Cutaneous composite hemangioendothelioma: case report and review of published reports

Abstract

Composite hemangioendothelioma (CHE) is a rare, locally aggressive, vascular tumor of intermediate-/low-grade malignancy, and is characterized by varying combinations of benign, low-grade malignant, and malignant vascular components. In cutaneous localization, only 22 cases have been reported so far. A new case of CHE of the gluteal region in a 58-year-old man is described. Microscopically, vascular neoplasm, situated mainly within the deep dermis and the subcutaneous fat tissue, was composed of sinusoidal hemangioma, arteriovenous hemangioma, retiform hemangioendothelioma (RHE), and angiosarcoma. An average number of mitoses within the angiosarcomatous component was 10 per 10 high-power fields. Immunohistochemically, the tumor cells were positive for factor VIII-related antigen, CD34, and CD31 and negative for D2-40 and GLUT-1. Ki-67 labeling index was 21%, 1.2%, and 0% in the areas of angiosarcoma, RHE, and sinusoidal hemangioma, respectively. No recurrent disease was noted 3 months after the surgery. The present case displayed the following features previously undescribed in CHE: a novel component of sinusoidal hemangioma and localization at the gluteal region. We also provide review of clinical, histopathological, and immunohistochemical characteristics of cutaneous CHE from the published cases.

Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant

“…Modern Pathology (2017) 30, 1589-1602 the constituent elements of composite hemangioendothelioma (eg, retiform hemangioendothelioma, epithelioid hemangioendothelioma, spindle cell hemangioma, etc) are themselves quite rare and difficult for many pathologists to diagnose. Although a case-by-case review of previously reported examples of composite hemangioendothelioma is beyond the scope of the present study, we suspect that some cases of composite hemangioendothelioma might be better interpreted as representing other benign and malignant vascular tumors, including sinusoidal hemangioma, 8,32 anastomosing hemangioma, 10,33 epithelioid hemangioma with partial spindled growth, 18,34 and conventional angiosarcoma. 9,13,16,20 The distinction of angiosarcoma from composite hemangioendothelioma containing 'foci resembling angiosarcoma' is particularly problematic, as angiosarcoma is a notoriously protean and deceptive lesion that may show hemangioma-like, retiform, and epithelioid features in a single tumor.…”

Cutaneous Vascular Neoplasms of Uncertain Biological Behavior

“…Cases have mostly been reported in adults; however, a large age distribution has been reported in the literature ranging from infancy to young children and teenagers, up to the eighth decade of life [ 52 , 53 , 54 ]. In addition, some studies have reported a potential association of CHE’s with previous radiation therapy and longstanding lymphedema [ 55 , 56 ].…”

“…Immunohistochemical stains generally reflect the individual histologic pattern characteristics staining patterns. Often the tumor show variable expression for vascular endothelial markers, including CD31, CD34, ERG, FLI-1, ET-related gene, and von Willebrand factor [ 54 , 56 , 58 , 59 ].…”

Composite Hemangioendothelioma With Neuroendocrine Marker Expression: Report of a “Paraganglioma-Like” Paravertebral Case

“…Nevertheless, based on the initial series by Nayler et al and subsequent reports, composite hemangioendotheliomas frequently affect the cutaneous or superficial tissue of distal extremities, and sometimes the deep soft tissues in the head and neck, back, mediastinum, bone, lymph node, and visceral organs. 3 -6 The tumors are often associated with preexisting vascular abnormalities and exhibit a high local recurrence rate after excision, with rare lymph node or distant metastasis. 3,4,7 Among the various vascular components, retiform hemangioendothelioma, epithelioid hemangioendothelioma, spindle cell hemangioma, angiosarcoma-like areas, and vascular/lymphatic malformation are more consistently reported.…”