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J B Beckwith

Publications and source records attributed to J B Beckwith.

At least 109 records · Page 6Linked to original sources
PubMed1989

Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms' Tumor Study Pathology Center.

We review 111 cases of rhabdoid tumor of kidney (RTK), including 79 entered on the National Wilms' Tumor Study (NWTS). Median age at diagnosis was 11 months, with a range from 0 to 106 months. The male:female ratio was 1.5:1. Gross features included a characteristic involvement of perihilar renal parenchyma. A wide histological spectrum was encountered, including nine major morphological patterns (classical, epithelioid, sclerosing, lymphomatoid, histiocytoid, etc.). These appearances invite confusion with other renal neoplasms. Ultrastructural studies were performed in 20 cases; immunocytochemical studies were performed in 11. Vimentin was demonstrated in all tumors; epithelial membrane antigen was seen in 7. Nonspecific decoration of cytoplasmic inclusions by a variety of immunostains was found in several cases. Several findings suggested that RTK might arise from primitive cells involved in formation of the renal medulla. There was no evidence of a histogenetic relationship to Wilms' tumor, although RTK may overlap with mesoblastic nephroma and clear cell sarcoma. Of the 70 NWTS patients with adequate follow-up, 56 (80%) have died. Every patient presenting with distant metastases died, whereas 10 of 20 with negative nodes survived. Survival rates were higher for girls (56.3% versus 11.1%). None of the histological variables had independent prognostic significance.

Antigens, Neoplasm↗
PubMed1988

Age distribution of Wilms' tumor: report from the National Wilms' Tumor Study.

Median ages at diagnosis were 36.5 and 42.5 mo for 1523 males and 1678 females with unilateral Wilms' tumor registered between October 1969 and December 1985; they were 23.5 mo and 30.5 mo for 100 males and 141 females with bilateral disease. The median age for multicentric, unilateral cases was intermediate between the bilateral and unicentric medians. Patients with hemihypertrophy in addition to their Wilms' tumor had a typical age distribution, whereas those with aniridia or characteristic genitourinary anomalies were substantially younger. Patients with perilobar nephroblastomatosis had a median age of 35.5 mo and those with intralobar nephroblastomatosis, a median age of 18.5 mo. Most of the bilateral disease occurred in the presence of one or both of these precursor lesions. These findings suggest heterogeneity in the pathogenesis of Wilms' tumor, having implications for genetic counselling, and call into question certain aspects of Knudson and Strong's two-stage mutational model for the origin of Wilms' tumor.

Adult↗
PubMed1988

Anaplasia in unilateral Wilms' tumor: a report from the National Wilms' Tumor Study Pathology Center.

Fifty-eight unilateral anaplastic Wilms' tumors entered on the Third National Wilms' Tumor Study (NWTS-3) were reviewed. The favorable outcome of stage I anaplastic tumors was confirmed. In stage II-IV tumors, the extent and severity of anaplasia were not of prognostic importance. However, two histologic features were correlated with extremely poor outcome; anaplasia in extrarenal tumor sites (P = .016) and predominantly blastemal tumor pattern (P = .020). The importance of these features in predicting relapse and death for children with anaplastic Wilms' tumor was supported by the study of 47 additional anaplastic Wilms' tumor cases from NWTS-2. These results emphasize the importance of examining sites of extrarenal spread in anaplastic Wilms' tumors. Cytologic criteria for anaplasia and clues to its recognition are reviewed, and the potential clinical and biological implications of our findings are discussed.

Cell Transformation, Neoplastic↗
PubMed1988

Intrathoracic petechial hemorrhages: a clue to the mechanism of death in sudden infant death syndrome?

(1) Intrathoracic petechiae are characteristic of most SIDS cases, and tend to be more numerous in these cases than in deaths from other causes, including mechanical asphyxia. (2) Their localization suggests that intrathoracic negative pressure plays a role in their genesis. (3) Several human and animal studies suggest that petechiae arising from the pulmonary circulation may differ from those originating from systemic vessels in the thorax. (4) Experimental studies suggest that vigorous respiratory efforts are responsible for their formation. This would seem to exclude respiratory paralysis as a mechanism for most SIDS deaths. (5) These petechiae are not consistent with cardiac arrest or failure as the primary agonal event in SIDS. (6) The fact that petechiae are more prominent in SIDS than in other deaths adds to the evidence that SIDS is not a "wastebasket" diagnosis, despite the imperfection of existing diagnostic criteria. (7) These petechiae do not prove that the final mechanism of most SIDS deaths is upper airway obstruction. However, they do provide substantial support for that thesis.

