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.
Between April 1988 and July 1995, 11 children with a lymphangioma were treated with intralesional OK-432 injection. In 7 patients it was the primary therapy and total shrinkage of the lesion was obtained in 5 of them. Two patients did not respond and the children underwent surgery. Following incomplete surgical removal or recurrence of the lymphangioma, intralesional OK-432 injection was used as secondary therapy in 4 patients. Total regression was observed in 2 cases and marked regression in the 2 others. No serious side-effects except fever lasting for 2-3 days and slight tenderness with swelling of the lymphangioma for 3-4 days after the injection was noted. Local inflammatory reaction did not cause any damage to the overlying skin and did not lead to scar formation. Depending on the size, location, and anatomical relationship to the airway, intralesional injections of the lymphangiomas were performed under general anaesthesia and the children were observed for 24 h. There was no recurrence after follow up periods ranging from 2 months to 7 years.
Among 460 children who had an orchiopexy (ORP), we identified 72 children who had attended our institution 1-12 years earlier and in whom the testicular position had been specified. Of the 72 boys 19 were had ascended testes (26%). The age at ORP of the children with ascent of the testes was 7.0 years (1.8-14.0 years). Light and electron microscopy of 13 testicular biopsies taken at ORP, showed alterations of germ cells and Sertoli cells, similar but less pronounced than alterations seen in congenital undescended testes. Conclusion. A normally positioned testis may ascend into the inguinal region or upper scrotum and remain there fixed. This secondary cryptorchidism does not usually respond to human chorionic gonadotropin treatment and must be corrected by orchiopexy.
Eight patients aged 1 month up to nine years with congenital diaphragmatic hernias (seven left sided postero-lateral, one right-sided antero-medial), who presented outside of the neonatal period, are reported, four are described in detail. Radiographic presentation was obvious in four patients, simulated inflammatory lung disease in one and pneumothorax in two. In one patient a Morgagni hernia was primarily missed. Two had previous normal chest X-rays. All cases with herniated bowel showed "connecting" bowel segments passing through the diaphragmatic defect. Primarily the lack of awareness of delayed presentation of congenital diaphragmatic hernia in children with uncharacteristic thoracic and/or abdominal symptoms led to an undesirable time delay between first chest X-ray and surgery up to 16 months in four of our cases. Life threatening complications can be the consequence of delayed diagnosis as in one of our cases.
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