The clinicopathological spectrum of pseudomyogenic hemangioendothelioma: report of an additional series with review of the literature.

The clinicopathological spectrum of pseudomyogenic hemangioendothelioma: report of an additional series with review of the literature.:

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The clinicopathological spectrum of pseudomyogenic hemangioendothelioma: report of an additional series with review of the literature.

Virchows Arch. 2020 Jan 24;:

Authors: Sun Y, Zhao M, Lao IW, Yu L, Wang J

Abstract

We present here our experience with 24 cases of pseudomyogenic hemangioendothelioma (PMHE) to further delineate its clinicopathological spectrum. There were 18 males and 6 females with a median age of 28 years (range 10~64 years). Most patients presented with erythematous nodules or papules, with or without pain. The majority (63%) occurred in the lower extremities, whereas a minority involved the trunk (25%), upper extremities (8%), and head and neck (4%). Six cases (25%) had a primary bone origin. With physical and radiological examinations, 16 cases (67%) manifested as multifocal disease, involving multiple tissue planes or different bones within the anatomic region. Six cases (25%) involved skin, soft tissue, and bone simultaneously. Histologically, all cases showed features consistent with a PMHE characterized by loose fascicles or sheets of plump spindled to epithelioid cells harboring brightly eosinophilic cytoplasm and vesicular nuclei. In addition, five cases (21%) contained a prominent myxoid matrix, and one case displayed perineural and intravascular invasion. The follow-up information available in 18 patients revealed local recurrence in 4 patients (22%) and persistent disease in 8 patients (44%), respectively. One patient developed bilateral pulmonary metastases which showed significant remission after systemic chemotherapy. None of the patients died of the disease. As the clinical appearance of PMHE can be deceptive, a radiological examination is essential in identifying an insidious multifocal disease. Although PMHE has a predilection for the distal extremities of young males, this rare tumor type could also occur in unusual sites and affect middle-aged adults of both genders. The striking myoid appearance in association with myxoid stromal change may represent a potential diagnostic pitfall. Biologically, PMHE has an indolent clinical behavior, albeit metastatic disease may occur in rare instance.

PMID: 31980959 [PubMed - as supplied by publisher]