Preoperative chemotherapy in the treatment of Wilms' tumor diagnosed with the aid of fine needle aspiration biopsy.
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Antineoplastic Combined Chemotherapy Protocols↗
Biomedical subjects
J B Beckwith
Publications and source records attributed to J B Beckwith.
Bone metastasizing renal tumor (clear cell sarcoma) of childhood with epithelioid elements.
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.
Bone Neoplasms↗
Recurrence of Wilms tumor after apparent cure.
The recurrence of Wilms tumor after a 5-year disease-free interval is rare. We present two patients who had recurrent disease after a disease-free interval of greater than 7 years. Three additional patients, registered with the National Wilms Tumor Study who had a recurrence after 5 years, are also described. Of these three patients, two had nephroblastomatosis. Because more patients are achieving long-term survival, careful surveillance after apparent "cure" is recommended, particularly if nephroblastomatosis is identified in the original nephrectomy specimen.
Child, Preschool↗
Anaplastic Wilms' tumor: clinical and pathologic studies.
A review of almost 1,200 children participating in the first and second National Wilms' Tumor Study (NWTS-1 and -2) has demonstrated a number of significant differences in the clinical presentation and response to therapy of anaplastic and nonanaplastic Wilms' tumor. Compared to their counterparts, children with anaplastic Wilms' tumor were generally one to two years older at diagnosis, more were non-white, and more had lymph node metastases at diagnosis. Consistent with previous studies, children with anaplastic Wilms' tumor survived for a significantly shorter time than those with non-anaplastic Wilms' tumor. A hopeful outlook, however, was suggested by the NWTS-2 experience since the more aggressive chemotherapies used in this study appear to have substantially improved the survival of patients with diffusely anaplastic tumors. Also, the survival of NWTS-2 patients with anaplastic Wilms' tumor was determined in part by clinicopathologic stage. It may be possible therefore to refine therapy according to stage so as to provide children with localized disease a chance for cure with fewer untoward treatment-related sequelae.
Anaplasia↗
Prognosis for Wilms' tumor patients with nonmetastatic disease at diagnosis--results of the second National Wilms' Tumor Study.
Multivariate statistical methods were used to study prognosis for 632 patients entered on the second National Wilms' Tumor Study who had nonmetastatic, unilateral disease at diagnosis. Separate analyses were conducted for each of four endpoints: abdominal recurrence, distant metastasis, relapse without regard to site, and death. The two most important predictors for metastasis and general relapse were an unfavorable (anaplastic or sarcomatous) histology and the presence of microscopically confirmed disease in the regional lymph nodes. Operative spillage of tumor increased the rates of abdominal recurrence and death, even after accounting for histology and lymph node effects. The presence of a tumor thrombus in the renal vein or IVC increased the risk of metastasis, and intrarenal vascular invasion was associated with general relapse after accounting for histology, lymph nodes, and spillage. However, these latter associations were weaker, and some uncertainty remains regarding the true prognostic import of such findings due to a high degree of collinearity among variables. By contrast to the results of a similar data analysis for the first National Wilms' Tumor Study, specimen weight had no bearing on outcome, and the effects of age at diagnosis were entirely explained by the association of age with other more critical factors.
Adolescent↗
Clear cell sarcoma of the kidney with emphasis on ultrastructural studies.
Clear cell sarcoma of the kidney (CCSK) is a malignant childhood tumor distinguished from Wilms' tumor by its gross, microscopic, and ultrastructural morphologic features and its propensity to metastasize to bone. Some clinical features of 75 cases of CCSK registered in the National Wilms' Tumor Studies I-III are presented along with the results of electron microscopic study of 12 tumors. Ultrastructural features of the tumor are compared to those of tumors of pericytes, Schwann's cells, renomedullary interstitial cells, fibroblasts, other renal tumors, and other clear cell tumors. Fine structural properties of primitive cells with variable numbers of organelles do not strongly suggest a histogenetic origin. The effect of formalin fixation on perinuclear cytoplasmic filaments and the tendency of tumor cell extensions to enfold the pale extracellular matrix explain the vacuolated appearance of some tumor cells.
Adolescent↗
The association of embryonal tumors originating in the kidney and in the brain. A report of seven cases.
