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;

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:

Causes

• Hemangiopericytoma may be caused by either [cause1], [cause2], or [cause3].
• Hemangiopericytoma is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
• There are no established causes for hemangiopericytoma.

Differentiating Hemangiopericytoma From Other Diseases

• Hemangiopericytoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of hemangiopericytoma is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of hemangiopericytoma was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop hemangiopericytoma.

• Hemangiopericytoma is more commonly observed among patients aged [age range] years old.
• Hemangiopericytoma is more commonly observed among [elderly patients/young patients/children].

Gender

• Hemangiopericytoma affects men and women equally.

• [Gender 1] are more commonly affected with hemangiopericytoma than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for hemangiopericytoma.

• Hemangiopericytoma usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop hemangiopericytoma.

Risk Factors

• Common risk factors in the development of hemangiopericytoma are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

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

Diagnostic Criteria

• The diagnosis of hemangiopericytoma is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• Hemangiopericytoma is usually asymptomatic.
• Symptoms of hemangiopericytoma may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with hemangiopericytoma usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with hemangiopericytoma.

• A [positive/negative] [test name] is diagnostic of hemangiopericytoma.
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of hemangiopericytoma.
• Other laboratory findings consistent with the diagnosis of hemangiopericytoma include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with hemangiopericytoma.

• [Imaging study 1] is the imaging modality of choice for hemangiopericytoma.
• On [imaging study 1], hemangiopericytoma is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• Hemangiopericytoma may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for hemangiopericytoma; the mainstay of therapy is supportive care.

• The mainstay of therapy for hemangiopericytoma is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

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].