Twelve children younger than 16 years affected by undifferentiated nasopharyngeal carcinoma (NPC) with advanced primary tumor (T3, T4) were treated with chemotherapy consisting of Adriamycin (ADM [doxorubicin; Adria Laboratories, Columbus, OH]), vincristine (VCR), and cyclophosphamide (CYC), and radiotherapy. Preradiation chemotherapy produced partial responses in eight of ten evaluable patients. Eleven of 12 patients achieved a complete response following radiotherapy. The actuarial 3-year continuous relapse-free survival (CRFS) was 75%. This represents a significant improvement when compared with the 8% rate obtained in a previous series of patients treated with radiotherapy either with or without adjuvant CYC.

A total of 20 children (median age 11 years) were treated for primary thyroid carcinoma from 1976 to 1990. Papillary adenocarcinoma was diagnosed in 19 and follicular in one case. Nineteen of 20 patients were considered amenable to surgery, which consisted of total thyroidectomy in 14 and partial thyroidectomy in 5. Only one patient with extensive perithyroid soft tissue infiltration was treated with external beam radiotherapy. Monolateral or bilateral cervical nodal dissection was performed in eight and six children, respectively; in nine cases without clinical evidence of metastatic nodes. Pathological examination showed that tumor extent was greater than that clinically assessed: Multiple tumor foci within the thyroid were assessed in 8/19, unilateral positive nodes in 8, and bilateral in 6, and soft tissue infiltration in 7. Subsequently 10 patients received thyroid-stimulating hormone (TSH) suppressive hormonotherapy. Relapses occurred in 7/20 at 2-48 months (median 18) from primary treatment: Four in cervical nodes, two in cervical nodes and lungs, and one in lungs. These seven patients were salvaged with node dissection and radioiodine therapy for lung metastases. All the 20 children are alive and disease-free after a median follow-up of longer than 10 years. The incidence of relapse was greater in the group of patients not given TSH-suppressive hormonotherapy. Total thyroidectomy produced permanent hypoparathyroidism in 5/14 (36%). Thyroid carcinoma in children of this series frequently presented with multiple tumor foci within the thyroid and cervical node metastases. Prognosis was favourable even after relapse and was not related to the extent of surgical treatment. Limited surgery and suppressive hormonotherapy may be adequate therapy for thyroid carcinoma in children.

8 long-surviving children, treated for medulloblastoma with combination radiotherapy and chemotherapy after surgery were examined in order to assess general intelligence, immediate attention and prolonged attention. Their siblings or first cousins were selected as controls. The children with medulloblastoma performed significantly worse than their peers, both in intelligence tests and attention tests. A trend towards a positive correlation between age at the start of treatment and IQs was confirmed in our study, while there was no evidence of a correlation between IQs and time elapsed from therapy. The role of chemotherapy, radiotherapy and their combination effect in producing neuropsychological sequelae is discussed.

One hundred and twenty-one consecutive patients with monostotic Ewing's sarcoma (ES) were treated according to three consecutive combined modality programs from 1974 to 1986. Their 3-year progression free survival (PFS) rate from diagnosis of 59% was identical to the event free survival (EFS) rate, since all the 50 events occurring within 3 years from diagnosis were tumor recurrences. Primary tumor was treated with radiotherapy in 75 cases, surgical resection plus radiotherapy in 38, and radical surgery in 8. Chemotherapy was given to all patients and each program included adriamycin, vincristine, and cyclophosphamide +/- dactinomycin. Median follow-up was 12 years, ranging from 6 to 19 years. The PFS rate decreased to 49% at 6 years and plateaued at 46% after the 7th year from diagnosis, even though some relapses were observed as late as 14 years from diagnosis. Second malignancies developed in 7 patients free from progressive ES and were represented by osteogenic sarcoma in previously irradiated bone in 4 cases and by breast carcinoma in 3. No other event but tumor relapse or second malignancy occurred in this series. EFS rate was 47% at 6 years and 39% at 12 years, further decreasing in the following years because of a number of late events. A continuous PFS longer than 7 years may be consistent with cure in the majority of patients with monostotic ES. However, these patients should be followed indefinitely because of risk of second malignancies.

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