Primary angiosarcoma of the spleen.

BACKGROUND AND OBJECTIVES

Primary splenic angiosarcoma is a very rare and aggressive neoplasm with a high metastatic rate and dismal prognosis. Since only a few cases have been adequately reported in the medical literature, we report here a further six cases.

METHODS

The records of all cases of primary splenic angiosarcoma treated at Chang Gung Memorial Hospital from April 1991 to July 2004 were retrospectively reviewed.

RESULTS

Of the six cases identified (three men and three women; range, 7-69 years; median, 44 years), four presented with palpable abdominal masses or left upper quadrant abdominal pain. Other systemic symptoms, such as bleeding gums, fatigue, fever, body weight loss, and gastrointestinal bleeding were noted. An abnormal hematogram was found in five patients, with all of these five showing anemia, and three thrombocytopenia. Splenomegaly was present in all patients. Hemoperitoneum due to splenic rupture was noted in one patient. Three patients had distant metastasis to the liver (n = 2), bone (n = 1), bone marrow (n = 1), and small bowel (n = 1) at diagnosis. Liver (n = 3), bone (n = 1), and bone marrow (n = 1) metastases were found in four patients after initial therapy. Five of the six cases underwent a splenectomy, one underwent partial hepatectomy, and one received chemotherapy. The respective 1-, 3- and 5-year survival rates were 60%, 40%, and 40%, and the median survival time was 36 months. One patient was disease-free 162 months after splenectomy.

CONCLUSIONS

The clinical presentations of splenic angiosarcoma were similar to those of previous reports apart from the higher rate of splenomegaly observed in this study. In contrast to reported pediatric cases, our patient achieved long-term disease-free survival after splenectomy alone.