Composite hemangioendothelioma

Radiopaedia • CC-by-nc-sa 3.0 • de

Composite hemangioendotheliomas are locally aggressive and rarely metastasizing vascular tumors consisting of different and histologically distinct elements.

Epidemiology

Composite hemangioendotheliomas are very rare and mostly seen in adults. Women are slightly more commonly affected .

Clinical presentation

The typical complaint is a lump with a reddish to purple appearance which has been there for a long time .

Associations

Composite hemangioendotheliomas are seen in patients with lymphedema .

Pathology

Composite hemangioendotheliomas are formed by at least two histologically different vascular tumor components. These parts often resemble retiform or epithelioid hemangioendotheliomas .

Etiology

Chronic lymphedema and irradiation are thought to have a role in the etiology .

Subtypes

Neuroendocrine composite hemangioendothelioma is the only subtype.

Location

Composite hemangioendotheliomas most frequently affect the skin and subcutaneous tissues of the distal extremities or the head and neck area but have been also found in other sites .

Macroscopic appearance

Macroscopically composite hemangioendotheliomas are nodular infiltrative ill-defined lesions .

Microscopic appearance

The microscopic spectrum of composite hemangioendotheliomas consists of a composition of different morphological vascular components and areas including the following :

Neuroendocrine composite hemangioendotheliomas are typically composed of epithelioid and retiform hemangioendothelioma-like parts with a nest-like appearance .

Immunohistochemistry

Immunohistochemistry stains usually express CD31, ERG and FLI1 and can be positive CD34 and D2-40 .

Genetics

Fusions of the PTBP1-MAML2 and EPC1-PCH2 genes have been observed in neuroendocrine composite hemangioendotheliomas .

Radiographic features

MRI

Composite hemangioendotheliomas are vascular lesions with a lobulated and heterogeneous appearance .

Signal characteristics
  • T1: low to intermediate signal intensity
  • T2: high signal intensity
  • T2FS/STIR: high signal
  • T1 C+ (Gd): avid enhancement 

Radiology report

The radiological report should include a description of the following features:

  • form, location and size
  • tumor margins
  • relation to the muscular fascia
  • relationship to bones, tendons and joints
  • relationship to local nerves and vessels

Treatment and prognosis

Due to the rarity of the tumors, there is no clear management strategy as yet. Treatment usually consists of surgical excision. Local recurrence is common and can happen multiple times. Lymph node metastases can occur. Distant metastases are seen in neuroendocrine composite hemangioendotheliomas, which are more aggressive .

History and etymology

Composite hemangioendotheliomas were first described in 2000 by SJ Nayler .

Differential diagnosis

The differential diagnosis of composite hemangioendotheliomas consists of the following: