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Malignant sacrococcygeal germ cell tumors in children: a 30-year experience from a single institution
Münevver Büyükpamukçu1, Ali Varan, Serhan Küpeli
1Institute of Oncology, Department of Pediatric Oncology, Hacettepe University, Ankara, Turkey.
Insights
Malignant sacrococcygeal germ cell tumors in children have a 50.9% overall survival rate. Elevated alpha-fetoprotein (AFP) levels at diagnosis positively impact prognosis, while other factors like tumor stage do not significantly affect survival.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Malignant sacrococcygeal germ cell tumors are rare pediatric cancers.
- Analyzing treatment outcomes and survival is crucial for improving patient care.
Purpose of the Study:
- To evaluate treatment results and survival characteristics of pediatric patients with malignant sacrococcygeal germ cell tumors.
- To identify prognostic factors influencing survival in this patient cohort.
Main Methods:
- Retrospective review of patient files from 1979-2009.
- Data collected included patient demographics, tumor characteristics, treatment details (surgery, chemotherapy, radiotherapy), and survival outcomes.
- Statistical analysis using univariate and multivariate models.
Main Results:
- 58 patients with malignant sacrococcygeal germ cell tumors were analyzed.
- Overall and event-free survival rates were 50.9% and 43.8%, respectively.
- Alpha-fetoprotein (AFP) levels at diagnosis significantly impacted event-free survival, with higher levels correlating with better outcomes.
Conclusions:
- Treatment outcomes for malignant sacrococcygeal germ cell tumors in this cohort were suboptimal compared to literature.
- Elevated AFP levels at diagnosis are a positive prognostic indicator.
- Tumor stage, metastasis, size, and histopathological subtype did not significantly influence survival.
Background:
Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors.
Procedure:
Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded.
Results:
A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥ 10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis.
Conclusions:
Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.
