Why the study?
Pseudomyogenic hemangioendothelioma is a rare entity with only around 20 cases of primary osseous involvement reported to date, making awareness critical for diagnosis.
Highlights the rare presentation of pseudomyogenic hemangioendothelioma as a primary osseous lesion, emphasizing the importance of awareness for accurate diagnosis.
Multifocal osseous PHE in adolescents merits inclusion in lytic lesion differentials; extends limited case reports, leaving management questions open.
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.
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Godkhindi et al. (2023) studied this question.
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