背景:肿瘤复发,肛门直肠和泌尿功能障碍,在婴儿骶尾部畸胎瘤(SCT)中观察到手术后下肢功能障碍。在本文中,我们进行了一项多机构回顾性观察性研究,以阐明日本的长期功能预后.
方法:这项研究是使用纸质问卷进行的,该问卷分发给日本儿科学会认可的192家机构,涵盖2000年至2019年在1岁以下接受根治性手术并在出生后存活至少180天的患者。
结果:本分析共纳入355例患者。Altman型为I-II型248例,III-IV型107例,中位最大肿瘤直径为6.1(范围:0.6-36.0)cm。有269个成熟畸胎瘤,69个未成熟畸胎瘤,和10个恶性肿瘤。325例全切除,27例次全切除或部分切除,54例手术并发症。术后中位随访时间为6.6(0.5-21.7)年。83例(23.4%)有功能性后遗症,包括62例(17.5%)肛门直肠功能障碍,56(13.0%)患有排尿功能障碍,下肢运动功能障碍15例(4.2%)。复发发生在42(11.8%),中位年龄为16.8(1.7-145.1)个月。功能障碍的危险因素包括早产,肿瘤直径大,AltmanIII-IV型,不完全切除,和手术并发症。复发的危险因素包括未成熟畸胎瘤或恶性肿瘤,不完全切除,和手术并发症。
结论:术后功能障碍不低,为23.4%,11.8%的患者在手术后10年以上复发,提示有危险因素的患者需要定期成像和肿瘤标志物评估。有必要建立治疗指南,以监测长期生活质量的最佳实践。
方法:二级回顾性研究。
BACKGROUND: Tumor recurrence, anorectal and urinary dysfunction, and lower limb dysfunction after surgery are observed in infantile sacrococcygeal teratoma (SCT). In this paper, a multi-institutional retrospective observational
study was conducted to clarify the long-term functional prognosis in Japan.
METHODS: This
study was conducted using a paper-based questionnaire distributed to 192 facilities accredited by the Japanese Society of Pediatric Surgeons, covering patients who underwent radical surgery at less than 1 year old and who survived for at least 180 days after birth from 2000 to 2019.
RESULTS: A total of 355 patients were included in this analysis. Altman type was I-II in 248 and type III-IV in 107, and the median maximum tumor diameter was 6.1 (range: 0.6-36.0) cm. There were 269 mature teratomas, 69 immature teratomas, and 10 malignant tumors. Total resection was performed in 325, subtotal or partial resection in 27, and surgical complications were noted in 54. The median postoperative follow-up was 6.6 (0.5-21.7) years. Eighty-three patients (23.4 %) had functional sequelae, including 62 (17.5 %) with anorectal dysfunction, 56 (13.0 %) with urinary dysfunction, and 15 (4.2 %) with lower limb motor dysfunction. Recurrence occurred in 42 (11.8 %) at a median age of 16.8 (1.7-145.1) months old. Risk factors for dysfunction included preterm delivery, a large tumor diameter, Altman type III-IV, incomplete resection, and surgical complications. Risk factors for recurrence included immature teratoma or malignancy, incomplete resection, and surgical complications.
CONCLUSIONS: Postoperative dysfunction was not low at 23.4 %, and 11.8 % of the patients experienced recurrence occurring more than 10 years after surgery, suggesting the need for periodic imaging and tumor markers evaluations in patients with risk factors. It is necessary to establish treatment guidelines for best practice monitoring of the long-term quality of life.
METHODS: Level II Retrospective
Study.