Two Cases of Intraosseous Pseudomyogenic (Epithelioid Sarcoma-Like) Hemangioendothelioma With Unusual Features, Expanding the Clinicopathological Spectrum

Abstract

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (PMHE) is a distinct vascular neoplasm mostly observed in the lower extremities of young adults with characteristic histopathological features. In this article, we present 2 unusual cases of PMHE. Case 1: A 28-year-old male presented with pain and stiffness in his left shoulder. Radiologic examination revealed an expansile, lytic, heterogeneously enhancing, destructive lesion in his left scapula, along with multiple avid marrow lesions in his other bones. Biopsy revealed a cellular tumor composed of plump spindly and epithelioid cells arranged in fascicles and focally, in clusters, admixed with neutrophils and histiocytes, with interspersed many osteoclast-like giant cells and reactive bone. Case 2: A 63-year-old male presented pain and swelling in his right middle finger, with no other lesion elsewhere in his body. Radiologic imaging disclosed a 1.5-cm-sized lobulated, expansile, lytic, destructive lesion in the distal end of the third metacarpal bone of his right hand. Biopsy examination revealed a cellular spindle cell tumor, composed of plump spindly cells, arranged in fascicles, including "tadpole-like" or "strap-like" cells and interspersed inflammatory cells. Immunohistochemically, tumor cells in both cases were positive for AE1/AE3, CD31, and Fli1, while negative for desmin and CD34. INI11 was retained. The presented cases of PMHE, occurring at unusual locations, in an older individual in the second case, along with variable histopathological features, noted in the first case, seem to expand the clinicopathological spectrum of these uncommon neoplasms. Differential diagnoses and review of similar cases are presented.

Keywords: CD31; epithelioid sarcoma–like hemangioendothelioma; immunohistochemistry of bone tumors; intraosseous pseudomyogenic hemangioendothelioma; vascular tumors of bone.