AMR SeriesSelected Case From the Arkadi M. Rywlin International Pathology Slide Series Lymphangiomatosis of the Spleen Associated With Ipsilateral Abdominopelvic and Lower Extremity Venolymphatic Malformations A Case Report and Review of the LiteratureBisceglia, Michele MD*; Chiaramonte, Antonio MD†; Lauriola, Walter MD‡; Galliani, Carlos A. MD§; Musto, Pellegrino MD*; Colacchio, Giovanni MD∥Author Information *IRCCS, Referral Cancer Center of Basilicata, Rionero in Vulture (Pz), Italy †Department of Surgery ‡Division of Vascular and Interventional Radiology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (Fg), Italy ∥Division of Vascular Surgery, IRCCS F. Miulli Hospital, Acquaviva delle Fonti (Ba), Italy §Department of Pathology, Cook’s Children Medical Center, Fort Worth, TX The authors have no funding or conflicts of interest to disclose. Reprints: Michele Bisceglia, MD, Via Santa Chiara, 9, Manfredonia 71043, Foggia, Italy (e-mail: [email protected]). All figures can be viewed online in color at http://www.anatomicpathology.com. Advances In Anatomic Pathology: July 2014 - Volume 21 - Issue 4 - p 291-299 doi: 10.1097/PAP.0000000000000031 Buy Metrics Abstract Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified. © 2014 by Lippincott Williams & Wilkins.