A well differentiated neuroendocrine tumor of the jejunum with peritoneal carcinomatosis: A case report

Abstract

Neuroendocrine tumors (NETs), belong to a group of neoplasms that arise from neuroendocrine cells and express markers such as synaptophysin and chromogranin A. The digestive system (DS) is the most common site of NET development. The World Health Organization classification divides NETs into low grade (G1-G2) tumors (NETs) and high grade carcinomas [neuroendocrine carcinomas (NECs)], based on mitotic index and histological criteria. NET prognosis depends on tumor stage and grade. Low grade G1 NETs are characterized by a low proliferative rate and an indolent clinical course with a 5-year survival rate ranging between 38% (pancreas) and 88% (rectum). The present study reports a case of a low grade (G1) multifocal jejunal tumor with histologically confirmed features of aggressiveness, namely peritoneal carcinomatosis, lymph node metastasis and vascular carcinomatous emboli. Prediction of clinical behavior and survival in such a case is challenging. Although multiplicity and nodal metastases is not unusual for low grade NETs in this part of the gastrointestinal tract, peritoneal carcinomatosis is an extremely rare finding. Surgeons and histopathologists should be familiar with such eventualities and tumor boards are required in order to conclude whether aggressive therapeutic interventions may have any impact on patients' long term survival.

Malignant behavior of a well‑differentiated digestive neuroendocrine tumor with peritoneal carcinomatosis: A case report

“…What stands out in the presented case is the rapid disease progression and the development of peritoneal metastases despite what appeared to be a histologically grade 1 siNEN, and which to our knowledge has been reported only twice (1,7).…”

“…NENs are a heterogeneous group of tumors arising from enterochromaffin cells, being multipotent stem cells that migrate from the neural crest to the gut ectoderm (1). The most common primary sites are the gastro-intestinal and respiratory tract.…”

Multiple Small Intestinal Neuroendocrine Tumors With Findings of Intestinal Obstruction

“…Regarding its pathophysiology, SI-NETs are derived from Kulchitsky (enterochromaffin) cells, which are multipotent stem cells that migrate from the neural crest cells at the crypts of Lieberkuhn base to the gut ectoderm [ 2 , 8 ]. The obstruction of the intestine can be caused by peritumoral fibrosis or tumor invasion of the mesentery, which could cause kinking of the bowel, or secondary to desmoplastic reaction with scarring of the mass, which can cause ischemic effects [ 2 , 9 ].…”