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: email@example.com.