A Retrospective Review of 126 High-Grade Neuroendocrine Carcinomas of the Colon and Rectum

Abstract

Background High-grade neuroendocrine carcinomas (HGNECs) of the colon and rectum are rare, constituting less than 1 % of colorectal cancers. The purpose of this study was to identify the natural history and oncologic outcomes of this disease, describe the use of surgery, and determine the clinical and pathological factors associated with outcomes. Methods Following Institutional Review Board approval, patients with HGNEC were identified from our institutional database. Patient charts and pathology reports were analyzed retrospectively for clinical and pathological factors. Results A total of 126 patients with a median follow-up of 9 months were identified. Median survival was 13.2 months, and 85 (67 %) patients had metastatic disease at diagnosis. Three-year overall survival (OS) was 5 and 18 % for patients with and without metastatic disease, respectively. Factors associated with improved OS on multivariable analysis were absence of metastatic disease and presence of an adenocarcinoma component within the tumor. In patients with metastatic disease, response to chemotherapy was the only factor associated with survival. In patients with localized disease, an adenocarcinoma component within the tumor was the only factor associated with survival. Resection of tumor was not associated with survival in either localized or metastatic disease. Conclusion High-grade colorectal NECs are extremely aggressive tumors with poor prognosis. Patients appear to have a marginally better prognosis if they present without metastatic disease, have an adenocarcinoma component within their tumor, or respond to chemotherapy. Surgery, particularly in the presence of metastatic disease, may not offer a survival benefit for the majority of patients.

Hindgut Neuroendocrine Neoplasia

“…Hindgut HGNECs present in the same manner as adenocarcinomas with rectal bleeding, change of bowel habit and tenesmus being the most common symptoms. [22] Diagnosis is again made by colonoscopy and biopsy. Often HGNEC can occur in conjunction with colorectal adenocarcinomas.…”

“…[25] There is some evidence that collision tumors, particularly if localized have an improved prognosis. [22] Histologically HGNEC can also be either the small cell type or large cell type with some reports that large cell HGNEC may have a better prognosis. [23] Staging for localized and metastatic disease is the same as that for colorectal adenocarcinoma.…”

“…For rectal HGNEC, similar to rectal adenocarcinomas, management should start with neoadjuvant chemoradiotherapy as HGNEC appear to radiosensitive. [22] As these tumors have a high systemic recurrence consideration should be given to follow on with systemic chemotherapy without surgery, particularly if there is a good response to radiotherapy. The reason for this is surgery; particularly major surgery like a rectal resection may delay systemic treatment, which is the patients' best way of preventing systemic recurrence.…”

“…In fact although the numbers are small in the largest series of colorectal HGNEC, resection of colorectal HGNEC did not appear to influence survival. [22] As hindgut HGNEC are uncommon with evidence limited to case series, chemotherapeutic treatment are derived from the management of the more frequent pulmonary HGNEC. Platinum based chemotherapy is therefore the treatment of choice.…”

“…In this scenario the standard adjuvant treatment for colorectal cancer, 5-flourouracil with oxaliplatin (FOLFOX) can be used as oxaliplatin has been shown to have a response in HGNEC also. [22] For metastatic disease, unlike in adenocarcinoma, surgical resection does not appear to offer any survival benefit. [22] Surgical management should therefore be reserved for emergency situations such as obstruction or bleeding.…”