Epithelioid Haemangioma of Bone: A Case Series and Comprehensive Literature Review Reappraising the Diagnostic Classification of All Epithelioid Vascular Neoplasms of Bone

Abstract

Epithelioid vascular neoplasms of the bone are classified by the World Health Organization (WHO) into only two tiers: low-grade epithelioid hemangioma (EH) and a more malignant category including both epithelioid hemangioendothelioma and epithelioid angiosarcoma. The World Health Organization defines bone EH as a locally aggressive neoplasm with no connotation of benign or intermediate malignancy. We reviewed three cases of EH in our lab archives with the perspective of appraising their histomorphological approach toward diagnosis. Patients were in the age range of 15-25 years. The site of the neoplasms ranged from the carpal bones to the metatarsal bones. Histomorphological examination of the lesions showed a nodular growth pattern of a vascular neoplasm without demonstrable vessel origin. The vasoformative area increased from the center to the periphery, with prominent epithelioid morphology of the endothelial cells at the periphery and an associated inflammatory infiltrate comprising eosinophils, lymphocytes, and plasma cells. The growth pattern was diffuse, with extension into the deeper dermis of overlying skin.

Epithelioid Hemangioma Masquerading as a Clavicular Mass: A Diagnostic Conundrum

“…The latter site was observed in our case. Based on recurrence and lymph node involvement, it can be classified as a more malignant or benign condition [ 4 ]. The tumor described in our case was benign in nature.…”

“…The treatment approach for epithelioid hemangioma varies depending on the individual case. In some asymptomatic cases, simple monitoring of the lesion may be sufficient, as it can regress over time [ 4 ]. However, in symptomatic cases, procedures such as curettage, embolization, or excisional biopsy may be performed to mitigate potential complications.…”

Prognostic Factors in Epithelioid Hemangioendothelioma: Analysis of a Nationwide Molecularly/Immunohistochemically Confirmed Cohort of 57 Cases

“…However, the model of Shibayama et al includes the size of multifocal lesions, assuming that a multifocal lesion equals unifocal disease. One could argue that multifocal EHE is early metastatic disease rather than simultaneous independent origin of multiple lesions [ 25 , 26 ]. This is supported by our finding that there is no significant difference in survival between the groups of multifocal and metastatic disease.…”