Department of Paediatric Surgery, Alder Hey Children's Hospital NHS Foundation Trust, University of Liverpool, Liverpool, United Kingdom.
Department of Paediatric Surgery, Alder Hey Children's Hospital NHS Foundation Trust, University of Liverpool, Liverpool, United Kingdom.
J Pediatr Surg. 2021 Nov;56(11):2032-2036. doi: 10.1016/j.jpedsurg.2021.02.063. Epub 2021 Mar 5.
To report a 20-year experience highlighting management and outcome(s) of paediatric testicular tumours.
All males (< 19 years) with an index diagnosis of testicular tumours during the era(s) 1998-2018 in North West England were identified. Data were collected regarding age at diagnosis, disease stage, surgical operations, tumour biology and outcome(s).
A total of 34 male patients were identified. Median age at primary diagnosis was 94 months (range: 0-229 months). Eighteen tumours were benign and 16 malignant. Twenty cases (59%) were recorded in pre pubertal children and 14 (41%) in post pubertal males . In the pre pubertal group (0-11 years) - 15 cases of germ cell tumours (unrelated to germ cell neoplasia in situ - non-GCNIS derived) were recorded, including six yolk sac lesions, eight teratomas and one mixed teratoma/yolk sac tumour (pre-pubertal type). Four males with sex cord-stromal tumours included one juvenile granulosa cell tumour, two Sertoli cell tumours and one Leydig cell tumour. One miscellaneous type tumour notably a papillary cyst adenoma was also identified. In the post pubertal male cohort (>12 years) (n = 14) - four non-GCNIS derived tumours were identified (3 epidermoid cysts and one teratoma), eight cases of germ cell tumour derived from germ cell neoplasia in situ (GCNIS derived) included one teratoma, six with mixed germ cell tumours and one embryonal carcinoma. Two males had sex cord stromal tumours: (Leydig cell and granulosa cell biology). Twenty-eight patients underwent high radical inguinal orchidectomy(s) with one male also requiring retroperitoneal surgery to clear distant locoregional disease and a further single case thoracotomy and metastasectomy. Six patients had lesions suitable for 'testicular sparing' surgery. Six patients had metastatic disease at presentation (18%). Overall study survival was 97%. A single fatality occurred in an adolescent male with a mixed GCT harbouring liver, lung and para-aortic disease who died 48 months after initiating treatment.
We highlight one of the largest study series of paediatric testicular tumours in the UK and Europe. Non-GCNIS derived tumours accounted for the most common tumour biology (56%). Survival for paediatric testicular tumours is reassuringly generally excellent. Delayed presentation however with a malignant testicular tumour may be associated with poor outcome(s).
报告 20 年的儿童睾丸肿瘤诊治经验和结果。
在英格兰西北部,回顾性分析了 1998 年至 2018 年期间所有确诊为睾丸肿瘤的男性(<19 岁)的临床资料,包括年龄、疾病分期、手术方式、肿瘤生物学特征和预后等。
共纳入 34 例男性患者,中位年龄为 94 个月(0-229 个月)。18 例为良性肿瘤,16 例为恶性肿瘤。20 例(59%)发生于青春期前,14 例(41%)发生于青春期后。在青春期前组(0-11 岁),记录了 15 例生殖细胞肿瘤(与生殖细胞原位肿瘤无关-非生殖细胞内肿瘤源性),包括 6 例卵黄囊肿瘤、8 例畸胎瘤和 1 例混合性卵黄囊肿瘤/畸胎瘤(青春期前型)。4 例性索-间质肿瘤包括 1 例幼年颗粒细胞瘤、2 例支持细胞瘤和 1 例间质细胞瘤。还发现了 1 例罕见的乳头状囊腺瘤。在青春期后组(>12 岁)(n=14),记录了 4 例非生殖细胞内肿瘤源性肿瘤(3 例表皮样囊肿和 1 例畸胎瘤)、8 例生殖细胞肿瘤源性肿瘤(生殖细胞内肿瘤源性),包括 1 例畸胎瘤、6 例混合性生殖细胞瘤和 1 例胚胎癌。2 例男性为性索-间质肿瘤(Leydig 细胞和颗粒细胞瘤生物学)。28 例患者接受了高位根治性腹股沟睾丸切除术,1 例患者还需要进行腹膜后手术以清除局部远处疾病,另有 1 例患者接受了胸腔镜手术和转移灶切除术。6 例患者的病变适合行“保留睾丸”手术。6 例患者在就诊时已有转移(18%)。总体研究生存率为 97%。1 例青少年男性混合生殖细胞瘤伴肝、肺和主动脉旁疾病,治疗 48 个月后死亡,是唯一的死亡病例。
本研究是英国和欧洲最大的儿童睾丸肿瘤系列研究之一,非生殖细胞内肿瘤源性肿瘤占最常见的肿瘤生物学类型(56%)。儿童睾丸肿瘤的预后通常较好。然而,恶性睾丸肿瘤的延迟诊断可能与不良预后相关。