Multiple papillary intralymphatic angioendotheliomas in the spleen

ABSTRACT

A 18-year-old man presented with multiple asymptomatic masses in the spleen that had been detected on ultrasonography performed during a physical screening. He had no history of tuberculosis, and was otherwise well. Abdominal MR demonstrated multiple masses with internal stellate scars, which appeared as marked hypointensity on T2WI and contrast-enhanced MR. Most lesions showed inhomogeneous enhancement. The capsular enhancement was also revealed at delay phase. The patient underwent laparoscopic splenectomy. Pathological examination indicated papillary intralymphatic angioendothelioma (PILA), with the following immunohistochemistry results: CK (-), CR (-), ERG (+), CD34 (+), CD31 (+), D2-40 (+), Ki67 (3%+). The patient was feeling well at 6 months of follow-up (AU)