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婴幼儿及儿童期骶尾部生殖细胞肿瘤的长期预后

Long-Term Outcomes of Sacrococcygeal Germ Cell Tumors in Infancy and Childhood.

作者信息

Niramis Rangsan, Anuntkosol Maitree, Buranakitjaroen Veera, Tongsin Achariya, Mahatharadol Varaporn, Poocharoen Wannisa, La-Orwong Suranetr, Tiansri Kulsiri

机构信息

Department of Surgery, Queen Sirikit National Institute of Child Health, Bangkok 10400, Thailand ; College of Medicine, Rangsit University, Bangkok 10400, Thailand.

出版信息

Surg Res Pract. 2015;2015:398549. doi: 10.1155/2015/398549. Epub 2015 Oct 4.

Abstract

Purpose. The aim of this study was to evaluate long-term outcomes of sacrococcygeal germ cell tumors (SC-GCTs) over a 15-year period. Materials and Methods. A retrospective review was conducted of all pediatric patients treated for SC-GCTs at our hospital from 1998 to 2012. Results. Fifty-seven patients were treated for SC-GCTs with the most common in Altman's classification type I. Age at surgery ranged from one day to 5.6 years. Tumor resection and coccygectomy were primarily performed in about 84% of the cases. Pathology revealed mature, immature, malignant sacrococcygeal teratomas (SCTs), and endodermal sinus tumors (ESTs) in 41 (72%), 4 (77%), 6 (10.5%), and 6 (10.5%), respectively. Recurrence of discase occurred in 3 of 41 patients with mature teratomas (7.3%); 2 recurrences with mature teratomas and one recurrence with EST. Five of 6 malignant SCTs and 3 of 6 ESTs responded well to the treatment. Alpha-fetoprotein (AFP) level was elevated in both malignant teratomas and ESTs. No immediate patient death was noted in any of the 57 cases, but 4 patients with malignant tumors and distant metastasis succumbed at home within 2 years of the initial treatment. Conclusion. Benign SCTs have a significant recurrence rate of approximately 7%. Close follow-up with serial AFP level monitoring should be done for 5 years after initial tumor resection and coccygectomy. The survival rate for malignant SC-GCTs with distant metastasis was unfavorable in the present study.

摘要

目的。本研究的目的是评估15年间骶尾部生殖细胞肿瘤(SC - GCTs)的长期预后。材料与方法。对1998年至2012年在我院接受SC - GCTs治疗的所有儿科患者进行回顾性研究。结果。57例患者接受了SC - GCTs治疗,最常见的为阿尔特曼分类中的I型。手术年龄从1天至5.6岁不等。约84%的病例主要进行了肿瘤切除和尾骨切除术。病理显示成熟、不成熟、恶性骶尾部畸胎瘤(SCTs)和内胚窦瘤(ESTs)分别为41例(72%)、4例(77%)、6例(10.5%)和6例(10.5%)。41例成熟畸胎瘤患者中有3例(7.3%)疾病复发;2例成熟畸胎瘤复发,1例EST复发。6例恶性SCTs中有5例、6例ESTs中有3例对治疗反应良好。恶性畸胎瘤和ESTs的甲胎蛋白(AFP)水平均升高。57例患者中均未出现即刻死亡,但4例有远处转移的恶性肿瘤患者在初始治疗后2年内在家中死亡。结论。良性SCTs的复发率约为7%,具有显著意义。初次肿瘤切除和尾骨切除术后应连续5年密切随访并监测AFP水平。在本研究中,伴有远处转移的恶性SC - GCTs的生存率不佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce34/4609489/67279b384d84/SRP2015-398549.001.jpg

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