Composite Hemangioendothelioma

Abstract

Eight cases of a previously uncharacterized vascular neoplasm, showing varying combinations of benign, low-grade malignant, and malignant vascular components are described. Seven tumors occurred in the dermis and/or subcutis and one occurred in the oral submucosa. The patients were all adults with a median age of 39.5 years (range, 21-71 years). Five patients were men. The tumors arose predominantly in the hands and feet, and the lesions were usually of several years duration. The tumors were composed of a complex admixture of histologic components that varied from tumor to tumor, such that no two tumors looked precisely the same. This was due to variation in the proportions of each component as well as the manner in which each component was distributed throughout each lesion. The predominant histologic components were epithelioid hemangioendothelioma (HE) and retiform HE, which were each present in seven of the tumors. Areas of spindle cell HE were identified in four lesions. Angiosarcoma-like elements were identified in seven tumors. One of the tumors was associated with an arteriovenous malformation and one was associated with an area of lymphangioma circumscriptum. Of six cases with follow up (median duration, 6.5 years), three have recurred locally and, to date, only one has metastasized. We think composite HE is best regarded as a low-grade malignant vascular neoplasm, and the available data suggest that it behaves more favorably than conventional angiosarcoma. The existence of these composite lesions has led to careful reexamination of the concept of HE. The term HE, in that it is currently synonymous with a low-grade malignant vascular tumor, should be reserved for lesions that have true metastatic potential, albeit with low frequency.

Angiosarcoma Arising in a Patient with a 10-Year-Old Hemangioma

“…However, our case is composed mostly of benign hemangioma with smaller areas of epithelioid hemangioendothelioma and an area of high grade angiosarcoma. This does not correlate with the prior descriptions of composite hemangioendotheliomas, which have only described areas of low-grade angiosarcoma [ 12 ]. In addition, it is currently unclear whether a composite hemangioendothelioma arises de novo or as a step-wise process from a precursor lesion.…”

“…The second possibility is a composite hemangioendothelioma. This rare entity was first described by in 2000 by Nayler et al [ 12 ]. So far, there have been approximately 30 cases of composite hemangioendothelioma reported in the literature [ 13 ].…”

“…This lesion occurs most frequently on the extremities but has been reported in the head and neck and kidney and spleen as well [ 14 , 15 ]. This lesion is usually composed of a mixture of epithelioid hemangioendothelioma, retiform hemangioendothelioma, spindle cell hemangioendothelioma, and low-grade angiosarcoma-like areas [ 12 ]. However, our case is composed mostly of benign hemangioma with smaller areas of epithelioid hemangioendothelioma and an area of high grade angiosarcoma.…”

Epithelioid Haemangioendothelioma of the mandibular gingiva: case report and literature review

“…This combination of reteform haemangioendothelioma and epithelioid haemangioendothelioma is now considered a distinct sub-type termed composite haemangioendothelioma. These lesions do occasionally metastasize but most commonly seem to occur in the skin and have a clinical course towards the more benign end of the spectrum [4] . A wider soft-tissue excision and curettage of the lingual gingivae between the LR2 and the LL4 and the buccal gingivae between LR1 to the LL3 was performed by an oral surgeon, within an excision margin of 3 mm.…”

Hemangioendothelioma arising from the spleen: A case report and literature review

“…The term ‘hemangioendothelioma’ (HE) has been used to describe a heterogenous group of vascular neoplasms, which are intermediate between benign and malignant tumors ( 1 ). Cases of HE often progress to rare metastasis and infrequent mortality, primarily arising in the soft tissues of the extremities and organs, including anterior mediastina and liver in adults ( 2 ).…”