Animals↗
PubMed1987

Relapse-associated variables in stage I favorable histology Wilms' tumor. A report of the National Wilms' Tumor Study.

All 24 cases of confirmed and evaluable Stage I favorable histology (FH) Wilms' tumor (WT) relapsing to date on the Third National Wilms' Tumor Study (NWTS-3) were compared with 48 matched control subjects who had not relapsed for at least 2 years after diagnosis. Fifteen of the clinical and pathological variables studied, including patient age and tumor size, had no significant relationship to the outcome of this study. Four histologic features, all related to the degree of tumor extension within the "tumor-kidney unit" proved to be significantly associated with relapse. These were (1) invasion of the tumor capsule, (2) presence of an "inflammatory pseudocapsule," (3) renal sinus invasion, and (4) tumor in intrarenal vessels. One or more of these features was present in 100% of relapsed cases (excluding one for which two variables were unevaluable), but occurred in only 46% of controls (P less than 0.0002). Therefore, no relapses occurred in the NWTS-3 when all four variables were negative. This result was confirmed by a review of all Stage I cases in the NWTS-1 who had relapsed and who were treated by single-agent chemotherapy. Again, no relapses occurred when all four factors were negative. These results demonstrate the feasibility of "microsubstaging" and could serve as a basis for future refinements of therapy for Stage I favorable histology Wilms' tumor.

Child↗
PubMed1987

Cystic hamartomata of lung and kidney: a spectrum of developmental abnormalities.

We report on a developmental malformation of the lung and kidney which has not been previously described and which we have chosen to call "cystic hamartomata of the lung and kidney" to emphasize the non-malignant nature of these lesions. We also confirm a previous case report by Weinberg and Zumwalt [1977] as a different distinct disorder that results in a multifocal cystic hamartomata of the lung with associated marked parenchymal overgrowth of the kidney (the Weinberg-Zumwalt syndrome). These cases represent a spectrum of abnormal morphogenesis affecting both kidney and lung. Patients 1 and 2 presented during infancy with abdominal masses and hypertension due to bilateral multilocular cysts of the kidney with associated hamartomatous pulmonary cysts; patient 2 also had one area of cellular mesoblastic nephroma. Patient 3 demonstrated markedly hyperplastic renomegaly with medullary dysplasia in association with bilateral cystic hamartomata of the lungs. During the fifth week of gestation, the ureteric bud invades the unsegmented mesoderm that becomes the metanephric system, and the lung bud invades the splanchic mesoderm, which provides the stimulus for its growth. We suggest that the predominant pattern of a congenital kidney or lung hamartoma might reflect the timing of a prenatal neoplastic event affecting these developmental processes.

Hamartoma Syndrome, Multiple↗
PubMed1987

Bilateral Wilms tumor.

Despite institutional variation in the treatment of synchronous bilateral Wilms tumor, a 76 per cent over-all 3-year survival rate has been achieved. We reviewed 145 patients with stage V disease (synchronous bilateral Wilms tumor) registered with National Wilms Tumor Studies 2 and 3. The majority of the patients (94, or 65 per cent) underwent initial surgical resection followed by chemotherapy with or without radiation, with a 3-year survival rate of 82 per cent. However, survival of 45 patients who underwent initial biopsy, postoperative chemotherapy and second-look surgery was 57 per cent at 3 years, a result not significantly different statistically from the 82 per cent figure. Only 38 per cent of the patients had all tumor resected at 1 or more operations. Therefore, emphasis is being placed on chemotherapy as the predominant treatment modality for this disease. Unfavorable histology was found in 10 per cent of the patients and 6 patients (4 per cent) had discordant histology, that is unfavorable histology on 1 side with contralateral favorable histology. Better prognostic factors were patient age less than 3 years at diagnosis, lower stage of the most advanced lesions, favorable histology and negative nodal involvement. Currently, routine bilateral biopsy of tumors is recommended, followed by chemotherapy according to the stage of the most advanced lesion. Impressive shrinkage of tumor can be expected with the hope that surgery will allow renal preservation (excisional biopsy or partial nephrectomy) rather than nephrectomy.

