The International Neuroblastoma Pathology Classification, a system based on a framework of the Shimada classification with minor modifications, is proposed for international use in assessing NTs.

EBV can infect smooth-muscle cells, at least in patients with AIDS, and it may contribute to the pathogenesis of leiomyomas and leiomyosarcomas in children with AIDS. EBV seems to play no part in smooth-muscle tumors in HIV-negative children.

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The entities commonly known as multi-locular cyst of the kidney (MLC) and cystic partially differentiated nephroblastoma (CPDN) were reviewed, based on material in the National Wilms' Tumor Study Pathology Center. The authors recommend several modifications of existing terminology and definitional criteria for these lesions. Because MLC probably represents a neoplastic lesion, the designation "cystic nephroma" (CN) is preferred. This term should be used only for predominantly cystic tumors composed entirely of differentiated tissues, without blastema or other embryonal elements. The designation CPDN should be applied to predominantly cystic lesions, lacking nodular solid regions, in which blastemal or other embryonal cells are present in the septa of the cysts. Solid Wilms' tumor with multifocal cystic change should be distinguished from CPDN. Five cases of CN and 18 cases of CPDN were reviewed. No CN, for which follow-up data was available, showed aggressive behavior. Only one case of CPDN underwent local recurrence, and there were no metastases. In general, nephrectomy alone appears to be adequate therapy for CPDN, but regular monitoring by noninvasive techniques would seem advisable.

Our review confirmed that the survival of infants with stage D(S) NB is excellent. However, subsets of patients with poor prognosis can be identified by young age and unfavorable biologic factors. More effective therapy is needed for the group of stage D(S) infants who show unfavorable clinical and biologic features.

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Neuroblastomas with N-myc amplification have a characteristic histopathologic phenotype and an aggressive clinical course. In contrast, neuroblastomas without N-myc amplification exhibit a wide range of histologic features that can define prognostic subsets.