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Biomedical subjects

J B Beckwith

Publications and source records attributed to J B Beckwith.

At least 91 records · Page 5Linked to original sources
PubMed1991

Nephrogenic rests in Wilms tumor patients with the Drash syndrome.

The histological specimens from 12 patients with the Drash syndrome were identified from the National Wilms Tumor Study Group and reviewed for the presence of nephrogenic rests. Of 7 patients with the complete Drash syndrome 6 were evaluable for nephrogenic rests, including 5 (83%) who demonstrated intralobar nephrogenic rests. Of 5 (80%) partial Drash syndrome cases 4 (80%) were also intralobar nephrogenic rest positive. Neither group had perilobar nephrogenic rests identified. In a control population of Wilms tumor patients without the Drash syndrome only 39 of 274 (14%) with unilateral tumor had intralobar nephrogenic rests identified, whereas 26 of 92 (28%) bilateral cases had intralobar nephrogenic rests. There was a significantly higher rate of intralobar nephrogenic rests in complete and partial Drash syndrome cases than in the general Wilms tumor population (p less than 0.001). Wilms tumor patients with intralobar nephrogenic rests and the Drash syndrome present at a younger age and have a higher rate of bilaterality than rest negative Wilms tumor patients. The strong association of intralobar nephrogenic rests in the Drash syndrome approaches that found in the aniridia complex. However, in other syndromes associated with Wilms tumor, such as the Beckwith-Wiedemann syndrome and hemihypertrophy, there is a high prevalence of perilobar nephrogenic rests. In view of the high incidence of intralobar nephrogenic rests in complete and partial Drash syndrome patients, it is probable that events leading to Wilms tumor in patients with the Drash syndrome occur at an early stage in nephrogenesis.

Disorders of Sex Development↗
PubMed1991

Remaining problems in the treatment of patients with Wilms' tumor.

Major advances have been made in the treatment of children with Wilms' tumor. Remaining areas of investigation include the role of adriamycin and the treatment of children with stage III and IV disease and the role of whole lung radiation therapy in the treatment of children with pulmonary metastases. Long-term follow up of successfully treated patients will provide valuable insight into the importance of genetic factors in the cause of Wilms' tumor.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms' Tumor Study Pathology Center.

Fifty-six renal neoplasms reviewed by the National Wilms' Tumor Study Pathology Center presented with histologic features that resulted in confusion with rhabdoid tumor of kidney, a usually lethal childhood renal tumor; all were eventually diagnosed as other entities. Conspicuous filamentous cytoplasmic inclusions or large nucleoli, typical findings in rhabdoid renal tumors, were the usual source of diagnostic difficulty. Most, but not all, tumors occurred in pediatric patients. Sixteen were examples of Favorable Histology Wilms' tumor, which invited confusion with rhabdoid tumors either on the basis of filamentous cytoplasmic inclusions (15 cases) or macronucleoli (one case). In most cases, foci of typical Wilms' tumor blastemal aggregation or evidence of definitive nephrogenic differentiation facilitated the correct diagnosis. All 10 patients for whom information about outcome was available were alive at last follow-up. The other 40 renal lesions mimicking rhabdoid tumor of kidney consisted of a clinically and histogenetically diverse group of neoplasms, including anaplastic Wilms' tumor, congenital mesoblastic nephroma, renal cell carcinoma, transitional cell carcinoma, collecting-duct carcinoma, oncocytoma, rhabdomyosarcoma, malignant neuroepithelial tumors, and lymphoma. Most of these lesions could be separated from renal rhabdoid tumors and correctly classified on the basis of careful attention to light microscopic details, but in several cases electron microscopy or immunocytochemical studies were helpful or essential.

Adenoma↗
PubMed1991

Benign hemorrhagic adrenocortical macrocysts in Beckwith-Wiedemann syndrome.

