Preirradiation cisplatin and etoposide in the treatment of high-risk medulloblastoma and other malignant embryonal tumors of the central nervous system: a phase II study.

Medulloblastoma, pineoblastoma, and cerebral neuroblastoma are malignant embryonal tumors of the CNS that may demonstrate similar histologic features, a propensity for neuraxis dissemination and sensitivity to radiation therapy and, in certain cases, chemotherapy. To evaluate the activity of preirradiation chemotherapy in such tumors, 11 newly diagnosed children with measurable residual disease and characteristics indicative of poor prognosis were treated postoperatively with cisplatin (CDDP) and etoposide (VP-16). Responses graded on the basis of radiographic findings in areas of either macroscopic residual tumor or metastatic disease included two complete responses (CRs), eight partial responses (PRs), and one stable disease (SD). Acute and subacute toxicity consisted of high-frequency hearing loss in four patients, reversible signs and symptoms of increased intracranial pressure in two patients, and transient neutropenia. Seven of eight patients with high-risk medulloblastoma and two of two with pineoblastoma remain free of tumor progression following neuraxis irradiation at 8 to 48 months postdiagnosis (median, 18 months). CDDP and VP-16 is a highly active drug combination when given before irradiation in children with high-risk medulloblastoma and other malignant embryonal tumors of the CNS, producing objective responses in at least one site of measurable disease in 10 of 11 newly diagnosed patients, including all of five with gross neuraxis dissemination.

Present-day concepts in the management of sarcomas in children.

1. NRSTS tumors represent 20-20% of all soft tissue sarcomas. In only the extremity primary are both RMS and NRSTS occurring with almost equal frequency. 2. Modern diagnostic imaging studies are invaluable to staging and preoperative planning. 3. The most important need is to standardize biopsy techniques, since an ill-conceived biopsy has an adverse effect on the definitive surgical procedure. 4. Standardization of surgical procedures is necessary to adequately define extent of local resection varying with site, type of tumor, and its biological behavior. Less radical procedures do not seem to have had an adverse effect in local control or survival as is evidenced in RMS of the orbit, vagina, and bladder. 5. Though complete surgical extirpation is the treatment of choice in RMS, incomplete surgical excision leaving microscopic disease can result in adequate local control following chemotherapy and radiation therapy. However, less than optimum responses to chemotherapy in the NRSTS makes it imperative that efforts be made to completely resect these lesions. 6. It is in the realm of initially unresectable primary lesion that the surgical oncologist's role be refined. Consideration here includes: (a) use of newer surgical techniques such as laser, or free microvascular grafts, (b) protocols to define the optimum timing of delayed surgery following preoperative chemotherapy with or without radiation therapy.

Challenges in the treatment of childhood fibromatosis.

Between 1968 and 1985, we treated 20 children for fibromatosis (also called desmoid tumor and aggressive fibromatosis). The primary sites included head and neck (seven patients), extremity (seven patients), and trunk (six patients). Lesions ranged from 3 to 18 cm in diameter. The tumors were smaller than 5 cm in 13 patients, and in seven patients they were larger than 5 cm. A total resection was not feasible in any of the patients with lesions larger than 5 cm. Ten of the 11 patients treated with wide local resection, in whom the margins were clearly negative or close, remained free of disease for six to 16 years. Nine patients required additional treatment with radiotherapy (nine patients) and chemotherapy (five patients). Two died of local disease progression. In the remaining seven children, the disease was controlled. We describe our strategies for managing this disease in a pediatric population.