A Rare Presentation of an Obstructing Metastatic Neuroendocrine Carcinoma of the Distal Ileum: A Case Report

Aims: The aim of the study is to demonstrate a case report on a rare presentation of an obstructing metastatic neuroendocrine carcinoma of the distal ileum.

Study Design: Case report.

Place and Duration of Study: Saudi German Hospital, Jeddah, Saudi Arabia (Departments of General Surgery, Medical Oncology and Pathology).

Background: Neuroendocrine carcinomas typically originated from cells of the endocrine and nervous systems. It can emerge from any part of the gastrointestinal tract and are rarely encountered in the small intestine. It is characterized by being extremely malignant disease with a poor outcome. The presence of metastatic neuroendocrine carcinomas in gastrointestinal system with a primary origin in the small intestine is really an exotic event.

Case Report: A 60-year-old woman attended the Emergency Department with the complaints of repeated vomiting, abdominal distention, pain and constipation, which had been present for 3 days. A CT scan of the abdomen was ordered and revealed a 2.4 cm focal obstructing thickening involving the terminal ileal loops with mesenteric lymph nodes in close vicinity to the lesion. Therefore, an exploratory laparotomy was commenced and resection of the involved segment with an adequate safety margin of more than 5 cm both proximally and distally was obtained together with removal of the mesentery with its contained lymph nodes.

The liver was grossly cirrhotic and involves multiple focal lesions that were palpable on its surface and documented later by an MRI, which confirmed metastatic lesions. The histopathology report confirmed a grade II invasive neuroendocrine Carcinoma of small intestine with 1/4 positive lymph node and the target neoplastic cells showed diffuse positive staining for neuroendocrine markers.

Conclusion: Metastatic obstructing NECs are rarely discovered. These poorly differentiated tumours usually arise in the oesophagus and large bowel. In addition, the disease presents with intestinal obstruction and liver metastatsis, which necessitate post-operative adjuvant chemotherapy.