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

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Islam Khaled
Hany Soliman
Amal Farid
Amr Dorgham


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.

Neuroendocrine carcinoma, metastatic distal ileum.

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How to Cite
Khaled, I., Soliman, H., Farid, A., & Dorgham, A. (2020). A Rare Presentation of an Obstructing Metastatic Neuroendocrine Carcinoma of the Distal Ileum: A Case Report. Asian Journal of Case Reports in Surgery, 4(1), 18-23. Retrieved from https://journalajcrs.com/index.php/AJCRS/article/view/30134
Case Study


Klimstra DS. Pathologic classification of neuroendocrine neoplasms. Hematol Oncol Clin North Am. 2016;30(1):1–19.
DOI: 10.1016/j.hoc.2015.08.005

Rindi G, Klimstra DS, Abedi-Ardekani B, Asa SL, Bosman FT, Brambilla E, et al. A common classification framework for neuroendocrine neoplasms: An Inter-national Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Mod Pathol. 2018;31(12):1770–1786.
DOI: 10.1038/s41379-018-0110-y

Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, et al. One hundred years after "carcinoid": Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26(18):3063–3072.
DOI: 10.1200/JCO.2007.15.4377.

Vinik AI, Chaya C. Clinical presentation and diagnosis of neuroendocrine tumors. Hematol Oncol Clin North Am. 2016;30(1): 21–48.
DOI: 10.1016/j.hoc.2015.08.006

Taal BG, Visser O. Epidemiology of neuroendocrine tumours. Neuroendocrino-logy. 2004;80(Suppl 1):3–7.
DOI: 10.1159/000080731

Egashira A, Morita M, Kumagai R, Taguchi KI, Ueda M, Yamaguchi S, et al. Neuroendocrine carcinoma of the esophagus: Clinic pathological and immuno-histochemical features of 14 cases. PLoS One. 2017;12(3):e0173501.
DOI: 10.1371/journal.pone.0173501
[PMID: 28288180; PMCID: PMC5348034]

Clift AK, Drymousis P, Al-Nahhas A, Wasan H, Martin J, Holm S, et al. Incidence of second primary malignancies in patients with neuroendocrine tumours. Neuroendocrinology. 2015;102(1-2):26-32.
DOI: 10.1159/000381716
[PMID: 25824138]

Shafqat H, Ali S, Salhab M, Olszewski AJ. Survival of patients with neuroendocrine carcinoma of the colon and rectum: A population-based analysis. Dis Colon Rectum. 2015;58(3):294–303.
DOI: 10.1097/DCR.0000000000000298.

Eads JR. Poorly differentiated neuroendocrine tumors. Hematol Oncol Clin North Am. 2016;30(1):151–162.
DOI: 10.1016/j.hoc.2015.09.007.

Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, et al. Neuroendocrine carcinomas of the colon and rectum. Dis Colon Rectum. 2004;47(2):163–169.
DOI: 10.1007/s10350-003-0038-1

Hung SS. Small cell carcinoma of the colon. A case report and literature review. J Clin Gastroenterol. 1989;11(3):335– 339.
DOI: 10.1097/00004836-198906000-00019

Kuo CH, Hsieh CC, Chan ML, Li AF, Huang MH, Hsu WH, et al. Small cell carcinoma of the esophagus: a report of 16 cases from a single institution and literature review. Ann Thorac Surg. 2011; 91(2):373–378.
DOI: 10.1016/j.athoracsur.2010.09.030

Strosberg JR, Coppola D, Klimstra DS, Phan AT, Kulke MH, Wiseman GA, et al. The NANETS consensus guidelines for the diagnosis and management of poorly differentiated (high-grade) extrapulmonary neuroendocrine carcinomas. Pancreas. 2010;39(6):799–800.
DOI: 10.1097/MPA.0b013e3181ebb56f