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Malignant presacral teratoma in children
Insights
Malignant presacral teratoma, a rare pediatric cancer, shows improved outcomes with multidisciplinary treatment. This study highlights successful outcomes in four out of six young female patients treated with surgery, irradiation, and chemotherapy.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Radiation Oncology
Background:
- Malignant presacral teratoma is a rare tumor predominantly affecting young female children.
- Historically, this disease had a poor prognosis, but advancements in treatment have improved patient outlooks.
Purpose of the Study:
- To review the clinical presentation, pathological features, and treatment outcomes of malignant presacral teratoma in pediatric patients.
- To discuss the evolution of treatment protocols for this rare malignancy.
Main Methods:
- Retrospective review of six pediatric cases diagnosed with malignant presacral teratoma at Memorial Hospital.
- Analysis of treatment modalities including surgery, irradiation, and multi-drug chemotherapy.
Main Results:
- Six female patients, aged 17 months to 13 years, presented with symptoms including masses, constipation, voiding difficulties, and pain.
- Four out of six patients achieved disease-free survival, with three remaining disease-free for over 24 months post-treatment.
- Pathological features were complex, with three cases showing predominant endodermal sinus tumor components.
Conclusions:
- Multidisciplinary treatment, encompassing surgery, irradiation, and chemotherapy, has significantly improved survival rates for malignant presacral teratoma.
- Early diagnosis and comprehensive treatment are crucial for managing this rare pediatric malignancy.
Abstract:
Malignant presacral teratoma is a rare tumor seen predominantly in young female children. The introduction of planned multidisciplinary treatment has improved the outlook for patients with this once dismal disease. Six female children were seen at Memorial Hospital with the diagnosis of malignant presacral teratoma. Five children were age 17 mo to 3 yr and the sixth child was 13 yr old at diagnosis. Presenting symptoms included masses in the buttock or groin, constipation, difficulty voiding, and local pain. Pathological features were varied and complex but three had predominantly endodermal sinus features. One child had the malignant presacral teratoma develop 18 mo after successful resection of a benign sacrosoccygeal teratoma in the newborn period. Treatment varied in the six cases since all were referred after failure of treatment elsewhere. All children had surgery, irradiation, and multiple drug chemotherapy. Four of the six children are surviving disease-free, 3 more than 24 mo off treatment. Evolution of treatment up to the present protocol management is discussed.