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Hemangiopericytoma - Skin
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Hemangiopericytoma - Skin

Contributors: Patricia Ting MD, Ben Barankin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed


Hemangiopericytomas (HAPs) are rare, benign, or low-grade sarcomas (ie, connective tissue tumors) originating from pericytes (mesenchymal-derived cells surrounding capillaries and venules). The etiology of HAP is unknown, although chemical carcinogens and radiation may be contributory.

HAP occurs mainly in adults; children are rarely affected. Both sexes are affected equally. Because of the asymptomatic nature of HAP, the diagnosis may be delayed by months or even years, particularly for low-grade and slow-growing lesions. More aggressive forms of HAP exist as well. Morbidity and mortality are attributed to locally invasive disease and hematogenous metastases to the lungs (most common) or the intra-abdominal or retroperitoneal region.

Pediatric Patient Considerations:
HAP is rare in children, accounting for less than 10% all cases. A rare form of infantile HAP (in children less than 1 year old) is considered congenital.

For more information on malignant HAP, see OMIM.


D48.1 – Neoplasm of uncertain behavior of connective and other soft tissue

134335004 – Hemangiopericytoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Angiosarcoma – any tumor of vessel origin; liver angiosarcoma is fatal and associated with vinyl chloride exposure
  • Chondrosarcoma, mesenchymal type – rarely associated with metastases
  • Fibrous histiocytoma – general category for any soft tissue sarcoma not otherwise specified 
  • Hemangioendothelioma 
  • Lymphangiosarcoma – arises in limbs with chronic primary or secondary lymphedema (upper > lower)
  • Solitary fibrous tumor – considered a benign neoplasm
  • Synovial sarcoma – occurs near joints
  • Vascular lesions (eg, vascular malformations or aneurysms) – audible bruits may sometimes be present due to the high vascularity of HAP

Best Tests

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Management Pearls

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Last Updated:06/30/2020
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Hemangiopericytoma - Skin
Hemangiopericytoma : Tumor, Multiple nodules, Red
Clinical image of Hemangiopericytoma
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