Akademik Veri Yönetim Sistemi
36th European Congress of Pathology, Florence, İtalya, 7 - 11 Eylül 2024, cilt.485, sa.1, ss.361, (Özet Bildiri)
Background & objectives: P s e u d o m y o g e n i c hemangioendothelioma(PHE) is a rare vascular tumour primarily affecting young adults. Histologically, PHE exhibits spindled or epithelioid cells with eosinophilic cytoplasm and vesicular nuclei. Diagnosing this tumour can be challenging due to its rarity and morphological similarities with other tumours. Methods: Here we present a case of PHE in an 11-year-old male, featuring multiple intramedullary lesions in the metaphysis of the left distal femur and proximal tibia, alongside additional lesions consistent with nonossifying fibroma. Following diagnosis via TruCut biopsy, excision was conducted, and the sample forwarded for histopathological evaluation. Results: In the biopsy, large epithelioid cells with an eosinophilic cytoplasm and prominent atypical nuclei were observed. Extensive immunohistochemical analysis revealed strong positivity for ERG, occasional positivity for keratin, and weak positive staining for smooth muscle actin. While CAMPTA1 was negative, TFE3 showed focal positivity. Based on both the immunohistochemical findings and morphological features, the diagnosis of PHE was established. Conclusion: In the differential diagnosis, epithelioid morphology of the lesion may prompt consideration of epithelioid sarcoma, rhabdomyosarcoma and epithelioid osteosarcoma. Therefore, due to its rarity, PHE may not be considered, potentially leading to misdiagnosis. Immunohistochemical methods can offer valuable assistance in such cases.