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

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

doi:10.1155/2015/398549

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.

Highlights

Pediatric germ cell tumors (GCTs) are neoplasms derived from primodial germ cells and may occur both inside the gonads and in extragonadal organs
This paper presents a study of a single tertiary institute for pediatrics in order to evaluate long-term outcomes of sacrococcygeal germ cell tumors (SC-GCTs) over a 15-year period
A total of 57 patients were treated for SC-GCTs during the study period

Summary

Introduction

Pediatric germ cell tumors (GCTs) are neoplasms derived from primodial germ cells and may occur both inside the gonads and in extragonadal organs. The five main histologic categories of GCTs are dysgerminomas (in the ovary), seminomas (in the testes), teratomas, choriocarcinomas, and endodermal sinus tumors (ESTs) or yolk sac tumors [1, 2] (Figure 1). The most common site of extragonadal GCTs in the pediatric population is the sacrococcygeal region followed by the anterior mediastinum, intracranial region, retroperitonium, neck, stomach, and vagina [2]. The most common sacrococcygeal germ cell tumors (SC-GCT) are teratomas which mostly behave as benign tumors and, less commonly, as malignant ones [1,2,3]. The least common SCGCT is EST which characteristically presents as a malignant tumor.

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