Department of Urology, University Hospital, University of Zurich, Frauenklinikstrasse 10, 8091, Zurich, Switzerland.
Department of Pathology of Molecular Pathology, University Hospital, University of Zurich, Zurich, Switzerland.
J Cancer Res Clin Oncol. 2020 Nov;146(11):2829-2841. doi: 10.1007/s00432-020-03326-3. Epub 2020 Jul 27.
Testicular granulosa cell tumors (tGrCT) are rare sex cord-stromal tumors. This review aims to synthesize the available evidence regarding the clinical presentation and clinicopathological characteristics, treatment and outcomes.
We conducted a systematic literature search using the most important research databases. Whenever feasible, we extracted the data on individual patient level.
From 7863 identified records, we included 88 publications describing 239 patients with tGrCT. The majority of the cases were diagnosed with juvenile tGrCT (166/239, 69%), while 73/239 (31%) patients were diagnosed with adult tGrCT. Mean age at diagnosis was 1.5 years (± 5 SD) for juvenile tGrCT, and 42 years (± 19 SD) for adult tGrCT. Information on primary treatment was available in 231/239 (97%), of which 202/231 (87%) were treated with a radical orchiectomy and 20/231 (9%) received testis sparing surgery (TSS). Local recurrence after TSS was observed in 1/20 (5%) cases. Metastatic disease was never observed in men with juvenile tGrCT but in 7/73 (10%) men with adult tGrCT. In 5/7 men with metastatic tGrCT, metastases were diagnosed at initial staging, while 2/7 patients developed metastases after 72 and 121 months of follow-up, respectively. Primary site of metastasis is represented by the retroperitoneal lymph nodes, but other sites including lungs, liver, bone and inguinal lymph nodes can also be affected. In comparison with non-metastatic adult tGrCT, men with metastatic adult tGrCT had significantly larger primary tumors (70 vs 24 mm, p 0.001), and were more likely to present with angiolymphatic invasion (57% vs 4%, p 0.002) or gynecomastia (29% vs 3%, p 0.019). In five out of seven men with metastatic disease, resection of metastases or platinum-based chemotherapy led to complete remission.
Juvenile tGrCT represent a benign entity whereas adult tGCTs have metastatic potential. Tumor size, presence of angiolymphatic invasion or gynecomastia represent risk factors for metastatic disease. The published literature supports the use of testis sparing surgery but there is only limited experience with adjuvant therapies. In the metastatic setting, the reviewed literature suggests that aggressive surgical and systemic treatment might cure patients.
睾丸支持细胞瘤（tGrCT）是一种罕见的性索-间质肿瘤。本综述旨在综合现有关于其临床表现和临床病理特征、治疗和结局的证据。
我们使用最重要的研究数据库进行了系统的文献检索。只要可行，我们就会从个体患者水平提取数据。
从 7863 条已确定的记录中，我们纳入了 88 篇描述 239 例 tGrCT 患者的文献。大多数病例被诊断为青少年 tGrCT（166/239，69%），而 73/239（31%）的患者被诊断为成人 tGrCT。青少年 tGrCT 的诊断平均年龄为 1.5 岁（±5 标准差），成人 tGrCT 的诊断平均年龄为 42 岁（±19 标准差）。239 例患者中，有 231 例（97%）的患者可获得原发性治疗信息，其中 202 例（87%）接受根治性睾丸切除术，20 例（9%）接受保留睾丸手术（TSS）。在接受 TSS 治疗的 20 例患者中，有 1 例（5%）发生局部复发。青少年 tGrCT 患者从未发生过转移性疾病，但 73 例成人 tGrCT 患者中有 7 例（10%）发生了转移性疾病。在 5/7 例转移性 tGrCT 患者中，转移在初始分期时即被诊断，而 2/7 例患者在 72 和 121 个月的随访后分别出现转移。转移的原发部位为腹膜后淋巴结，但其他部位包括肺、肝、骨和腹股沟淋巴结也可能受到影响。与非转移性成人 tGrCT 相比，转移性成人 tGrCT 的男性患者原发肿瘤明显更大（70 毫米 vs 24 毫米，p 0.001），更有可能出现血管淋巴管侵犯（57% vs 4%，p 0.002）或男性乳房发育（29% vs 3%，p 0.019）。在 7 例转移性疾病患者中，有 5 例患者通过转移灶切除术或铂类化疗实现了完全缓解。
青少年 tGrCT 是一种良性肿瘤，而成人 tGrCT 具有转移潜能。肿瘤大小、存在血管淋巴管侵犯或男性乳房发育是发生转移的危险因素。已发表的文献支持保留睾丸手术的应用，但辅助治疗的经验有限。在转移性疾病中，本综述中的文献表明，积极的手术和系统治疗可能治愈患者。
