Multifocal intraosseous pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: A rare presentation of an uncommon tumor

Vishwapriya M Godkhindi; Vidya Monappa; Sharada Mailankody; Umesh Velu; Shuiab M V Mohammed; Aisharya Banerjee

doi:10.4103/ijpm.ijpm_995_22

Multifocal intraosseous pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: A rare presentation of an uncommon tumor

Indian J Pathol Microbiol. 2023 Jul 26. doi: 10.4103/ijpm.ijpm_995_22. Online ahead of print.

Authors

Vishwapriya M Godkhindi¹, Vidya Monappa¹, Sharada Mailankody², Umesh Velu³, Shuiab M V Mohammed¹, Aisharya Banerjee¹

Affiliations

¹ Department of Pathology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.
² Department of Medical Oncology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.
³ Department of Radiotherapy and Oncology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.

PMID: 38391317
DOI: 10.4103/ijpm.ijpm_995_22

Abstract

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal neoplasm of intermediate malignant potential showing endothelial differentiation. Around 20 cases of primary osseous PHE have been reported to date. A 16-year-old boy presented with complaints of pain in his right leg. Imaging revealed multifocal intramedullary and cortical-based lytic lesions involving long and small bones. Microscopic examination revealed plump, spindled cells arranged in fascicles and admixed "epithelioid" and "rhabdoid" cells sans vasoformative areas. By immunohistochemistry, the lesional cells were reactive for AE1/AE3, CD31, Erg, Fli1, and SMA, while immunonegative for CD34, myogenin, and S100. Nuclear expression of the INI1/SMARCB1 protein was retained. PHE is a rare entity, more so as a primary osseous lesion; therefore, awareness of the presence of this entity in the bone is the key to making a diagnosis. We discuss its clinicopathological features, differential diagnosis, and an attempt a short review of the literature.