High-Grade Neuroendocrine Carcinoma of the Urachus - Report of 3 Cases

Publication date: Available online 18 August 2017
Source:Human Pathology
Author(s): Gang Wang, He Huang, Ashish M. Kamat, Arlene Siefker-Radtke, Colin P. Dinney, Patricia Troncoso, Bogdan Czerniak, Charles C. Guo
The majority of urachal malignancies are composed of pure adenocarcinoma with mucin production. Urachal neuroendocrine carcinoma (NEC) is extremely rare, with only a few cases reported in the literature. Here we report 3 cases of urachal NEC, the largest series of this rare disease from a single institution. The patients were young, with a mean age of 27years (range, 23–34). The urachal tumors showed 2 distinct components, high-grade NEC and enteric-type adenocarcinoma. The urachal NECs were composed of small cell carcinoma (n=2) or large cell NEC (n=1). The subsequent resection specimens showed that all the tumors were at advanced Sheldon stages. All 3 patients developed metastases, which were composed of NEC exclusively. Two patients died from disease in 10 and 31months, respectively, and the third patient was alive with widespread metastases with at 21months. Our findings suggest that urachal NEC is an aggressive variant with an overwhelming growth advantage over conventional adenocarcinoma. The presence of high grade NEC in the urachus is associated with poor prognosis.



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