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Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome–Associated Renal Cell Carcinoma Showing High FDG Uptake

Xiao, Zhengguang, MM*; Dong, Aisheng, MD; Wang, Yang, MD

doi: 10.1097/RLU.0000000000002495
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Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is a rare autosomal-dominant disease, characterized by the development of cutaneous and uterine leiomyomas and renal cell carcinoma (RCC). Approximately 20% to 30% of patients with HLRCC syndrome develop RCC resembling sporadic type 2 papillary RCC, which is aggressive and associated with a poor prognosis. Information on the clinical usefulness of FDG PET/CT in HLRCC syndrome-associated RCC is limited. We present a case of HLRCC syndrome-associated RCC showing high FDG uptake in both the primary RCC and retroperitoneal lymph node metastases. In addition, the patient had 2 hypermetabolic uterine leiomyomas.

From the *Department of Radiology, Tongren Hospital, Shanghai Jiao Tong University School of Medicine;

Department of Nuclear Medicine, Changhai Hospital, Second Military Medical University; and

Department of Pathology, Shuguang Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China.

Received for publication December 17, 2018; revision accepted December 30, 2018.

Conflicts of interest and sources of funding: none declared.

Correspondence to: Zhengguang Xiao, MM, Department of Radiology, Tongren Hospital, Shanghai Jiao Tong University School of Medicine, 1111 Xianxia Rd, Changning District, Shanghai 200336, China. E-mail:

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