Composite Hemangioendothelioma of the Submandibular Region

Abstract

Composite hemangioendothelioma (HE) is a rare vascular neoplasm of intermediate malignant potential that predominantly occurs within the dermis or subcutis of the extremities, and occurs in a wide age range. It is locally aggressive with a high rate of local recurrence, and more rarely regional lymph node or distant metastasis. Histologically, it is composed of a complex admixture of benign, intermediate and malignant vascular components. Although composite HE may contain angiosarcoma-like areas, its prognosis is better than that of pure angiosarcoma. We describe a case of composite HE presenting as a submandibular mass in a 43 year-old male, which included areas of prominent 'high grade' epithelioid angiosarcoma. This adds to the range of anatomic sites of these neoplasms, highlights the importance of recognition of the head and neck as a potential site, and emphasizes the importance of accurate diagnosis for correct management (including of long term follow up) and prognostication.

Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant

“…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. 35 This issue is highlighted by a recent case of composite hemangioendothelioma reported by Leen et al, 7 containing elements closely resembling conventional and epithelioid angiosarcoma. Although the (to date) benign clinical course of this patient would seem to support the authors' classification of this particular tumor as composite hemangioendothelioma, we have little doubt that this case would engender significant debate, even among expert soft tissue pathologists.…”

Composite hemangioendothelioma of the forehead and right eye; a case report

“…In most cases, the CHE are solitary lesions that occur as dermal papules or nodules; however, they may be multiple. The colors of these lesions are commonly red, purple, or black violaceous, and they can be scaly or hemorrhagic [ 1 , 2 ]. According to previous studies, there is an association between the CHE and some tumors (such as vascular tumors) and hemangioma [ 3 ] .…”

Case Report: Cutaneous Pleomorphic Lymphangiosarcoma in a Dog Exhibiting Features of Human Composite Hemangioendothelioma

“…HEs are typified by their ability to recur locally; however, their metastatic potential varies based on the pathologic subtype. In particular, composite hemangioendothelioma (CHE) is a rare subtype of low-to-intermediate grade angiosarcoma that microscopically is composed of a mixture of benign, intermediate, and malignant vascular components (6)(7)(8)(9). CHEs demonstrate some histologic variability because their morphology consisting of a combination of at least two HE subtypes, with retiform hemangioendothelioma (RH) and epithelioid hemangioendothelioma (EH) being the most common combination of subtypes.…”