Fibrofolliculoma with ancient/pseudosarcomatous features.

Fibrofolliculoma is a benign skin lesion that, when multiple, can be part of the Birt-Hogg-Dube syndrome. We report on a case of solitary fibrofolliculoma arisen on the nose of a 63-year-old woman, with peculiar histological and immunohistochemical features. The lesion was characterized by the presence of bizarre multinucleated perifollicular stromal cells, positive for factor XIIIa, in a background of CD34-positive cells, and by a peripheral population of CD34-positive spindle cells organized in fascicles haphazardly infiltrating the deep dermis, and surrounded by scattered factor XIIIa-positive dendrocytes. We consider the bizarre perifollicular cellular component as an 'ancient' feature of fibrofolliculoma, hypothesis corroborated by the co-expression of CD34 and factor XIIIa, whereas the peripheral spindle cell fascicles represent a pseudosarcomatous proliferation of CD34-positive cells, normally surrounding adnexal structures, stimulated by factor XIIIa-positive dendrocytes.

Over 700 Whipples for Pancreaticobiliary Malignancies: Postoperative Morbidity Is an Additional Negative Prognostic Factor for Distal Bile Duct Cancer.

BACKGROUND: Distal cholangiocarcinomas and pancreatic cancers both arise from pancreaticobiliary epithelium. Despite their common origin, there is a possible discrepancy in outcome. We analysed the surgical, pathological and survival outcome of resected distal cholangiocarcinoma compared with pancreatic cancer. METHODS: All cases of resected distal cholangiocarcinoma and pancreatic cancer from 1998 to 2014 were extracted from our database. Outcomes were compared. RESULTS: There were 54 (7.6%) cases of cholangiocarcinoma and 656 (92.4%) pancreatic cancer. Cholangiocarcinoma showed lower T and N stage, lymphatic and perineural invasion (p < 0.05), worse surgical outcome (p < 0.05) and less access to adjuvant therapy if compared with pancreatic cancer (72.7 vs. 83.1%, p = 0.05). Both showed a similar disease-specific survival (35 vs. 29 months, p = 0.3). Independent predictors of prognosis for pancreatic cancer were resection margin, grading, perineural invasion, T and N status, whereas for cholangiocarcinoma were grading and occurrence of POPF. CONCLUSION: Considering a large cohort of resected periampullary cancers, cholangiocarcinoma is extremely rare. An earlier diagnosis is associated with better pathological predictors of outcome but increased postoperative morbidity compared to pancreatic cancer, particularly POPF. Consequent decrease in the access to adjuvant therapy for complicated cholangiocarcinoma might explain why survival is as poor as for pancreatic cancer.