Posters— Surgical Treatment of Sarcomas

HEMANGIOPERICYTOMA OF BONE: THE RIZZOLI EXPERIENCE

L. Campanacci, N. Fabbri, D. Vanel, M. Mercuri Dep. of Musculo Skeletal Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy

Hemangiopericytoma is a rare malignant vascular tumor, originating from the perivascular cells. Usually arising in the soft tissues, it is extremely rare in the skeleton. We coudn’t t find large series reports in the literature.

Treatment is surgery, sometimes associated to radiation and/or chemotherapy. At the Rizzoli Institute, since September 1900 up to date, 24855 cases of bone and soft tissue tumors and pseudotumoral lesions are filed. Out of 6434 cases of malignant bone tumors (excluding metastases), only 13 cases of hemangiopericytoma primary of the bone were observed (2 of which consultations).

There were 4 males and 9 females, aged 8 to 79 years (average 41). Pelvic bones and/or lumbo-sacral spine were involved in 6 cases, a rib in 1, upper and lower limbs in 3 cases each (femur in 2, fibula in 1, and humerus in 3). In 2 cases the lesion involved two adjacent bones.

Symptoms were always present since a long period of time (from 6 to 60 months, average 25,5). Radiologically all cases presented as purely ostelitic, containing trabecular septae, with expanded cortical bone; in 10 cases a soft tissue mass was also present. In 2 cases small osteolitic images distal to the primary lesion were observed; 2 cases presented with a pathological fracture.

Among the 11 cases treated at our Institution, 5 were managed with surgery alone, 1 with surgery and radiation-therapy; 3 patients were treated by chemo- and radiation-therapy (surgery was not possible at presentation). One case (8 years old girl) was cured with chemotherapy alone.

Six patients died of the disease (all developed lung metastases, 1 a local recurrence) after 5 months to 6 years; 5 are continuous disease free at 4 to 21 years of follow-up.

Hemangiopericytoma still represent a difficult issue for diagnosis and treatment, its course can be rapidly aggressive, and lung metastases can appear even after years; the role of chemotherapy is still uncertain in improving the outcome. Delaied diagnosis has an ominous prognosis.