Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients.

Background. Undifferentiated (embryonal) sarcoma of the liver is a rare, highly malignant, mesenchymal tumor presenting predominantly in late childhood. Four girls, ages 6–13 years, who were treated with combined-modality regimens are reported. Methods. In the first and second patients, hemihepa-tectomy resulted in complete removal of the tumor, and multiple-agent chemotherapy was administered postoperatively. In the third patient, only partial resection could be accomplished initially. By synchronous radiation therapy and chemotherapy, the tumor decreased to an extent that it could be resected completely and was totally devitalized on histologic examination. Postoperative chemotherapy was discontinued after 8 weeks. In the fourth patient, the tumor was not resectable at diagnostic biopsy. Polychemotherapy led to a significant reduction of the tumor size, and resection with clear margins could be performed subsequently. Because histologic necrosis amounted to about 95%, postoperative chemotherapy was also discontinued after 6 weeks. Results. All four patients remain well without evidence of tumor recurrence after 79, 41, 36, and 22 months from diagnosis. Conclusions. The authors suggest that a multimodal therapeutic regimen should be used in patients with undifferentiated hepatic sarcoma.