Neuroendocrine carcinoma of the ampulla of Vater

Primary small-cell neuroendocrine carcinoma of the duodenum – a case report and review of literature

“…The small-cell NEC occurring in the duodenum and elsewhere in gastrointestinal tract are similar to the small-cell carcinoma of the lung [7,8]. Only eight cases of small-cell NEC in the duodenum have been reported, with the present case being the ninth (Table 2) [7-12]. Most cases occurred in middle-aged or geriatric males with lesions in the ampulla of Vater.…”

“…Most of these show well-differentiated features and are classified as carcinoids or somatostatinomas [3-6]. Occurrence of carcinoma is rare, and carcinomas with anaplastic character, which are classified as small-cell carcinomas, are even less frequent [7-12]. The most common small-cell neuroendocrine carcinoma (NEC) is the small-cell undifferentiated carcinoma of the lung [13,14].…”

Large-cell neuroendocrine carcinoma of the ampulla of Vater

“…In the gastrointestinal tract, SCC is more closely related to squamous mucosa (esophagus and anal canal), whereas LCNEC mostly arise in glandular mucosa (stomach, colon) [7]. In the duodenum, HGNEC occur primarily in the region of the papilla of Vater [6,14,23,24]. In the latest WHO classification of tumors of the digestive system, LCNEC are said to have not been well described in the gastrointestinal tract because of their apparent low frequency [3][4][5].…”

Primary Extra‐ampullary Duodenal Neuroendocrine Carcinoma in an Adult Male

“…World literature reports available with respect to age, sex, size and site distribution show them to be prevalent in geriatric males with mean age of 64.33 years (51–86 years) and with a mean size of 27.5 mm(15–35 mm).There was one unusual case seen in a middle‐aged woman 3,6 . Until now there have only been nine cases reported, of which only two were extra‐ampullary, the rest seen commonly in the ampullary region 3,7–9 . Statistics from India show only one case to be reported in a series of 55 cases of duodenal carcinomas published as yet; however, immunohistochemical confirmation of that case was not possible due to unavailability of stains 10 .…”

“…Most cases have a poor outcome irrespective of radical resection and/or chemotherapy due to rapid recurrence and metastasis. The mean survival time is approximately 7.5 months 7–9 . Only two cases have shown better survival, one adult woman survived more than 18 months 7 and a 57‐year‐old man with gastrointestinal bleeding survived more than 48 months 3 .…”