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Treatment strategies for infants with malignant sacrococcygeal teratoma
Insights
Surgery alone is insufficient for malignant sacrococcygeal teratoma in children. Combined chemotherapy (vincristine, actinomycin D, cyclophosphamide, and adriamycin) and radiation therapy show promise but carry risks, including fatal pulmonary toxicity.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Radiation Oncology
Background:
- Malignant sacrococcygeal teratoma (MSCT) is a rare pediatric cancer.
- Embryonal carcinoma component indicates a poor prognosis.
Purpose of the Study:
- To evaluate the outcomes of surgical, chemotherapeutic, and radiotherapeutic interventions for MSCT.
- To identify optimal treatment strategies for this rare malignancy.
Main Methods:
- Retrospective analysis of 12 children with MSCT treated between 1971 and 1980.
- Treatment modalities included surgery, vincristine, actinomycin D, cyclophosphamide (VAC) chemotherapy, adriamycin, and radiation therapy (RT).
Main Results:
- Complete surgical excision alone was inadequate, with all 4 patients recurring.
- Combined VAC chemotherapy and RT improved survival but resulted in fatal pulmonary toxicity in some cases.
- One patient treated with VAC, adriamycin, and pelvic RT survived disease-free for 6 years.
Conclusions:
- Surgery alone is insufficient for treating malignant sacrococcygeal teratoma.
- Combined modality treatment with chemotherapy and radiation can be effective but carries significant risks.
- The optimal therapeutic program for pediatric MSCT remains under investigation.
Abstract:
Twelve children with malignant sacrococcygeal teratoma containing embryonal carcinoma, were treated at the Children's Hospital of Philadelphia between 1971 and 1980. Their ages at diagnosis ranged from 2 days to 23 mo; 8 of the 12 (67%) were girls. Five of the 12 patients presented with localized tumors which were grossly completely excised. Four received no further therapy, and all 4 recurred with histologically documented embryonal carcinoma. Despite subsequent treatment with radiation therapy (RT) and chemotherapy (vincristine, actinomycin D, and cyclophosphamide, collectively called VAC, in all 4 plus adriamycin in 3, only 1 has survived free of disease, 8 yr from diagnosis. The other 3 died of tumor (2) or pneumonia after pulmonary RT (1). The fifth patient in this group received VAC with adriamycin (total dose, 350 mg/sq m) and died of autopsy-proven cardiotoxicity without tumor. The remaining 7 patients presented with either unresectable local tumor (3) or distant metastases (4). One of these 7 died 6 days after biopsy with massive liver metastases. The other 6 children were treated after operation with VAC (5) or vincristine and actinomycin D (1) chemotherapy, and 5 also received RT to the pelvis. One has survived free of disease at 6 yr after treatment with VAC and adriamycin and pelvic RT, but the other 5 died of tumor (2) or of pneumonia after pulmonary RT (3). Surgery alone is inadequate for successful management of children with malignant sacrococcygeal teratoma. Chemotherapy with VAC, with or without adriamycin, can be effective when combined with radiation, but fatal pulmonary toxicity can result from such combined therapy. The optimal therapeutic program for children with malignant sacrococcygeal teratoma is still evolving.