Pseudoangiosarcomatous squamous cell carcinoma: first case report on penis.

Background: Pseudoangiosarcomatous squamous cell carcinoma, also called pseudovascular, pseudoangiomatoid or adenoid pseudovascular carcinoma, is an uncommon and highly aggressive variant of squamous cell carcinoma. Histologically, it is characterized by proliferation of atypical keratinocytes with acantholysis and formation of pseudovascular spaces, forming anastomosed channels lined with neoplastic cells that invade the dermis. These cells are positive for cytokeratin and vimentin and negative for vascular markers such as CD31 and CD34. There are few reports of this tumor variant in the literature. Skin, breast, lung and vulva involvement have been described. But to the best of our knowledge, no cases involving the penis have been described. This article aims to describe the first case of angiosarcomatous squamous cell carcinoma of the penis. Case Report: The patient presented with a painful lesion in the penis associated with urinary retention. Macroscopic findings exhibited an ulcerative vegetating lesion that involving the entire glans and part of the penile body, as well as infiltration of penile structures and scrotal skin. Microscopy shows atypical proliferation of sarcomatous keratinocyte pattern mimicking vascular spaces. These neoplastic squamous cells were positive for the cytokeratin marker and were negative for the CD31 and CD34 markers. Human papilloma virus biomarkers, p16, E6 protein and PCR, were all negative. Conclusion: This report presents the first reported case of penile pseudoangiosarcomatous squamous cell carcinoma, as an important differential diagnosis.