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- W2920902046 abstract "Patients with IBD have an increased risk of colorectal cancer compared with the general population due to chronic inflammation. Extraintestinal malignancies are thought to occur in the setting of immunosuppression. We present a rare case of concurrent colon adenocarcinoma and small cell neuroendocrine tumor in a young patient with ulcerative colitis. A 26-year-old male with pan-ulcerative colitis of 16 years duration, not maintained on IBD-directed therapy, presented with a 3 month history of weakness, abdominal pain, 25-lb weight loss, and hematochezia. Abdominal CT on presentation revealed a 10 cm mass abutting the hepatic flexure and pancreas; it appeared to invade the portal vein and gallbladder (Figure 1). Colonoscopy revealed severe pancolitis with a malignant-appearing, intrinsic severe stenosis at the hepatic flexure (Figure 2). Biopsies of the colonic stricture demonstrated invasive moderately differentiated adenocarcinoma. EUS revealed a mass in the porta hepatis; FNA returned positive for poorly differentiated small cell neuroendocrine carcinoma (NEC). Features of the NEC included brisk mitotic activity (up to 35 mitosis per 10 high power field) and positive staining for synaptophysin and CAM5.2; Ki-67 staining approached 100% (Figure 3). PET-CT showed hepatic metastases. The patient was treated with steroids, cisplatin, and etoposide targeted at the NEC, with plans for possible future surgical resection. Unfortunately, the patient developed intra-abdominal obstruction and abscess, and transitioned to comfort care. He died 57 days from presentation. Poorly differentiated colonic NECs associated with UC are exceedingly rare and only 15 cases are reported. Their discovery portends an extremely poor prognosis for the patient, and our patient's sudden decline after years of stability were likely related to the highly aggressive nature of the NEC tumor. Similar to our patient, several cases in the literature reported pathology consistent with mixed adenoneuroendocrine carcinoma (MANEC), which require that adenocarcinoma account for at least 30% of the tumor mass. We were unable to define our patient's tumor as MANEC as it was not surgically resected, although he received appropriate treatment with cisplatin and etoposide. The interrelationship between NEC, UC, and adenocarcinoma is yet to be elucidated.2040_A Figure 1. Computer tomography showing A 10 cm mass with central necrosis abutting the hepatic flexure and pancreas.2040_B Figure 2. A. Colonoscopy reveals severe continuous inflammation, sigmoid colon shown. B. Malignant appearing stenosis at the hepatic flexure.2040_C Figure 3. A.Invasive, moderately differentiated colon adenocarcinoma. B. Small cell NEC. C. Small cell NEC with significant Ki 67 staining. D. Small cell NEC with positive synaptophysin staining." @default.
- W2920902046 created "2019-03-22" @default.
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- W2920902046 date "2018-10-01" @default.
- W2920902046 modified "2023-09-27" @default.
- W2920902046 title "IBD-Associated Malignancies: An Unusual Case of Concurrent Colon Adenocarcinoma and Small Cell Neuroendocrine Tumor" @default.
- W2920902046 doi "https://doi.org/10.14309/00000434-201810001-02040" @default.
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