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Fistula-associated anal cancer in Crohn's disease (CD) can be challenging to diagnose and treat. Patients with longstanding fistulas in the setting of CD who present with a sudden change in their symptoms should undergo biopsy under anesthesia with extensive sampling, followed by staging imaging. Pelvic magnetic resonance imaging (MRI) can be helpful in identifying the extent of the disease locally. Patients often present in the later stages due to the challenges associated with diagnosing these patients. Two subtypes of this disease include squamous cell carcinoma and adenocarcinoma, and treatment depends on diagnosis. Small sample size and lack of uniform data on treatments make it difficult to say which treatment modalities are optimal, but aggressive combined therapy is likely the best approach for survival. This will include chemotherapy and radiation and often radical resection as well. Despite this, survival is poor, although more recent data suggest that outcomes are improving.

Extrahepatic biliary neuroendocrine tumors (EBNETs) are extremely rare and difficult to diagnose. The vast majority are diagnosed postoperatively on histological evaluation of surgical specimens. Workup and treatment principles are largely based on retrospective series and case reports. Complete surgical resection is the gold standard treatment for these lesions. Here we present a case of a 77-year-old male with a biopsy-proven EBNET incidentally discovered during evaluation for fatty liver disease. Further workup did not show any other suspicious lesions. Resection of the tumor and multiple Roux-en-Y hepaticojejunostomy was performed. Final pathology revealed grade 1, well-differentiated neuroendocrine tumor. This is the third case reported in the literature with a confirmed preoperative EBNET diagnosis based on endoscopic biopsy results. This case highlights the feasibility of preoperative diagnosis of EBNETs and emphasizes the importance of complete surgical resection.

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