Hemangiopericytoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: HPC; Solitary Fibrous Tumor

Overview

Hemangiopericytoma (HPC) is a type of soft tissue vascular sarcoma that originates in the pericytes in the walls of capillaries. Typically, hemangiopericytomas have large vessels especially located at its periphery, and commonly involve the lower limbs (35% of cases), especially the thigh, pelvis andretroperitoneum (25%).

Historical Perspective

• Hemangiopericytoma was first described by Arthur Purdy Stout and Margaret Ransone Murray, two American pathologists, in 1942.

Classification

• Hemangiopericytoma may be classified into 4 groups:

• Hemangiopericytoma of the spleen
• Dural haemangiopericytomas
• Myopericytoma
• Infantile myofibromatosis
• Sinonasal HPCs.

Pathophysiology

• Hemangiopericytomas arise from pericytes, which are normally involved in the formation of capillaries walls.
• The NF2 gene mutation has been associated with the development of hemangiopericytoma.
• On gross pathology, characteristic findings of hemangiopericytoma, include:

• Poorly demarcated

• On microscopic histopathological analysis, characteristic findings of hemangiopericytoma, include:

• Large polygonal (ganglion-like) and/or spindled cells with:
• Vesicular (clear) nuclei
• Prominent nucleoli
• +/-Binucleation
• Loose myxoid stroma
• Frequent typical mitoses

Causes

• There are no established causes for hemangiopericytoma.

Differentiating Hemangiopericytoma From Other Diseases

• Hemangiopericytoma must be differentiated from other diseases that cause a slow growing painless mass, such as:

• Liposarcoma
• Sinovial sarcoma
• Angiosarcoma

Epidemiology and Demographics

• Hemangiopericytoma represents only about 1-2% of all soft-tissue tumors.

Age

• The median age at diagnosis is 45 years.
• Hemangiopericytoma is more commonly observed among patients aged between 45 to 50 years old.
• Hemangiopericytoma is less commonly observed among children.

Gender

• Hemangiopericytoma affects men and women equally.

Race

• There is no racial predilection for hemangiopericytoma.

Risk Factors

• There are no associated risk factors in the development of hemangiopericytoma.

Natural History, Complications and Prognosis

• The majority of patients with hemangiopericytoma are asymptomatic.
• Early clinical features include painless mass, or slow enlargement.
• If left untreated, the majority of patients with hemangiopericytoma may progress to develop bone invasion.
• Common complications of hemangiopericytoma, may include:

• May profusely bleed during resection.

• The mean survival rate of patients with hemangiopericytoma is approximately 13 years.

• 1-year survival rate is 95%
• 5-year survival rate is 82%
• 10-year survival rate is 60%
• 20-year survival rate is 23%

Diagnosis

Symptoms

• Hemangiopericytoma is usually asymptomatic.
• There are no hallmark symptoms of hemangiopericytoma.

Physical Examination

• Patients with hemangiopericytoma usually are well-appearing.
• Physical examination may be remarkable for:

• Palpable mass
• No tenderness
• Located in the extremities (thigh, axilla or pelvis)

Laboratory Findings

• There are no specific laboratory findings associated with hemangiopericytoma.

Imaging Findings

• MRI is the imaging modality of choice for hemangiopericytoma.
• On MRI, characteristic findings of hemangiopericytoma, include:

• Solitary fibrous tumor
• Demonstrates a deep soft-tissue mass
• Heterogenous high signal intensity on coronal STIR imaging.

Treatment

Medical Therapy

• The mainstay of therapy for hemangiopericytoma is adjuvant chemotherapy and radiation therapy.

Surgery

• Surgery is the mainstay of therapy for hemangiopericytoma.
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of hemangiopericytoma.
• [Surgical procedure] can only be performed for patients with [disease stage] hemangiopericytoma.

Prevention

• There are no primary preventive measures available for hemangiopericytoma.

• Effective measures for the primary prevention of hemangiopericytoma include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with hemangiopericytoma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].