Hemangiopericytoma of the orbit: a clinicopathologic study of 30 cases

The clinical and pathologic features of 30 orbital hemangiopericytomas were reviewed. An effort was made to determine the clincopathologic characteristics that may correlate with the biologic behavior. Histopathologic criteria evaluated included degree of cellularity, nuclear atypia, mitotic activity, tumor size, and the presence of hemorrhage and necrosis. Histopathologically, 16 tumors were classified as benign, five as borderline, and nine as malignant. Follow-up information was obtained in 27 cases (mean duration of follow-up period was five and one-half years). Eight tumors recurred (30 per cent). Five patients died, four with widespread metastases (15 per cent) and one of unrelated causes. Tumors that recurred or metastasized were distributed among all three histologic groups. The interval between the onset of symptoms and metastasis for three of the four lethal tumors was 31 years, 28 years, and 8 1/2 years; in the fourth case it was unknown. For the entire series, the five-year actuarial survival rate was 89 per cent. Although the number of cases is too small to serve as a basis for firm conclusions, the lack of unequivocal correlation between the histologic features and clinical behavior is consistent with the unpredictable behavior of this neoplasm, as concluded by other investigators. Because of the apparent circumscription of most of these tumors, complete surgical excision rather than exenteration is recommended. Metastasis usually develops late in the clinical course of the disease, indicating that long-term follow-up studies are required before a cure can be assumed.