• Xian Li, Xiaowei Ma, Jingwen Hao, Chuning Dong, and Yunhua Wang.
• Department of Nuclear Medicine/PET Center, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
• Medicine (Baltimore). 2021 Apr 2; 100 (13): e25065e25065.

RationaleEpithelioid hemangioendothelioma (EHE) is a rare low-to-intermediate grade malignant vascular neoplasm. We report a primary splenic EHE with diffused metastasis who underwent 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT). Our case emphasizes that EHE should be considered a differential diagnose of 18F-FDG-avid splenic malignancies.Patient ConcernsA 39-year-old man presented with abdominal distension and chest distress for 20 days and lumbago for 2 days. Transthoracic echocardiography suggested a large amount of pericardial effusion. Contrast-enhanced CT imaging showed splenomegaly with multiple low-density nodules with ring enhancement. A large irregular mass was also found in the right superior mediastinum with heterogeneous density and enhancement. 18F-FDG PET/CT imaging revealed splenomegaly, filled with intense hypermetabolic nodules and masses. And multiple regions of increased 18F-FDG uptake were observed in the mediastinum, left pleura, and bones.DiagnosisEHE of the spleen.InterventionsHalf a month after the diagnosis was confirmed, the patient then underwent chemotherapy, Docetaxel combined with carboplatin, and Endu were administrated every 3 weeks.OutcomesDuring the 6-month follow-up period, the patient has finished 4 cycles of chemotherapy combined with 2 months of targeted drug. Efficacy assessment is partial remission through CT imaging, and clinical symptoms of patient improved significantly.LessonsPrimary splenic EHE is extremely rare, especially with diffuse systemic metastasis. Our report suggested that EHE should be considered a differential diagnosis of 18F-FDG-avid splenic malignancies. Furthermore, 18F-FDG PET/CT plays critical role in staging and accessing disease extent of EHE.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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