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Published on: May 26, 2023
Long-Term Outcomes of Sacrococcygeal Germ Cell Tumors in Infancy and Childhood
Rangsan Niramis1, Maitree Anuntkosol1, Veera Buranakitjaroen1
1Department of Surgery, Queen Sirikit National Institute of Child Health, Bangkok 10400, Thailand ; College of Medicine, Rangsit University, Bangkok 10400, Thailand.
Insights
Sacrococcygeal germ cell tumors (SC-GCTs) in children show a 7% recurrence rate for benign types. Malignant SC-GCTs with metastasis had poor survival, necessitating long-term monitoring.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Developmental Biology
Background:
- Sacrococcygeal germ cell tumors (SC-GCTs) are rare in pediatric patients.
- Long-term outcomes and recurrence patterns require further elucidation.
Purpose of the Study:
- To evaluate the long-term outcomes of pediatric SC-GCTs over a 15-year period.
- To analyze recurrence rates and survival in relation to tumor type and treatment.
Main Methods:
- Retrospective review of 57 pediatric patients treated for SC-GCTs from 1998 to 2012.
- Surgical resection and coccygectomy were primary interventions.
- Tumor pathology, recurrence, and alpha-fetoprotein (AFP) levels were analyzed.
Main Results:
- Mature sacrococcygeal teratomas (SCTs) were most common (72%).
- A 7.3% recurrence rate was observed in benign SCTs.
- Malignant SCTs and endodermal sinus tumors (ESTs) showed variable response to treatment; 4 patients with metastatic disease died post-treatment.
Conclusions:
- Benign SCTs have a significant recurrence risk, underscoring the need for vigilant follow-up.
- Close monitoring, including serial AFP level assessment for 5 years post-surgery, is crucial.
- Malignant SC-GCTs with distant metastasis present a poor prognosis.
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.