A new pseudotumorous lesion found in the adrenal cortex of six infants with Beckwith-Wiedemann syndrome is described. These cystic masses were discovered either prenatally by using sonography or early in the neonatal period as palpable flank masses. Imaging studies, including sonography and CT, could not confidently exclude malignancy. After the masses were removed surgically, histologic examination showed them all to be benign hemorrhagic macrocysts within the capsule or permanent cortex (in contrast to neonatal adrenal hemorrhage, which usually occurs more centrally in the fetal cortex). The cysts were as large as 8 cm in diameter, and in one case a solitary cyst was predominant. Hemihypertrophy was present in all cases. Four of the six lesions were right-sided, and there was a male-female ratio of 5:1. Benign hemorrhagic adrenocortical macrocysts are a cause of abdominal mass in the fetus and neonate with Beckwith-Wiedemann syndrome.

Adrenal Cortex Diseases↗
PubMed1990

Pathologic delineation of the papillonodular type of cystic partially differentiated nephroblastoma. A review of 11 cases.

Eleven cases of a previously unrecognized papillonodular variant of cystic partially differentiated nephroblastoma (CPDN) are described. This type of CPDN has all the features of the conventional type of CPDN; however, in addition, there are grossly demonstrable papillonodular projections extending from the septa into the cyst lumina. The septa do not show any expansile tumor masses. Like most cases of the conventional type, this new type of CPDN was usually diagnosed in infants. Nephrectomy (total in 10 and partial in 1) was done in these cases. Additional chemotherapy with or without radiation therapy was given in seven cases. No recurrence was noted during the period extending from 21 months to 8 years in the eight cases in which follow-up data are available. Nephrectomy with regular follow-up visits for possible recurrence may be the management of choice. Pathologists should be aware of this variant of CPDN so that overtreatment can be avoided. The revised criteria for CPDN can be summarized as follows: (1) The discrete entirely cystic tumor contains luminal papillonodules in some cases. (2) Septa and the papillonodules, when present, are the only solid portion of the tumor and contain blastemal cells admixed with their normal and aberrant derivatives. (3) The tumor without and with papillonodules is classified as a conventional and papillonodular type of CPDN, respectively.

Adolescent↗
PubMed1990

Better survival after combined modality care for adults with Wilms' tumor. A report from the National Wilms' Tumor Study.

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.

Adolescent↗
PubMed1990

Overo lethal white foal syndrome: equine model of aganglionic megacolon (Hirschsprung disease).

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.

Animals↗
PubMed1990

Clear cell sarcoma of the kidney: accuracy of imaging methods for detecting bone metastases. Report from the National Wilms' Tumor Study.

To evaluate the accuracy of various imaging methods to screen for bone metastases in patients with clear cell sarcoma of the kidney (CCSK), all such cases reported on the National Wilms' Tumor studies from 1969-1983 were reviewed. Eighty-two CCSK children were identified, 19 of whom (23%) sustained bone metastases sometime during their course. Thirteen metastatic sites were evaluated by both radiography and bone scanning. In seven instances both modalities were positive; in four the radiography was positive but not the scan; and in only two (15%) was the scan positive in light of a negative radiograph. We recommend both imaging modalities in evaluating children with CCSK in order to detect all metastatic sites in all patients, and to further our observation that "hot" lesions on scintigraphy are more amenable to treatment than "cold" lesions.

Bone Neoplasms↗
PubMed1990

An anatomic comparison of cebocephaly and ethmocephaly.

