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The importance of measuring attitudes of trainee professionals to people with disabilities was addressed. A questionnaire which included the Scale of Attitudes toward Disabled Persons (SADP) and the Intellectual Disability Misconceptions Scale (IDMS) was completed by first and final year tertiary students enrolled in a 3-year course on intellectual disability. The attitude measures did not discriminate between the two groups of students, psychometric properties were poor and the factor structure of the SADP did not replicate previous findings. As expected, there was some convergence between the two attitude measures, and relationships to subject variables were consistent with previous research. Recommendations for subsequent research were discussed.

Adults with Wilms' tumor (WT) have had a poor prognosis. More recently, therapies used in children, who have a better outlook, are also being employed for adults. This study was undertaken to see whether adults with WT have benefited. The results of treatment of 27 adults with WT were reported to the National Wilms' Tumor Study (NWTS) from 1979 to 1987. The ages of the 27 adults ranged from 16 to 74 years (median, 24 years). Four had anaplastic WT, and 23 had favorable histology (FH) WT. All but one patient underwent nephrectomy, 21 were given postoperative radiation therapy, and 25 received chemotherapy. The agents used most often included actinomycin D (AMD), vincristine (VCR), and doxorubicin (Adriamycin; ADR). There were six Stage I, five Stage II, four Stage III, 11 Stage IV, and one Stage V patients. The 3-year survival rate is 67%. These results are better than the 24% reported by the NWTS in the past for adults with WT. Analyses of the therapies given to the 27 adults lead to the following recommendations: for Stage I/FH patients, 6 months of postoperative chemotherapy using AMD + VCR without postoperative radiation therapy; and for Stage II, III, and IV/FH, VCR + AMD + ADR for 15 months + 2000 cGy to the tumor bed, 1200 to 1500 cGy to the lungs, 2000 cGy to the liver, and 3000 cGy to other sites as appropriate in patients with metastases at diagnosis.

The lethal white foal syndrome (LWFS) is a congenital abnormality of overo spotted horses which is a model for human aganglionic megacolon or Hirschsprung disease. Foals with LWFS have an all white, or nearly all white, coat. They also present clinically with an intestinal obstruction that proves fatal within the first few days of life. The LWFS involves both melanocytes and intestinal ganglion cells, and appears to result from a genetic defect involving neural crest cells. This report describes pathologic studies of two recent cases of LWFS. Two different hypothetical models of inheritance of LWFS are presented and discussed.

In the Third National Wilms' Tumor Study, (NWTS-3), 190 patients with Favorable Histology (FH) Wilms' tumor (WT) were identified as having tumor adherent to, directly invading, or metastatic to the liver at diagnosis. Analyses of the 3-year relapse-free survival and survival of these patients show that adhesion to the liver surface, direct invasion of the liver, and liver metastases have no additional detrimental effect on prognosis stage-for-stage. The authors conclude that hepatic involvement, when present at the time of diagnosis, should not be regarded as different from other patterns of the disease. Treatment policies should follow those appropriate for stage.

A case of bone metastasizing renal tumor (clear cell sarcoma) of childhood (BMRTC) is presented. Histologic examination demonstrated the typical histopathologic features of this type of tumor. In addition, epithelial cells were present which occurred in groups, either around a central eosinophilic core or occasionally around a small central lumen. To the authors' knowledge, the presence of epithelial cells in BMRTC has not been reported before. The histogenetic implication of this finding is discussed.

Detailed histological analysis of 427 cases entered on the first National Wilms' Tumor Study revealed that lesions with foci of marked cytological atypism (anaplasia), and those composed predominantly of sarcomatous stroma, were associated with unfavorable outcome. Twenty-five patients had anaplasia, and 24 had sarcomatous lesions of which a total of 28 (57.1%) died of tumor. Three hundred and seventy-eight patients had tumors which showed neither of these features, and only 26 (6.9%) died of tumor. Seven of ten deaths due to tumor in patients diagnosed before two years of age were associated with sarcomatous lesions. Three sarcomatous patterns were recognized, of which one, designated "clear cell" sarcoma, had a predilection for bony metastases. Using criteria defined and illustrated in this paper it is possible to identify in advance those patients likely to do poorly using current therapeutic approaches.

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.