Research Article
Qasem E, Nangalia M, Jones H,
Abstract
Small cell carcinoma (SCC) is a rare malignancy that can affect the gastrointestinal tract. Current knowledge is limited to individual case series. Here we present three cases from our own institution and report the results of a systematic review of the literature. Methods and findings: A ten-year review of our institutions pathology database for rectal SCC was performed. A systematic search of Embase, PubMed and Google Scholar with cross-referencing was performed applying suitable exclusion criteria. Three cases were identified in our institution from 2004-2014. All were positive for synaptophysin, TTF-1, CD56 and CK positive. All had metastatic disease at presentation and had chemoradiotherapy. Median survival was 13 months. A systematic search found 112 articles of which 41 were included (121 patients). Optimal survival (37 months) was observed with a combination of surgery, chemotherapy and radiotherapy. Conclusion: SCC rectum is a rare entity that is usually metastatic at presentation and has poor long-term survival despite aggressive treatment schedules. Prospective registries would optimize management of this unusual neuroendocrine tumour.