Cebocephaly (hypotelorism, single-nostril nose) and ethmocephaly (hypotelorism, interorbital proboscis) lie in the middle of the spectrum of craniofacial changes associated with holoprosencephaly. Because these defects and thorough anatomic studies of them are rare, knowledge concerning morphologic as well as pathogenetic relationships is lacking. We report the autopsy findings and anatomic features of the dried skull of a 31 week fetus with cebocephaly and the craniofacial dissection of a 36 week fetus with ethmocephaly. Both manifested dysplastic changes of the ethmoid bone and anterior portion of the sphenoid bone, with concomitant hypotelorism and defects of the medial orbital walls. Through these latter defects, the eyes were joined in the ethmocephalic fetus (synophthalmia). Other changes of bone (single optic foramen, approximated maxillae, choanal atresia, thickened palate) and soft tissue (eccentric or fused extraocular muscles, single optic nerve) in both fetuses resembled those reported in other cases of cebocephaly and ethmocephaly, as well as cyclopia. In the 19th century, both cebocephaly and ethmocephaly were classified as two-orbit variants of cyclopia, a view supported by the present study.

Ethmoid Bone↗
PubMed1990

Benign nodal lesions mimicking metastases from pediatric renal neoplasms: a report of the National Wilms' Tumor Study Pathology Center.

Regional lymph node status is a key factor in the staging of pediatric renal tumors on the National Wilms' Tumor Study (NWTS). A review of cases entered on the NWTS has uncovered a number of cases where benign lymph node findings were mistaken for metastases. Most frequently, this was due to the presence of complexes of epithelial cells and Tamm-Horsfall protein within nodal sinuses. The epithelial cells were derived from damaged nephrons, usually resulting from obstruction by tumor. Another epithelial pseudometastic lesion, intranodal squamous epithelial cells, was found to originate from metaplastic calyceal urothelium. Benign mesothelial or coelomic inclusions similar to those previously described in pelvic and periaortic lymph nodes of adult females were found in nodes of four patients, including two boys, who are, to our knowledge, the first to be described with this finding. Other sources of confusion included protrusion of lymphoid follicles or germinal centers into nodal sinuses, thick endothelial cells of postcapillary venules mimicking epithelial tubules, nodal megakaryocytes resembling anaplastic nuclear changes, and histiocytic granulomas. Immunocytochemical methods were useful in evaluating some of these phenomena. Recognition of these pseudometastatic lesions is essential in order to avoid unnecessary and potentially hazardous therapeutic intensification.

Child↗
PubMed1990

Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms' tumor.

A new classification and terminology is proposed for precursor lesions of Wilms' tumor (WT), based upon morphology and natural history. The generic term nephrogenic rest (NR) is used for all WT precursors. Two major categories of NR are recognized: perilobar (PLNR) and intralobar (ILNR). Nephroblastomatosis signifies the presence of multiple or diffuse NRs. Nephroblastomatosis can be classified into four categories: (a) perilobar (PLNR only); (b) intralobar (ILNR only; (c) combined (PLNR and ILNR); and (d) universal. The individual rests can be subdivided into (a) nascent or dormant NRs; (b) maturing or sclerosing NRs; (c) hyperplastic NRs; and (d) neoplastic NRs. Of 282 evaluable unilateral WT specimens, 28.4% were definitely rest-positive, and an additional 12.4% were probably positive, with equal prevalence of PLNRs and ILNRs. Median age at diagnosis of WT was 36 months with PLNRs, 16 months with ILNRs, and 12 months if both types were present. PLNRs were strongly associated with synchronous bilateral WTs, and ILNRs with metachronous contralateral WTs. ILNRs were associated with aniridia and Drash syndrome, whereas PLNRs were more commonly found with hemihypertrophy and/or Beckwith-Wiedemann syndrome. The delineation of two distinct categories of WT precursors suggests pathogenetic heterogeneity for WTs. The biological and clinical implications of NRs are considered in the context of this classification.

Antineoplastic Agents↗
PubMed1989

Treatment of Wilms' tumor. Results of the Third National Wilms' Tumor Study.

