Department of Pediatric Urology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Department of Pediatric Urology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
J Pediatr Urol. 2018 Oct;14(5):443.e1-443.e7. doi: 10.1016/j.jpurol.2018.02.030. Epub 2018 Apr 3.
Prepubertal testicular tumors are fundamentally distinct from their postpubertal and adult counterparts. Racial and ethnic differences contribute to the incidence, characteristics, and histological distribution of prepubertal testicular germ cell tumors (PTGCTs).
To elucidate the characteristics and treatment outcomes of PTGCTs in a Chinese cohort.
Data were retrospectively reviewed from consecutive PTGCT patients aged <12 years who received treatment at the current institution from January 2007 to December 2015. Exclusion criteria were: patients aged >12 years, non-primary testicular germ cell tumors, and para-testicular tumors.
A total of 167 patients qualified for the study (Table). The median age at diagnosis was 20 months (range 1-142). Pathology revealed 87 (50.9%) teratomas, 50 (29.2%) yolk sac tumors (YSTs), 33 (19.3%) epidermoid cysts, and one (0.6%) mixed germ cell tumor. Of the teratomas, 70 (80.5%) mature teratomas (MTs) and 17 (19.5%) immature teratomas (ITs) were detected. ITs manifested at a younger age and were larger in size compared with MTs (P < 0.001). Testis-sparing surgery (TSS) was performed for all MTs and 10 ITs. Radical orchiectomy (RO) was performed for seven ITs. No metastatic or local recurrence was detected in benign tumors during follow-up. Obviously elevated alpha fetoprotein (AFP) levels were detected in all the YSTs. Of these, 47 (94%) were diagnosed as Stage I, one (2%) as Stage II, and two (4%) as Stage IV. After RO, recurrence was detected in one Stage I patient with a median follow-up of 46 months. One Stage II and two Stage IV patients died due to tumor progression.
Racial and ethnic differences were detected in PTGCTs. It is believed that the present study is the largest study detailing the clinicopathologic characteristics and treatment outcomes of PTGCTs in a Chinese cohort.
Teratoma was the most common subtype of PTGCTs, followed by YST. ITs manifested as benign behavior and were more likely to be present at a younger age and have a larger tumor size compared with MTs. TSS was reliable for benign lesions. For Stage I YST, RO alone was valid. Salvage chemotherapy was effective and preventative retroperitoneal lymph node dissection may not be necessary for YST. The prognosis of PTGCTs was favorable.
青春期前睾丸肿瘤与青春期后和成人睾丸肿瘤有根本的不同。种族和民族差异导致了青春期前睾丸生殖细胞肿瘤（PTGCT）的发病率、特征和组织学分布不同。
阐明中国队列中 PTGCT 的特征和治疗结果。
本研究回顾性分析了 2007 年 1 月至 2015 年 12 月在本机构接受治疗的年龄<12 岁的连续 PTGCT 患者的数据。排除标准为：年龄>12 岁、非原发性睾丸生殖细胞肿瘤和副睾丸肿瘤。
共有 167 名患者符合研究条件（表）。中位诊断年龄为 20 个月（范围 1-142）。病理结果显示 87 例（50.9%）为畸胎瘤，50 例（29.2%）为卵黄囊瘤（YST），33 例（19.3%）为表皮样囊肿，1 例（0.6%）为混合性生殖细胞肿瘤。在畸胎瘤中，70 例（80.5%）为成熟畸胎瘤（MT），17 例（19.5%）为不成熟畸胎瘤（IT）。IT 比 MT 发病年龄更早，且肿瘤更大（P<0.001）。所有 MT 和 10 例 IT 均行保留睾丸手术（TSS），7 例 IT 行根治性睾丸切除术（RO）。在随访中，良性肿瘤均未发现转移或局部复发。所有 YST 的甲胎蛋白（AFP）水平均明显升高。其中，47 例（94%）诊断为Ⅰ期，1 例（2%）诊断为Ⅱ期，2 例（4%）诊断为Ⅳ期。RO 后，1 例中位随访 46 个月的Ⅰ期患者复发。1 例Ⅱ期和 2 例Ⅳ期患者因肿瘤进展死亡。
PTGCT 存在种族和民族差异。本研究认为，这是目前详细描述中国队列中 PTGCT 的临床病理特征和治疗结果的最大研究。
畸胎瘤是 PTGCT 最常见的亚型，其次是 YST。与 MT 相比，IT 表现为良性行为，更可能发生在更年轻的年龄，并且肿瘤更大。TSS 对良性病变是可靠的。对于Ⅰ期 YST，RO 单独有效。挽救性化疗有效，预防性腹膜后淋巴结清扫可能对 YST 不必要。PTGCT 的预后良好。
