Background: Composite hemangioendothelioma is the most recently described entity of the hemangioendothelioma (HE) spectrum. To the best of our knowledge, only eight cases of this entity have been hitherto described. All of the previous cases affected adults; local recurrences were observed in three cases, and one case showed lymph node metastasis. We herein describe a new and previously unreported congenital case of this rare vascular tumor, arising on the acral extremity of the left forearm, which was diagnosed when the patient was 23 years old.
Results: The histological examination disclosed a heterogeneous vascular neoplasm composed of retiform HE-like (80%), spindle cell hemangioma-like (15%), cavernous hemangioma-like (approximately 3%), epithelioid HE-like (approximately 2%) areas, and rare foci with an angiosarcoma-like pattern (< 1%). A distinctive and unique finding of the present case was the presence of large granular eosinophilic macrophages filling some vessels of the retiform HE-like areas. A below-elbow amputation was performed. The patient is alive and well, without evidence of residual or metastatic disease 7 years after the treatment.
Conclusions: The authors expand the concept of composite hemangioendothelioma by adding a congenital case and provide a reappraisal of the hemangioendothelioma spectrum.