Poorly Differentiated Neuroendocrine Carcinoma of the Sigmoid Tract in Long-Standing Ulcerative Colitis: Report of a Case and Review of the Literature

Abstract

A 37-year-old male with long-standing and extensive ulcerative pancolitis developed a rapidly lethal poorly differentiated neuroendocrine carcinoma (NEC) in the sigmoid colon. Prior biopsies obtained from multiple sites of the colon during endoscopic surveillance showed minimal inflammatory changes and no sign of dysplasia. Patients with inflammatory bowel disease (IBD) are at increased risk of colorectal malignancies, and adenocarcinoma is the most common type of colorectal neoplasm associated with ulcerative colitis and Crohn's disease, but other types of epithelial and nonepithelial tumors have also been described in IBD. NECs arising in the setting of ulcerative colitis are very rare and are reported as anecdotic findings. We describe the clinicopathological features of an IBD-related NEC and review the previously reported cases.

Long-Standing Ulcerative Colitis May Trigger a Multilineage Cancerization Field

“…Consistently with the observation of Bolzacchini et al, 1 our report confirms that longstanding colonic inflammation may trigger both the neuroendocrine oncogenesis 2 (as supported by the occurrence of poorly differentiated NEC), and the "traditional type colonic adenocarcinoma," as more usually observed in association with UC. 3…”

“…A recent article by Bolzacchini et al 1 reported the 15th case of neuroendocrine carcinoma (NEC-G3) arising from long-standing ulcerative colitis (UC). This report adds an additional 16th case of poorly differentiated large cell carcinoma to the detailed list of neuroendocrine malignancies (including both NEC and mixed neuroendocrine-non-neuroendocrine neoplasms [MiNEN]) as provided by the authors.…”

Mixed large cell neuroendocrine carcinoma and squamous cell carcinoma of the colon: detailed molecular characterisation of two cases indicates a distinct colorectal cancer entity

“…Possible causes for this squamous colonic carcinoma are chronic inflammation in the context of ulcerative colitis, schistosomiasis, human papillomavirus infection, abdominal sinus or fistula, or pelvic radiation [4,21]. Associations between neuroendocrine carcinomas or MiNEN of the colon and ulcerative colitis, as seen in case 1, are sporadically reported [22,23]. The combination of the two neoplasm types in the colorectal region is highly exceptional and so far very little is known about the underlying mutational landscape of such combined carcinomas.…”

Looking into digestive mixed neuroendocrine – nonneuroendocrine neoplasms: subtypes, prognosis, and predictive factors

“…Interestingly, rare cases associated with either Crohn's disease or ulcerative colitis have been reported. [40][41][42] It worth noting that mixed neoplasms characterised by adenoma and NET can be encountered in the colon, and they have an excellent prognosis, thus not requiring large surgical resection. 23 Three main pathogenetic mechanisms have been described for colonic adenocarcinomas.…”