Hemangiopericytoma

Signs and symptoms

Symptoms of hemangiopericytoma vary greatly depending on both tumor stage and affected organs. Most patients report pain and mass-related symptoms, while others also report vascular disease-related symptoms, and some have no symptoms until late in the disease process. Hemangiopericytomas are most commonly found in the meninges, lower extremities, retroperitoneum, pelvis, lungs, and pleura.

Histopathology

Hemangiopericytomas are tumors that are derived from specialized spindle shaped cells called pericytes, which line capillaries.

Diagnosis

Computerized tomography and magnetic resonance imaging are not effective methods for diagnosis of hemangiocytomas. In practice, a presumptive diagnosis is often reached through exclusion of other soft tissue tumors, and a tissue biopsy is required to confirm diagnosis.

Treatment

Depending on the grade of the sarcoma, it is treated with surgery, chemotherapy, and/or radiotherapy. Though surgery is the current standard of care for hemangiopericytomas, metastasis and tumor recurrence occur in more than 30% of patients, in particular recurrence in the pelvis and retroperitoneum and metastasis in bone and lungs. Radiotherapy does not appear to provide a significant survival benefit but is recommended for use in patients with tumors greater than 5 cm in diameter or with inadequate resection margins after surgery. Clinical benefits of chemotherapy in soft tissue tumors remains unclear. However, the combination of surgery and chemotherapy appears to worsen survival in hemangiopericytoma patients.

More research is needed to determine efficacy of different types of treatment.

Epidemiology

In one series, the median age of affected individuals was 45 years, with a 10-year survival rate of 70 percent. In another study, age over 45 and female sex were associated with worse survival rates in hemangiopericytomas.

References

References

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8. (September 2021). "Intracranial solitary fibrous tumor/hemangiopericytoma: tumor reclassification and assessment of treatment outcome via the 2016 WHO classification". Journal of Neuro-Oncology.
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