Rescorla F J, Sawin R S, Coran A G, Dillon P W, Azizkhan R G
J.W. Riley Hospital, Indianapolis, IN 46202, USA.
J Pediatr Surg. 1998 Feb;33(2):171-6. doi: 10.1016/s0022-3468(98)90426-2.
BACKGROUND/PURPOSE: Sacrococcygeal teratomas (SCT) are a relatively uncommon tumor affecting neonates, infants, and children. This study was designed to determine the effect of therapy on the long-term outcome of neonates and children with sacrococcygeal teratomas (SCT).
The authors conducted a retrospective review of children with SCT treated at 15 Childrens Cancer Group institutions from 1972 to 1994.
One hundred twenty-six children presented with SCT diagnosed prenatally (n = 32), at birth (n = 79), or later in infancy (n = 15). For neonates, complete resection was performed except in two babies with lethal associated defects. All others (n = 15) underwent resection at the age of diagnosis. Six had a sacral mass identified at birth but had delayed surgery (1.5 to 11 months) and of these, two were malignant. Resection was via sacral (n = 96) or abdominosacral (n = 28) approach. Histology showed mature teratoma (MT, 69%), immature teratoma (IT, 20%), or endodermal sinus tumor (EST, 11%) at presentation. Seven neonates (5.6%) died of perioperative complications, whereas the remaining 117 were available for long-term follow-up. Between 6 and 34 months postresection, recurrent disease developed in 9 of 80 MT patients (11%) followed-up for a mean of 5 years. Recurrent disease was MT (n = 2) and EST (n = 7). The recurrent EST patients were treated with adjuvant chemotherapy. Six are alive with mean follow-up of 114 months, whereas one with metastatic disease was lost to follow-up. Recurrence (MT) developed in only 1 of 24 IT patients, and all are alive and well at mean follow-up of 39 months. Patients presenting with EST (n = 13) underwent excision, with two dying from non-EST causes. Six EST patients received no chemotherapy, with two of the six (33%) experiencing recurrence within 11 months and both disease free after salvage chemotherapy. The remaining five EST patients received adjuvant chemotherapy; four are alive and one died of metastatic disease. Of the 18 EST patients followed-up after resection (presentation, 11, recurrent teratoma, 7), 16 (89%) are free of disease with a mean follow-up of 91 months.
(1) Benign teratomas have a significant recurrence rate mandating close follow-up for more than 3 years. (2) Surgical resection alone is adequate therapy for nonmetastatic malignant tumors. (3) Survival for malignant lesions with metastases is excellent with modern chemotherapy.
背景/目的：骶尾部畸胎瘤（SCT）是一种相对罕见的肿瘤，影响新生儿、婴儿和儿童。本研究旨在确定治疗对患有骶尾部畸胎瘤（SCT）的新生儿和儿童长期预后的影响。
作者对1972年至1994年在15个儿童癌症研究组机构接受治疗的SCT患儿进行了回顾性研究。
126例患儿被诊断为SCT，其中产前诊断32例，出生时诊断79例，婴儿期后期诊断15例。对于新生儿，除了两名伴有致命相关缺陷的婴儿外，均进行了完整切除。其他所有患儿（15例）在诊断时接受了切除。6例出生时发现有骶部肿块，但手术延迟（1.5至11个月），其中2例为恶性。切除通过骶部入路（96例）或腹骶部入路（28例）进行。组织学显示，初诊时成熟畸胎瘤（MT，69%）、未成熟畸胎瘤（IT，20%）或内胚窦瘤（EST，11%）。7例新生儿（5.6%）死于围手术期并发症，其余117例可供长期随访。在切除术后6至34个月，80例MT患者中有9例（11%）复发，平均随访5年。复发病变为MT（2例）和EST（7例）。复发性EST患者接受了辅助化疗。6例存活，平均随访114个月，但1例有转移性疾病患者失访。24例IT患者中仅1例复发（MT），所有患者均存活良好，平均随访39个月。初诊为EST的患者（13例）接受了切除，2例死于非EST原因。6例EST患者未接受化疗，其中2例（33%）在11个月内复发，经挽救化疗后均无病生存。其余5例EST患者接受了辅助化疗；4例存活，1例死于转移性疾病。18例EST患者切除术后接受随访（初诊11例，复发性畸胎瘤7例），16例（89%）无病生存，平均随访91个月。
（1）良性畸胎瘤有显著的复发率，需要密切随访3年以上。（2）对于非转移性恶性肿瘤，单纯手术切除是足够的治疗方法。（3）对于有转移的恶性病变，现代化疗的生存率很高。
