rapunzel syndrome with gastric perforation that has presented in the fourth decade of life. Here we discuss the etiology, pathogenesis, diagnosis and treatment of this unusual syndrome.

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