Department of Paediatric Surgery, Royal Manchester Children's Hospital, Manchester, UK.
Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, UK.
Pediatr Blood Cancer. 2023 Jan;70(1):e29994. doi: 10.1002/pbc.29994. Epub 2022 Oct 13.
Sacrococcygeal teratoma (SCT) is a rare paediatric germ cell tumour (1:40,000). Long-term data regarding urinary tract and bowel function after SCT resection is limited to few studies. A UK Children's Cancer and Leukaemia Group (CCLG) Surgeons multicentre study aimed to critically analyse long-term functional outcomes in patients following resection of SCT.
Nationwide study of UK paediatric surgical oncology centres using a standardised data collection form. All index cases of newborn infants and children <16 years with SCT diagnosis during 2005-2015 were included.
165 SCT patients treated at 14 UK paediatric surgical oncology centres were included. Median age at presentation was 1 day [interquartile range, IQR: 0-25]; median age at surgery was 10 days [IQR: 4-150]. One hundred seventeen (70%) were female and 48 (30%) male. Antenatal diagnosis was made in 44% index cases. Total 59% of patients were Altman Stage I or II lesions. Follow-up data were available in 83% cases. Tumour recurrence occurred in 13 (7%) patients at median age 13 months [IQR: 8.75-30 months]. Fifty-nine (36%) of 165 patients had documented adverse bladder or bowel dysfunction. Twenty-two (37%) cases required urinary clean intermittent catheterisation (CIC) urology health care, with eight patients (14%) needing operative intervention to control management of bowel dysfunction.
This UK CCLG study showed 36% of SCT patients develop bladder or bowel dysfunction after primary tumour resection. Functional assessment of bladder and bowel function is mandatory during after-care follow-up of all SCT patients. A multidisciplinary care pathway, with surgeon speciality groups including surgical oncology, paediatric urology and paediatric colorectal specialists, is strongly advised to facilitate 'best practice' monitoring of long-term health and improve patient quality of life (QoL) into adulthood.
骶尾部畸胎瘤（SCT）是一种罕见的小儿生殖细胞肿瘤（1:40000）。关于 SCT 切除术后尿路和肠道功能的长期数据仅限于少数研究。英国儿童癌症和白血病组（CCLG）外科医生的一项多中心研究旨在对 SCT 切除术后患者的长期功能结果进行批判性分析。
使用标准化数据收集表对英国儿科肿瘤外科中心进行全国性研究。纳入 2005-2015 年间诊断为新生儿和<16 岁儿童 SCT 的所有指数病例。
纳入了来自英国 14 个儿科肿瘤外科中心的 165 名 SCT 患者。中位发病年龄为 1 天[四分位距（IQR）：0-25]；中位手术年龄为 10 天[IQR：4-150]。117 例（70%）为女性，48 例（30%）为男性。44%的指数病例有产前诊断。总共有 59%的患者为 Altman Ⅰ期或Ⅱ期病变。83%的病例可获得随访数据。中位年龄 13 个月[IQR：8.75-30 个月]时，13 例（7%）患者肿瘤复发。165 例患者中，59 例（36%）有记录的膀胱或肠道功能障碍。22 例（37%）患者需要进行间歇性导尿（CIC）泌尿外科保健，8 例（14%）患者需要手术干预来控制肠道功能障碍的治疗。
这项英国 CCLG 研究表明，36%的 SCT 患者在原发肿瘤切除后出现膀胱或肠道功能障碍。所有 SCT 患者的随访中都必须进行膀胱和肠道功能的功能评估。强烈建议建立一个多学科护理途径，包括肿瘤外科、小儿泌尿科和小儿结直肠专科医生的专业团体，以促进“最佳实践”监测长期健康状况，并提高患者的生活质量（QoL），直至成年。
