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

Abstract

Aims Pseudomyogenic 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 results We 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. Conclusion To 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.

Cytomorphological features of an unusual case of a multifocal pseudomyogenic (epithelioid sarcoma‐like) hemangioendothelioma in a young adult male

“…Morphologically, the various differential diagnoses of PMHE on smears comprise spindle cell sarcomas, including a spindle cell rhabdomyosarcoma; an ES, spindle cell melanoma, and a sarcomatoid carcinoma, the latter tumor especially in relatively older patients with multiple lesions. A recently reported case of a PMHE involving the breast was initially misdiagnosed as a metaplastic carcinoma by FNAC 7 . Spindle cell sarcomas, as well as angiosarcomas invariably show significant nuclear pleomorphism, mitotic figures, and multiple prominent nucleoli, unlike the present case 8 .…”

Clinicopathological study of pseudomyogenic hemangioendothelioma

“…PHE is rare. It generally occurs in the limbs of young men, especially in the distal lower limbs, but can also occur in various other tissues, including the oral cavity [ 6 , 7 ], chest wall [ 1 , 8 , 9 ], breast [ 10 ], esophagus [ 11 ] and external genitalia [ 1 , 12 , 13 ]. It can involve multiple tissue planes, including the dermis, subcutaneous tissue, bone, and skeletal muscle.…”

Pazopanib as Treatment Option for Pseudomyogenic Hemangioendothelioma: A Case Report

“…Pseudomyogenic hemangioendothelioma (PHE), also known as epithelioid sarcoma-like hemangioendothelioma (ES-H), was first described in 2013 by WHO. It is a vascular tumor biologically behaving like benign hemangioma and malignant angiosarcoma and rarely metastasizes [ 2 , 3 ]. In the fifth edition of WHO classifications of bone and soft tissue tumors published in 2020, PHE was further characterized by a balanced t(7;19) translocation causing a SERPINE1-FOSB fusion [ 4 ].…”