Neuroendocrine carcinomas of the colon and rectum



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Dis Colon Rectum. 2004 Feb;47(2):163-9. Related Articles, Links

Neuroendocrine carcinomas of the colon and rectum.

Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, Thaler H,
Guillem J, Paty P, Cohen AM, Wong WD.

Colorectal Service, Memorial Sloan-Kettering Cancer Center, New York,
New York 10021, USA.

PURPOSE: This study was designed to review experience with
neuroendocrine carcinomas of the colon and rectum at a single institution,
with emphasis on the pathology and clinical characteristics of this
uncommon malignancy.

METHODS: A study group of patients was identified from a prospective
colorectal service database. Pathology was reviewed and neuroendocrine
tumors were classified by a single pathologist. Medical records were
retrospectively reviewed.

RESULTS: From March 1975 to September 1998, 38 patients with
neuroendocrine carcinomas were identified from the colorectal service
database comprising 6495 patients (0.6 percent). These neuroendocrine
carcinomas did not include carcinoid tumors. Average patient age was 57
years (range, 29-86 years). There were 17 males (44.7 percent) and 21
females (55.3 percent). Tumors were located as follows: 17 colon, 14
rectum, 6 anal canal, and 1 appendix. The diagnosis of neuroendocrine
carcinoma was suggested preoperatively from tissue biopsy in 59.3 percent
(16/27) of patients evaluable. Pathology was reviewed and tumors were
categorized as small cell carcinoma (n = 22) or large cell neuroendocrine
carcinoma (n = 16). Most tumors (20/25 evaluable, 80 percent) stained
positive by means of immunohistochemistry for neuroendocrine markers,
including chromogranin (18/19), synaptophysin (10/15), and/or
neuron-specific enolase (14/15). Metastatic disease was detected at the
time of diagnosis in 69.4 percent of the patients (25/36). Tumors were
advanced at the time of diagnosis, with American Joint Committee on Cancer
(AJCC) Stage I (n = 6), Stage III (n = 7), and Stage IV (n = 25) tumors.

As a group, these tumors had a poor prognosis, with a median survival of
10.4 months. One-year, two-year, and three-year survival was 46 percent,
26 percent, and 13 percent, respectively. There was no significant
difference in survival based on pathologic subtypes. Median follow-up time
was 9.4 months (range, 0.6-263.7 months).

CONCLUSIONS: Neuroendocrine carcinomas of the colon and rectum are
uncommon, comprising less than 1 percent of colon and rectal cancers.
Pathologically, these tumors are poorly differentiated carcinomas with
distinctive cytoarchitectural features and are often immunoreactive for
markers of neuroendocrine differentiation. The prognosis for high-grade
neuroendocrine carcinomas is poor, as most patients have metastatic
disease at the time of diagnosis.
PMID: 15043285 [PubMed - indexed for MEDLINE]

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