Neuroblastoma is one of the most common tumors in childhood. However, it often has been difficult to compare clinical and laboratory studies of this disease due to a lack of uniform criteria for diagnosis, staging, and response. An international group of conferees addressed each of these issues and reached a consensus. Specific criteria for making a diagnosis of neuroblastoma are defined. A new neuroblastoma staging system is proposed that takes into account the most important elements of current but incompatible systems. Finally, criteria for response to treatment are standardized. The criteria proposed herein represent an international consensus of essentially every major pediatric oncology group or organization in the United States, Europe, and Japan. The staging system should be referred to as the International Neuroblastoma Staging System, and the response criteria as the International Neuroblastoma Response Criteria. Implementation of these criteria will greatly facilitate the comparison of clinical and laboratory studies by different groups and countries. Furthermore, these criteria should serve as a foundation on which future modifications or improvements can be based.
Thirty-three patients with malignant testicular teratomas and para-aortic metastases exceeding 2 cm in diameter have been treated with radiotherapy, chemotherapy (vinblastine and bleomycin, with or without cis-platinum) or both, followed by surgical excision of the residual para-aortic mass. Removal of a poorly functioning ipsilateral kidney was necessary in 7 cases (21%) and a segment of vena cava was resected in 2 (12.5%) of 16 patients with primary right-sided tumours. Removal was incomplete in only one patient, who had femoral nerve root involvement, and he subsequently died of secondary haemorrhage (operative mortality 3%). Undifferentiated tumour was found in 8 (61%) of 13 patients (Group 1) after radiotherapy, compared with only 2 (15%) of 13 (Group 2) after chemotherapy and radiotherapy, and 4 (57%) of 7 (Group 3) who had chemotherapy only prior to surgery; however, 3 of the Group 3 patients were referred because of evidence of activity in the para-aortic region.
This study demonstrates that with effective multiagent chemotherapy, histologic subtype does not influence outcome. The high relapse rates in stage I MC subtype indicates that MC HD is biologically aggressive and systemic treatment with or without local irradiation may be indicated. The high relapse rate in stage IV patients appeared to be independent of histology.
al., 1985). Whilst factors predictive of relapse in both adults (Kennedy et al., 1985) and children (Mauch et al., 1983;Robinson et al., 1984;Russell et al., 1984)
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