Hemangiopericytoma of the spleen

•Hemangiopericytoma of the spleen is a very rare tumor with only single case reports in the literature.•No standard treatment protocol has yet been proposed.•This article adds five more cases to the 14 reported so far to suggest a standard approach to the treatment of the disease.

IntroductionHemangiopericytoma of the spleen is a very rare tumor, with 14 isolated reports. It was our aim to review our experience and compare it with all the reported cases in an attempt to standardize surgical treatment, adjuvant treatment and follow-up protocol of this infrequent condition.MethodsA consecutive case series study, with a mean follow-up of 44 months. Five patients (mean age, 49 years) underwent simple splenectomy for hemangiopericytoma limited to the spleen followed by adriamycin-based chemotherapy in one patient.ResultsAll the patients are alive and free from disease.ConclusionsFor tumors confined to the spleen, simple splenectomy can be considered curative, without any need for further adjuvant treatment. On review of the medical literature, cure can still be achieved with complete resection of recurrences, when feasible, with adjuvant chemotherapy being also indicated. The slow-growing pattern of the tumor suggests a 10-year follow-up.