Abstract
AimsPseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) is a rare vascular tumor of intermediate malignancy that commonly occurs in soft tissue of distal extremities of young adults. PHE typically has a multifocal presentation and can involve several tissue planes, including the dermis, subcutis, muscle and bone.Methods and resultsWe present here a unique case of PHE/ESH that arose in the breast as well as a review of the published literature. The initial biopsy was interpreted as a metaplastic carcinoma. However, complete resection largely revealed plump epithelioid cells, and a more spindled cell component was also noted. The cells displayed abundant eosinophilic cytoplasm and central vesicular nuclei arranged in loose fascicles, with a mild, mixed acute and chronic inflammatory infiltrate. Overall, linear membranous staining of CD31 and lack of CD34 expression were highly suggestive of PHE. At the same time, FOSB immunoreactivity was observed, which supported PHE/ESH instead of metaplastic carcinoma. The patient has not shown recurrence in the half year follow up after total mastectomy.ConclusionTo our knowledge, this is the first report of breast involvement in this neoplasm. Recognition of its histopathological features and immunohistochemical reactivity will prevent misdiagnosis of breast lesions.
Highlights
In 2013, Pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) has been accepted as a new vascular fumor entity by WHO
To our knowledge, this is the first report of breast involvement in this neoplasm
We describe a rare case of PHE/Epithelioid sarcoma (ES)-H of the breast first misdiagnosed as metaplastic carcinoma
Summary
In 2013, Pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) has been accepted as a new vascular fumor entity by WHO. PHE/ESH is a distinct, uncommon tumor with an endothelial phenotype that usually arises in soft tissue, and its biological behavior is intermediate between a benign. Ge et al Diagnostic Pathology (2019) 14:79 clinically misdiagnosed with other pathologies [3]. Increased awareness of this new entity is essential for both clinicians and pathologists. We describe a rare case of PHE/ES-H of the breast first misdiagnosed as metaplastic carcinoma. To the best of our knowledge, this case represents the first case of PHE/ESH in breast. The present study demonstrates the diagnostic dilemma due to an exceedingly unusual location of PHE/ESH appearing as breast metaplastic carcinoma
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