[Malignant hemangiopericytomas in the head and neck area]

Since 1975 we have treated nine patients with a malignant haemangiopericytoma. Three patients died 5, 9 and 14 years later of metastasis in the lung, liver, skeletal system and mastoid. However, five of the patients are still alive. Regular clinical and radiological follow-up is performed, the average period of follow-up being 8.2 years. Four patients suffered local recurrence within 1-5 years. Surgical resection was the primary therapy in five cases, supplemented in two cases by local radiation. Recurrences were treated by surgery on four occasions and six by radiation, but no complete remission was achieved. Chemotherapy (CyVADic regimen) was attempted in three patients with metastases and one patient with a huge local recurrence, but proved to be ineffective. A re-examination of the histological specimen of all primary tumours and recurrences did not reveal a correlation between histological appearance and clinical behaviour. Our experience in the treatment of malignant haemangiopericytoma and the data in the literature suggest that primary resection with a wide safety margin is the therapy of choice. Radiotherapy is recommended for incomplete resection, local recurrence, and metastases and inoperable tumours. Since recurrence and metastasis can occur after many years (up to 7 years in our patients) a lifelong regular follow-up is necessary.