Adolescent↗
PubMed1987

Ultrastructure and histogenesis of the renal tumors of childhood: an overview.

This review discusses the ultrastructural and immunohistochemical features of the common childhood renal tumors, with an emphasis on their diagnostic usefulness. Speculations regarding their histogenesis also are presented, with the hope that these may serve to diminish some of the confusion surrounding the classification of these morphologically diverse lesions.

Antibodies, Neoplasm↗
PubMed1986

Clinicopathologic features and prognosis for Wilms' tumor patients with metastases at diagnosis.

Comparisons were made between 236 Wilms' tumor patients with metastasis to the lungs and/or liver at initial diagnosis who were registered on the National Wilms' Tumor Study (NWTS) during 1969 to 1983, and 1755 patients who did not have overt metastases at diagnosis. Patients with evidence of regional spread of disease beyond the kidney, especially if to the renal vein or lymph nodes, were much more likely to have overt metastases present at diagnosis than those with apparently localized disease. The presence of metastases was also correlated with age at diagnosis, ranging from 1% among infants younger than 1 year of age to 24% for those aged 6 years or older. The percentage of tumor deaths for patients with metastases at diagnosis (Stage IV) and a primary tumor of favorable histology (FH) declined from 29% at 2 years postdiagnosis on the first therapeutic trial (NWTS-1) to 9% for the most recent one (NWTS-3), and is now comparable to that for patients without metastases but with nonresectable local invasion at diagnosis (Stage III). The local extent of disease also influenced the survival outcome for Stage IV/FH patients. Survival was poor for those with anaplastic or sarcomatous (unfavorable) histology, regardless of local staging or trial. There was no difference in survival according to metastatic site (liver +/- lung vs. lung only) if present prior to treatment. By contrast, patients who developed liver metastases during or after treatment had an especially poor chance for survival as compared with those who developed lung deposits at those times.

Adolescent↗
PubMed1986

Fatty liver in sudden childhood death. Implications for Reye's syndrome?

We studied the livers of 21 children dying a traumatic death or sudden unexpected natural death and found that fatty metamorphosis of the liver appears to be ubiquitous. Therefore, the finding of microvesicular panlobular fatty liver alone does not justify the diagnosis of Reye's syndrome. In fact, because fatty change appears to be so prevalent, previous reports that relied on fatty changes as diagnostic of Reye's syndrome cannot be validated.

Adolescent↗
PubMed1986

Wilms' tumor in the neonate: a report from the National Wilms' Tumor Study.

Renal neoplasms in the neonate are quite uncommon. Twenty-seven of the 3,340 patients (0.8%) registered on the National Wilms' Tumor Studies from 1969 through April 1984, were 30 days old or less. Of these 27 patients, 18 had mesoblastic nephroma, 1 had a malignant rhabdoid tumor of the kidney, and 4 others had nonneoplastic lesions. The remaining four infants were reviewed in detail. All had favorable histology Wilms' tumors; none had distant metastasis at diagnosis. Treatment ranged from surgery alone to excision plus three-drug therapy for 15 months. All fared well. The patient with Stage I rhabdoid tumor died at eight weeks of age in spite of aggressive four-drug therapy. This review supports the view that Wilms' tumor in the neonate is extremely rare.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Calvarial hyperostosis: a benign X-linked recessive disorder.

We report a family with what appears to be a unique X-linked recessive disorder of isolated hyperostosis of the calvarium. Although irregularity of the calvarium and exophytic prominences of the frontoparietal bones were apparent in infancy, premature cranial suture closure did not occur and there was no evidence of increased intracranial pressure despite a Luckenshadel appearance of the skull. Other membranous bones and the tubular bones were not involved. Calvarial bone biopsy from one patient showed vacuolated histiocytes suggesting a storage disease; however, neurologic deterioration, hepatosplenomegaly, and dysostosis multiplex did not occur. The affected family members had normal stature, normal occipitofrontal circumference, and no other medical problems. The biochemical basis of this disorder is not known. Although storage of abnormal material is possible, the long-term prognosis seems favorable.

Female↗