• Atsushi Tomioka, Mitsuhiro Asakuma, Nao Kawaguchi, Koji Komeda, Tetsunosuke Shimizu, Kazuhisa Uchiyama, and Sang-Woong Lee.
• General and Gastroenterological Surgery, Osaka Medical and Pharmaceutical University, Takatsuki City, Osaka, Japan.
• Medicine (Baltimore). 2022 Nov 25; 101 (47): e31642e31642.

IntroductionUndifferentiated pleomorphic sarcoma (UPS) primarily occurs in the soft tissues of the extremities, trunk, and retroperitoneum. As the primary UPS of the spleen (splenic UPS) is extremely rare, to the best of our knowledge, only 19 cases have been reported in English literature. No cases of long-term survival without a local or distant recurrence have been reported.Patient ConcernsWe report the case of a 37-year-old man who was referred to our hospital for a splenic tumor. He had no past medical or relevant familial history. On abdominal computed tomography (CT), a low attenuation solid mass and cystic component with mural calcifications were present at the lower pole of his spleen. The fluorodeoxyglucose-positron emission tomography (CT) indicated it as malignant tumor of the spleen.DiagnosesThe patient's provisional diagnosis was deduced to be angiosarcoma, which was the most common malignant tumor of the spleen.InterventionsAn elective laparoscopic splenectomy was performed, and the histology of the tumor was consistent with UPS (pT1, pN0, cM0, and AJCC8th). No adjuvant therapy was administered.OutcomesTen years have passed since the patient's splenectomy, and he continues to do well, without evidence of local or distant recurrence.LessonsTo the best of our knowledge, this is the first case of long-term recurrence-free survival after surgical management of a splenic UPS. It is probable that radical splenectomy during the disease played the most important role in the patient's long-term survival. Understanding the characteristic findings of a splenic UPS in an abdominal CT may help to diagnose properly.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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