The case of a 15-year-old boy with a severe swelling of the 2nd toe in the left foot and a large swelling of the left inguinofemoral region is described. Histologic examination of the foot lesion showed a typical epithelioid hemangioma of bone that extended into the soft tissues, with a secondary location in the groin, suggestive of lymph node involvement. Subsequently, imaging also revealed swelling of iliac and para-aortic lymph nodes, which probably indicates further lymph node spread.. Besides the fact that epithelioid hemangioma of the toe has not been reported yet, our case showed a non-continuous localization, most likely in draining lymph nodes. This finding suggests metastatic disease, an event that is extremely rare for epithelioid hemangioma. As such, epithelioid hemangioma might be comparable to another benign vascular tumor, the retiform hemangioendothelioma, which occasionally metastasizes to the lymph nodes without systemic spread.
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