Congenital composite hemangioendothelioma: case report and reappraisal of the hemangioendothelioma spectrum

• Type: Article in International Scientific Journal
• Date: 2002

Authors

Reis, JS (Author) Other; Palva, ME (Author) Other; Lopes, JM (Author) FMUP

Journal

Title: Journal of Cutaneous Pathology

Vol. 29

Pages: 226-231

ISSN: 0303-6987

Publisher: Wiley-Blackwell

Indexing

ISI Web of Knowledge - 40 Citations

Scopus - 54 Citations

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Authenticus ID: P-000-PVP

DOI: 10.1034/j.1600-0560.2002.290405.x

Abstract (EN): 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 (similar to3%), epithelioid HE-like (similar to2%) 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 hemangioendotheliorna spectrum.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 6