FAMILIAL MULTIPLE MESOTHELIAL CYSTS OF THE SPLEEN

A B-month-old boy who was diagnosed as having hydrops fetalis at 25 we eks' gestation had severe ascites of unknown origin. At the age 12 mon ths, ultrasonography and computed tomography showed multiple cysts in the spleen that were increasing in size rapidly. Splenectomy resulted in complete disappearance of the ascites. These cysts were diagnosed a s mesothelial cysts because the cell lining of the splenic cysts stain ed positively with alcian blue and cytokeratin. The boy's mother had u ndergone splenectomy for splenic and retroperitoneal lymphangiomas at 4 years of age. Histological reevaluation showed that the lining of he r splenic cysts had the same mesothelial components as her son's. Thei r chromosomal assay showed normal karyotypes. Mesothelial cyst of the spleen appears similar to splenic lymphangioma morphologically; howeve r, bleomycin and OK-432 were not effective. Familial splenic mesotheli al (epidermoid) cysts have been reported in three sets of siblings, bu t this is the first report of their occurrence in a mother and son. (C ) 1995 by W.B. Saunders Company