Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features

Aims: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. Methods and results: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA)+, muscle specific actin (MSA)+, vimentin+, CD31-, CD34-, CD21-, CD8-, CD68(2/3 cases), S100-, while the lining cells were CD34+, vimentin+ and SMA-, with variable CD31 and factor VIII related antigen expression. Elongated SMA+, MSA+ cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. Conclusions: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)- angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen.