The role of chemotherapy in childhood soft tissue sarcomas other than rhabdomyosarcomas: experience of the Northern Israel Oncology Center.

Forty-three children with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS) were treated at the Northern Israel Oncology Center in Haifa, Israel, from 1971 to 1996. The male:female ratio was 1.5:1 and the median age of patient was 10 years (range, 3 months-18 years). The most common histopathologic diagnoses were fibrosarcoma (32.5%) and synovial sarcoma (16%). The sites of primary tumor were lower limb (35%), trunk (18%), upper limb (16%), head and neck (16%), and retroperitoneum (11%). By Intergroup Rhabdomyosarcoma Study classifications, 13 patients presented as group I, 15 patients as group II, 10 patients as group III, and 5 patients as group IV. Median follow-up time was 63 months (range, 6 months-18 years). The estimated survival after a 5-year period is 72% (SE +/- 17) for patients in group I, 75 +/- 15% in group II, 90 +/- 9% for patients in group III, and 40 +/- 21% for patients in group IV. Eleven patients relapsed; 4/6 who developed local relapse were cured and are alive with disease, while 4/5 who developed distant metastases are dead. For the 28 patients who underwent complete resection at diagnosis, the estimated survival after a 5-year period is 87 +/- 5% vs. 60 +/- 17% for the 15 patients who underwent partial excision or biopsy. Local radiotherapy was delivered after surgery to group III patients. Preoperative and postoperative chemotherapy was delivered to the patients of groups III and IV, and postoperative chemotherapy only to group II patients. Chemotherapy produced demonstrable gain in survival for group II and III patients but not for patients with metastases. The authors conclude that an aggressive surgical approach is needed in patients with NRSTS. Chemotherapy may help as a preoperative treatment in bulky disease or as a postoperative treatment for microscopic residual disease.

Multifocal desmoid tumor in childhood: report of two cases and review of the literature.

We present two cases of multifocal desmoid tumors arising in two teenagers. Different, separate lesions developed in the same limb over a period of several years, one of which recurred on several occasions. The literature regarding the therapeutic aspects of multifocal fibromatosis is reviewed and discussed.

[Optic glioma in children with type 1 neurofibromatosis].

During the period 1985-95 we treated 5 girls and 13 boys with optic glioma associated with Type 1 neurofibromatosis (median age 3 years, range 2-10 years). 6 were treated with radiotherapy alone, 1 with surgery and radiation therapy and 1 with chemotherapy in order to postpone irradiation to an older age; 1 is being followed with no therapy. All children are alive, 2-10 years from diagnosis (mean follow-up time 5.4 years). 3 had improvement of vision following therapy and in 4 visual ability stabilized, including the child being followed without treatment. In 1 vision deteriorated despite therapy.

Bleomycin and cyclophosphamide toxicity simulating metastatic nodules to the lungs in childhood cancer.

Two pediatric oncology patients with Ewing's sarcoma and one with mixed germ cell tumor were treated with drug regimens that included bleomycin or cyclophosphamide. Despite progress to apparently complete remission, all manifested pulmonary nodules on computed tomography during or at the end of treatment. Thoracoscopic biopsy to confirm metastasis revealed instead fibrotic lesions apparently attributable to bleomycin or cyclophosphamide. After cessation of chemotherapy, the pulmonary lesions resolved and all three patients sustained their remissions. The case histories and comments on the diagnosis and management of pulmonary nodules are reviewed.