A 29-year-old woman had a history of an upper quadrant abdominal mass for about 6 years. An ultrasound examination revealed splenomegaly containing several cysts. On exploratory laparotomy, multiple cysts were found in the spleen. Because of the extensive involvement of the spleen, splenic salvage could not be performed, and total splenectomy was done. Both morphological and histological features of the removed spleen were typical of cystic lymphangiomatosis.
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