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Hemangiopericytoma - Skin
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Hemangiopericytoma - Skin

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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
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Synopsis

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.

Codes

ICD10CM:
D48.1 – Neoplasm of uncertain behavior of connective and other soft tissue

SNOMEDCT:
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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Therapy

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References

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Last Updated: 03/29/2017
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Hemangiopericytoma : Tumor, Multiple nodules, Red
Clinical image of Hemangiopericytoma
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