Hemangioendothelioma
Template:DiseaseDisorder infobox
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign or malignant in their activity. They have been described as masses that fall between a hemangioma and angiosarcoma. They are vascular tumors that commonly present with an enlarging mass and have been reported in the head and neck, intestines, lungs, lymph nodes, pleura, retroperitoneum, stomach, and many other body sites. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses.
In hemangioendotheliomas, the cell of origin is the endothelial cell, and they range from benign to frankly malignant lesions.
They typically occur in young patients, especially in the soft tissues of the limbs and are often closely related to veins, from which they are thought to arise. Less frequently they can arise in bone (osseous haemangioendothelioma), in which case they are most often seen in the calvaria, spine or lower limbs.
Types of Hemangioendotheliomas
- Epithelioid
- Kaposiform
- Retiform
- Infantile hemangioendothelioma
Diagnosis
Radiologically they appear the same as angiosarcomas and haemangiopericytomas.
X-rays
Nonspecific soft tissue mass. When involving bone, they are predominatly lytic, and may mimic haemangiomas with a course honeycomb appearance.
Angiography
Dense, well-circumscribed areas of enhancement with early draining veins and shunting. Hemangiopericytoma are described as having a pedicle formed by the arteries supplying the tumor from which vessels branch to encircle the tumor.
CT
Non specific soft tissue mass, with density similar to muscle but demonstrating bright contrast enhancement. CTA may demonstrate large feeding vessels.
MRI
Brightly enhancing soft tissue mass, often hyperintense on T2WI, with prominant flow voids, most marked in hemangiopericytomas.
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References
- Murphy M. et al "Musculoskeletal Angiomatous Lesions" RadioGraphics: July 1, 1995 -- Volume 15, Number 4
External links
- Hemgioendothelioma Support Group
- Maxillofacialcenter
- H.E.A.R.D. Hemangioendothelioma, Epithelioid Hemangioendothelioma And Related vascular Disorders Support Group and International H.E.A.R.D. Registry