Department of Nuclear Medicine, Changhai Hospital, Second Military Medical University.
Department of Pathology, Shuguang Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Clin Nucl Med. 2019 May;44(5):420-423. doi: 10.1097/RLU.0000000000002495.
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.
遗传性平滑肌瘤病和肾细胞癌(HLRCC)综合征是一种罕见的常染色体显性疾病,其特征为皮肤和子宫平滑肌瘤以及肾细胞癌(RCC)的发展。大约 20%至 30%的 HLRCC 综合征患者发生类似于散发性 2 型乳头状 RCC 的 RCC,这种 RCC 具有侵袭性,预后不良。关于 FDG PET/CT 在 HLRCC 综合征相关 RCC 中的临床应用的信息有限。我们报告了一例 HLRCC 综合征相关的 RCC 病例,该病例在原发性 RCC 和腹膜后淋巴结转移中均显示出高 FDG 摄取。此外,该患者还有 2 个高代谢性的子宫平滑肌瘤。