3.P.04 TWO CASES OF EPITHELOID HAEMANGIOENDOTHELIOMA WITH BONE INVOLVEMENT

Abstract

Epitheloid haemangioendothelioma is a rare tumour of vascular origin. It is characterised by the appearance of epitheloid endothelial cells and occurs typically in soft-tissue, skin, and liver. Less frequently it is found in bone. The tumour is more often located in the long bones of the lower extremities, and the pelvis than in the upper extremities, vertebral column, and flat bones. The lesion nearly affects all age groups and there is a male predilection.

Case 1: A 71-year old woman had pain in the area of her right hip after a downfall. X-ray showed a lucency of the cortical substance of the right femur. Scintigraphy showed a cortical lesion, oedema of the bone-marrow and an involvement of soft-tissue. Carcinoembryonic antigen, CD 31, and CD 8 were positive. An open biopsy verified an epitheloid haemangioendothelioma. Staging was negative. A wide resection of the proximal femur and reconstruction with a tumour-prosthesis were performed. Four months later the patient had osteolytic metastases of os ilium, os pubis, acetabulum and in the fifth lumbar vertebra. The patient died 8 months after the wide resection of the tumour because of myocardial infarction.

Case 2: An epitheloid haemangioendothelioma of the liver was diagnosed in a 21-year old male patient. Twelve years after the primary tumour the patient had osteolyses of the first cervical vertebra, manubrium sterni, and ribs. An open biopsy verified the metastatic spread. Local radiotherapy was performed. Furthermore the patient developed a destruction of processus spinosus and a pathologic fracture of first thoracic vertebra. The patient died of metastatic disease 2 years later or 14 years after the initial diagnosis.

Epitheloid haemangioendothelioma of bone is a rare tumour and the diagnosis is quite difficult. Metastatic rate is about 20–30% and mortality about 10–20%. As presented in our cases bone involvement could either be attributed to primary haemangioendotheliomas of bone or to metastases of non-osseous forms. As in our cases it has been reported, that predicting prognosis is difcult, however nuclear atypia, mitotic activity, spindling of cells, and necrosis have been reported as negative prognostic factors.