van Heurn Lieke J, Derikx Joep P M, Hall Nigel, Aldrink Jennifer H, Bailez Maria M, Chirdan Lohfa B, Fumino Shigehisa, Hesse Afua, Soyer Tuktu, StPeter Shawn, Twisk Jos, Yang Tianyou, van Heurn Ernst L W
Department of Paediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam.
University Surgery Unit, Faculty of Medicine, University of Southampton, Southampton, UK.
Int J Surg. 2024 Nov 1;110(11):7177-7186. doi: 10.1097/JS9.0000000000002045.
Sacrococcygeal teratoma (SCT) is a rare congenital tumour. The risk of malignancy and recurrence is not well defined. Previous studies are small and report differing conclusions about the timing of surgery and the duration of follow-up. The authors studied the risk of malignant transformation and SCT recurrence after surgery to address these gaps.
This was a global retrospective cohort study. Data of consecutive SCT patients was obtained from 145 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence or death due to malignancy, and its risk factors were analysed.
Of the 3612 included patients, 3407 entered analysis. The risk of malignant transformation of the initial tumour was 3.3, 5.1, 10.1, and 32.9% at age 3 months, 6 months, 1 year, and 2 years, respectively. After 6 years, the censored risk of malignancy (64%) did not further increase. Recurrent SCT was diagnosed in 349 (10.2%) children with 126 (36.1%) malignant recurrences. Risk factors for recurrence were Altman type II [odds ratio (OR): 1.6, 95% confidence interval (CI): 1.2-2.2], Altman type III (OR: 1.6, 95% CI: 1.2-2.3), initial immature histology (OR: 1.9, 95% CI: 1.4-2.6), and initial malignant histology (OR: 4.0, 95% CI: 2.9-5.4).
The risk of malignancy at initial resection in SCT increases with age reaching a plateau at 6 years of age. Recurrence after resection occurred in 10% of patients and 36% of these were malignant at that time. Altman type II or type III, and immature or malignant histology were associated with recurrence.
Level III.
骶尾部畸胎瘤（SCT）是一种罕见的先天性肿瘤。其恶变和复发风险尚不明确。既往研究规模较小，且对于手术时机和随访时长的结论各异。作者开展此项研究以探讨手术治疗后恶变及SCT复发的风险，填补这些空白。
这是一项全球范围的回顾性队列研究。连续纳入来自62个国家145家机构的SCT患者数据。分析了初始切除时即为恶性、恶性复发或因恶性肿瘤死亡所定义的恶变情况及其危险因素。
纳入的3612例患者中，3407例进入分析。初始肿瘤恶变风险在3个月、6个月、1岁和2岁时分别为3.3%、5.1%、10.1%和32.9%。6年后，恶性肿瘤的审查风险（64%）未进一步增加。349例（10.2%）儿童被诊断为复发性SCT，其中126例（36.1%）为恶性复发。复发的危险因素包括阿尔特曼II型[比值比（OR）：1.6，95%置信区间（CI）：1.2 - 2.2]、阿尔特曼III型（OR：1.6，95%CI：1.2 - 2.3）、初始组织学为未成熟型（OR：1.9，95%CI：1.4 - 2.6）以及初始组织学为恶性型（OR：4.0，95%CI：2.9 - 5.4）。
SCT初始切除时的恶变风险随年龄增加，6岁时达到平台期。切除术后10%的患者出现复发，其中36%在复发时为恶性。阿尔特曼II型或III型以及未成熟或恶性组织学与复发相关。
三级。
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