Pseudomyogenic Hemangioendothelioma

Abstract

First described in 2003 as epithelioid-sarcoma-like hemangioendothelioma and later in 2011 as pseudomyogenic hemangioendothelioma, this rare vascular tumor is of intermediate malignant potential. It was officially included for the first time in the most recent World Health Organization's Classification of Tumours of Soft Tissue and Bone. It typically affects young adults with a predilection for the distal lower extremity. This tumor lacks morphologic features of vascular differentiation but shows unequivocal evidence of such differentiation with the use of relevant immunohistochemical stains such as FLI1, ERG, and CD31. Pseudomyogenic hemangioendothelioma can be diagnostically challenging and might be confused with other tumors, such as epithelioid sarcoma. In this review we discuss the clinical, morphologic, and immunohistochemical features of this tumor with particular emphasis on the differential diagnosis. Salient molecular and prognostic features are also reviewed.

Pseudomyogenic hemangioendothelioma of bone with rare WWTR1‐FOSB fusion gene: Case report and literature review

“…PMHE is a locally aggressive tumor with high risk for local recurrence following surgical excision; however, it is a rarely metastasizing neoplasm 1‐4 . It is essential to differentiate this tumor from other epithelioid soft tissue neoplasms including epithelioid hemangioma (EH), epithelioid hemangioendothelioma (EHE), epithelioid sarcoma (ES), and epithelioid angiosarcoma (EAS).…”

“…Pseudomyogenic hemangioendothelioma (PMHE) is a rare endothelial neoplasm of intermediate malignant potential that usually arises in the soft tissues of the lower and upper extremities 1‐4 . Its occurrence in bone is a rare event.…”

A rare case of pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) of the breast first misdiagnosed as metaplastic carcinoma by FNAB and review of the literature

“…A total of 82 patients with PHE/ESH have been reported, with follow-up available for 61 (74%); only 3 patients (5%) developed distant metastasis at 4, 8.5 and 16 years after the initial diagnosis. Almost half of patients had local recurrence or new lesions in the same region as the initial tumor, especially in the first year after diagnosis [18]. The efficacy of treatment is only partially known and still the object of study.…”

Successful Treatment of Recurrent Epithelioid Sarcoma-like Hemangioendothelioma with Mohs Micrographic Surgery

“…ES-H is an uncommon low-grade vascular tumor most commonly presenting as a deep-seated firm dermal to subcutaneous nodule with occasional involvement of the overlying epidermis. [1][2][3] The most common primary sites are in the lower extremities, although it can involve a variety of locations including bony structures. 3,4 Microscopically, ES-H lesions demonstrate sheets and fascicles of round, epithelioid cells infiltrating soft tissues.…”

“…[1][2][3] The most common primary sites are in the lower extremities, although it can involve a variety of locations including bony structures. 3,4 Microscopically, ES-H lesions demonstrate sheets and fascicles of round, epithelioid cells infiltrating soft tissues. These tumor cells typically have abundant strongly eosinophilic cytoplasm, large vesicular nuclei, prominent nucleoli, and lack morphologic signs of vascular differentiation.…”