Hemangiopericytoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Hemangiopericytomas, along with hemangioendotheliomas and angiosarcomas are tumors that arise from vascular structures. A hemangiopericytoma (HPC) is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries (they arise from the cells of Zimmerman, which surround vessels). Both benign and malignant forms are encountered. These tumors are most frequently seen in middle-aged adults.

Typically they have large vessels especially located at its periphery, and commonly involve the lower limbs (35% of cases), especially the thigh, pelvis and retroperitoneum (25%)

Primary osseous lesions are rare.

Diagnosis

Radiologically they appear the same as angiosarcomas and heemangioendotheliomas.

X-rays

Nonspecific soft tissue mass. When involving bone, they are predominatly lytic, and may mimic hemangiomas 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.

Multi Sliced 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 prominent flow voids, most marked in haemangiopericytomas.

References

• Murphy M. et al "Musculoskeletal Angiomatous Lesions" RadioGraphics: July 1, 1995 -- Volume 15, Number 4

External links

• Hemangiopericytoma Personal Story
• Hemangiopericytoma Support Forums Group
• Photo of tissue
• Children's Hospital Boston
• MedPix Images of HPC
• USUHS