Composite Hemangioendothelioma With Neuroendocrine Marker Expression: Report of a "Paraganglioma-Like" Paravertebral Case. (October 2020)

• Record Type: Journal Article
• Authors: Cheuk, W.; Shum, K. S.; Ng, W. K.; Chan, John K. C.
• Is Part Of: International journal of surgical pathology. Volume 28:Number 7(2020)
• Page Start: 759
• Page End: 763
• Publication Date: 2020-10
• Subjects: composite hemangioendothelioma -- paraganglioma -- synaptophysin -- retiform hemangioendothelioma -- D2-40; Pathology, Surgical -- Periodicals; 617.07
• DOI: 10.1177/1066896920924120
• Languages: English
• ISSNs: 1066-8969

Abstract

Composite hemangioendothelioma is a rare vascular tumor morphologically comprising several distinct vascular components and exhibits a borderline malignant potential. We described the case of a 53-year-old female who presented with an infiltrative mass in the paravertebral soft tissue. The tumor showed discrete nests of synaptophysin-expressing epithelioid cells accompanied by rich vasculature, features highly reminiscent of sympathetic paraganglioma. Further analysis revealed areas resembling spindle cell hemangioma, retiform hemangioendothelioma, cavernous hemangioma/lymphangioma, and epithelioid hemangioendothelioma without the myxohyaline matrix in the tumor, and a final diagnosis of composite hemangioendothelioma with synaptophysin expression was made. Critical appraisal of this recently described entity and its possible pathogenic relationship with retiform hemangioendothelioma were discussed.