Multifocal primary pseudomyogenic hemangioendothelioma of bone managed with denosumab

Pseudomyogenic hemangioendothelioma (PMH) is a rare tumor of vascular origin with intermediate malignant potential which commonly presents as a subcutaneous and soft-tissue mass with or without concurrent bone involvement. However, PMH presenting as primary multifocal bone lesions is rare. Histomorphologically, it mimicks other epithelioid tumors and cytokeratin expression in PMH can prompt an erroneous diagnosis of metastatic carcinoma, especially in an elderly patient. Diligent histopathological examination and judicious immunohistochemistry panel can guide to the correct diagnosis. Due to its rarity, the optimal therapeutic strategy has not been established till date. We present a rare case of PMH of primary bone with multifocal bony disease in a 23-year-old male who presented with severe bone pains. The patient has been managed with four weekly denosumab, and the disease is stable with symptomatic relief after 6 months.