Large Cell Neuroendocrine Carcinoma of the Colon With Carcinomatosis Peritonei

Abstract

Colorectal large-cell neuroendocrine carcinomas (NECs) are extremely rare and have very poor prognosis compared to adenocarcinomas. A 74-year-old man presented with abdominal pain, diarrhea and hematochezia. The histopathologic report of colonoscopic biopsy performed at a local clinic was a poorly differentiated carcinoma. An abdominopelvic computed scan revealed irregularly enhanced wall thickening at the sigmoid colon with regional fat stranding and lymphnode enlargement. He underwent a laparoscopic high anterior resection with selective peritonectomy for peritoneal carcinomatosis, intraoperative peritoneal irrigation chemotherapy, and early postoperative intraperitoneal chemotherapy for 5 days. The tumor had a high proliferation rate (mitotic count > 50/10 HPFs and 90% of the Ki-67 index) and lymph-node metastases had occurred. On immunohistochemistry, the tumor cells expressed CD56 and synaptophysin. Large-cell NEC was confirmed. Systemic chemotherapy with cisplatin/etoposide was done. The patient is still alive after 3 years with no evidence of recurrence.

Large cell neuroendocrine carcinoma of the colon with brain metastasis: A case report

“…Bernick et al publish a study that showed that 0.6% of patients with malignant colorectal tumors had neuroendocrine carcinoma and only 0.2% of those were large cell neuroendocrine carcinomas [ 6 ]. There is paucity of paper reporting large cell colonic neuroendocrine carcinomas (LCNEC) in literature [ [7] , [8] , [9] , [10] , [11] , [12] ].…”

A case of colorectal large cell neuroendocrine carcinoma accompanied by disseminated peritoneal leiomyomatosis

“…There are few previous reports on colorectal LCNECs, excluding MiNENs, with only 12 cases reported in English literature (Table 1) [4][5][6][7][8][9][10][11][12]. Most patients with Stage IV died within a few months after diagnosis.…”

“…Most patients with Stage IV died within a few months after diagnosis. Only one patient with localized peritoneal dissemination survived beyond 3 years [ 11 ]. The present case is the only report of extra-regional lymph node metastasis.…”

“…A definitive diagnosis of NEC was not made based on the previous doctor’s biopsy. In previous reports, NEC cases were difficult to diagnose by biopsy [ 11 , 13 ]. Although resection of the primary tumor is not recommended for asymptomatic Stage IV colorectal cancer in Japanese Society for Cancer of the Colon and Rectum guidelines for the treatment of colorectal cancer [ 14 ], if the diagnosis based on biopsy is difficult to confirm, total resection of the primary tumor for both the diagnosis and treatment should be considered.…”

Isolation and Characterization of Two Novel Colorectal Cancer Cell Lines, Containing a Subpopulation with Potential Stem-Like Properties: Treatment Options by MYC/NMYC Inhibition

“…CRC comprises the development of malignancies in the colon (72%) and the rectum (28%) and is the most common malignancy in the gastrointestinal tract [ 2 ], with 90% of all CRCs being an adenocarcinoma (AC) originating from epithelial mucosa cells [ 3 ]. A comparatively rare and poorly understood subtype of CRC is the neuroendocrine carcinoma (NEC), representing a subgroup of neuroendocrine neoplasms comprised of poorly-differentiated neoplasms originating from epithelial cells of the endocrine or nervous system [ 4 ]. According to the WHO classification of 2010, colorectal gastro-entero-pancreatic neuroendocrine-carcinoma (GEP-NEC) is a rare and aggressive variant of neuroendocrine tumors (NET) as well as CRC with more than one third having metastatic disease at the time of diagnosis [ 5 , 6 ].…”