The Third National Wilms' Tumor Study sought to reduce treatment for low-risk patients and find better chemotherapy for those at high risk for relapse. Eligible patients (1439) were randomized according to stage (I-IV) and histology (favorable [FH] or unfavorable [UH]), and contributed data to survival and relapse-free survival (RFS) analyses. Four-year (postnephrectomy) survival percentages and randomized treatment regimens for low-risk patients were 96.5% for 607 Stage I/FH patients who received dactinomycin (Actinomycin D [AMD], Merck Sharp & Dohme, West Point, PA) and vincristine (VCR) for 10 weeks versus 6 months; 92.2% for 278 Stage II/FH patients; and 86.9% for 275 Stage III/FH patients who received AMD + VCR +/- Adriamycin (ADR, Adria Laboratories, Columbus, OH) for 15 months. Stage II/FH patients also had either zero or 2000 cGy irradiation (RT) postoperatively and Stage III/FH patients either 1000 or 2000 cGy. Four-year survival was 73.0% for 279 high-risk patients (any Stage IV, all UH) who received postoperative radiation therapy (RT) and AMD + VCR + ADR +/- cyclophosphamide (CPM). Statistical analysis of survival and RFS experience shows that the less intensive therapy does not worsen results for low-risk patients and CPM does not benefit those at high risk.

Adolescent↗
PubMed1989

Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis.

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.

Adolescent↗
PubMed1989

Hyperdiploidy including trisomy 8 in a cystic partially differentiated nephroblastoma.

Cystic partially differentiated nephroblastoma (CPDN), a rare, cystic, renal lesion of childhood, has not been previously karyotyped. It is distinguished histologically from multilocular renal cyst by the presence of blastemal cells, and from Wilms' tumor by lack of expansile, solid growth and by indolent clinical behavior. In the present case, ten of 20 analyzed cells from a 3-week culture obtained from the tumor had a clonal, hyperdiploid karyotype. The modal chromosome number was 51, with chromosomes 8, 12, 17, 19, and 20 usually being present in three copies. Trisomy 8 was present in every hyperdiploid cell examined. A normal 46,XY constitutional karyotype was also observed. In degree and significance, the hyperdiploidy of CPDN is thus distinct from that reported in the prognostically unfavorable, anaplastic Wilms' tumor, where the DNA index is typically near-tetraploid. Trisomy 8, as a constitutional mosaicism, has been previously reported in children with bilateral CPDN and/or undifferentiated sarcomas, although none of their tumors were karyotyped. The present findings support a neoplastic nature for CPDN, while emphasizing its pathogenetic distinctiveness from Wilms' tumor, and provide further evidence for significance of trisomy 8 in the pathobiology of this tumor.

Chromosomes, Human, Pair 8↗
PubMed1989

The value of the pediatric postmortem examination.

Requesting an autopsy is never a pleasant or comfortable experience, either for physician or for family members. Sometimes the family has had previous unhappy experiences with this procedure, and certainly many clinicians have been repelled by sights, sounds, and behaviors they have observed in morgues. Such experiences can have far-reaching negative effects and reflect poorly upon the profession of pathology. Berger's important paper contains important messages for pathologists concerned with the performance of pediatric autopsies and for clinicians requesting consent. His paper should be read and heeded by both groups. Several studies have looked at factors that might affect autopsy rates, especially in perinatal deaths. Early separation of mother from baby emerged as an important factor in one of these, whereas maternal age, race, and sociocultural background played no important role. One of these studies investigated attitudes and experiences of the requesting physicians, and found these factors to be key determinants in the family's response to a request to perform a postmortem examination. One result of that study was that more experienced physicians were more likely to request a postmortem examination, and to receive an affirmative answer. The pediatric postmortem examination remains an indispensable part of clinical management. Its importance for science, clinical practice, and medical administration is beyond question. However, its greatest and most unique value is to the families of the deceased children. Clinicians and pathologists must continue to work together to ensure that this procedure is performed whenever indicated, that the information gained is maximized, and that the results are communicated to the family promptly, accurately, clearly, and with sensitivity.(ABSTRACT TRUNCATED AT 250 WORDS)

Autopsy↗