Pseudomyogenic hemangioendothelioma/epithelioid sarcoma-like hemangioendothelioma of the lower limb: report of a rare case

Diagnostic Pathology
Chuifeng FanEn-Hua Wang

Abstract

Pseudomyogenic hemangioendothelioma is an extremely rare soft tissue tumor, also named as epithelioid sarcoma-like hemangioendothelioma, which occurs more frequently in young adult males. It was originally recognized as a variant of epitheloid sarcoma, however it is now concluded as a distinctive, rarely metastasizing endothelial neoplasm. We present a case of pseudomyogenic hemangioendothelioma in the lower limb in a 49-year-old female who has a long course of disease and suffered from twice local recurrences and lymph node affection of the tumor. The mass was subcutaneous and the margins were ill-defined. Morphologically, the tumor cells show diversity, composed of large spindle cells and round cells, both with abundant eocinophilic cytoplasm, mimicking rhybdomyoplasts and epitheloid cells respectively. The tumor cells show diffuse strong expression of Factor VIII, Fli-1, INI-1, vimentin, MDM2, and CDK4, local expression of CD31, AE1/AE3, EMA and P63, and no expression of CD34, S-100, actin-sm, desmin, MyoD1, and HMB45. Based on these information, this case is diagnosed as pseudomyogenic hemangioendothelioma after ruling out the main differential diagnosises including epithelioid sarcoma, malignant peripheral nerve sheath tum...Continue Reading

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Citations

Feb 7, 2018·The American Journal of Dermatopathology·Emily RaftopoulosWalter Rush
Dec 5, 2019·Vascular Medicine·John R Bartholomew, Michael Tran
Feb 29, 2020·Clinical Nuclear Medicine·Nicolas PlouznikoffRaymond Lambert
Jan 26, 2020·Virchows Archiv : an International Journal of Pathology·Yuefang SunJian Wang
Mar 27, 2021·Journal of Chest Surgery·Abdul-Rahman Fadi DiabAli Mousa Alabbadi

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