This report documents, in seven infants younger than 2 years of age, a previously unrecognized association of a renal embryonal neoplasm (malignant rhabdoid tumor in six patients and a Wilms' tumor in one) with an embryonal primary tumor originating in the central nervous system. The neuroepithelial tumors included three cerebellar medulloblastomas, one pineoblastoma, one primitive neuroepithelial tumor (probably cerebral neuroblastoma), one malignant subependymal giant cell astrocytoma, and one cerebellar medulloepithelioma with divergent glial and neuronal differentiation. There is no evidence that this association is based on the selective neoplastic transformation of embryonal cells of similar histogenetic or cytogenetic origin. The relationship between these dissimilar, embryologically unrelated tumors remains enigmatic.
Astrocytoma↗
Left ventricular hypoplasia in congenital diaphragmatic hernia.
Quantitative anatomic study of the hearts of eight infants with left-sided congenital diaphragmatic hernia (CDH) has revealed significantly decreased cardiac mass, due to hypoplasia of the left atrium and ventricle and interventricular septum. These morphologic deficiencies may be a result of compression of mediastinal structures by herniated abdominal viscera during prenatal life. Left ventricular hypoplasia is likely to be an important factor in the pathogenesis of cardiac insufficiency in patients with left CDH.
Female↗
Rhabdoid tumors of the kidney contain mesenchymal specific and epithelial specific intermediate filament proteins.
The intermediate filament proteins of rhabdoid tumors of the kidney were investigated with a panel of monoclonal antibodies to different intermediate filament proteins. Rhabdoid tumor cells are decorated by an antivimentin antibody and by an antibody made against a 54-kilodalton (kd) cytokeratin from human hepatoma cells. The rhabdoid tumor cells fail to react with an antibody generated against keratin from stratum corneum or with an anti-200-kd neurofilament protein antibody. Cytoskeleton preparations of rhabdoid tumor cells grown in vitro demonstrate the presence of vimentin (58 kd) and the 54-kd cytokeratin. Thus, these cells contain two different intermediate filament proteins characteristic of epithelial and mesenchymal cells. We also demonstrate that rhabdoid tumor cells can form tumors in athymic (nude) mice and that the intracytoplasmic globules are present in the nude mouse lesions.
Animals↗
Wilms' tumor and other renal tumors of childhood: a selective review from the National Wilms' Tumor Study Pathology Center.
Selected studies of Wilms' tumor and related renal neoplasms in children, which have been based on the National Wilms' Tumor Study Pathology Center collection of more than 2,600 renal tumors of childhood, are reviewed. The purpose of the review is to illustrate the value of the collaborative approach to uncommon pathologic specimens and to distinguish several tumors often confused with Wilms' tumor, including renal adenocarcinoma, renal teratoma, and renal neurogenic tumors. The unfavorable prognostic significance of anaplastic cells in Wilms' tumor is emphasized. Two recently described clinicopathologic entities--clear cell sarcoma of kidney and malignant rhabdoid tumor of kidney--and morphologic variants often confused with Wilms' tumor or congenital mesoblastic nephroma of infancy are discussed. Clear cell sarcoma of kidney and malignant rhabdoid tumor of kidney are, apparently, distinctive neoplasms, not Wilms' tumor variants.
Adenocarcinoma↗
Homogeneous scales for the neurotic triad of the MMPI.
This study used item statistics for the nonduplicated items of the MMPI and the CPI to develop homogeneous scales for the neurotic triad (Hs, D, Hy) of the MMPI. The construction and partial cross-validation of scales were based on data from a predominantly clinical sample of 563 male subjects and 347 female subjects. Two non-overlapping scales of high internal consistency were developed which contained embedded subscales designed to be relatively independent of factor dimensions previously identified in the data. The first of the two scales comprised items exclusively related to health concern, and the second was a more concentrated measure of poor morale than the MMPI Depression scale. The nature of the revised scales was analyzed in relation to the standard MMPI scales and factor dimensions from previous research. Implications of the study for development of an hierarchical inventory were discussed.
Adolescent↗
Histopathology as a prognostic indicator in tumors of childhood.
Because of their relative rarity and structural complexities, many of the solid tumors of children present formidable obstacles to the development of histopathological grading systems. However, the cooperative clinical trials now in progress in many nations provide a unique opportunity to overcome these difficulties. The results of the NWTS are briefly summarized as an illustration of the potential value of this approach.
Child↗
A specific antigenic defect of the basement membrane is found in basal cell carcinoma but not in other epidermal tumors.
The basement membrane of basal cell carcinoma was characterized by indirect immunofluorescence using antibodies to laminin, type IV collagen, and bullous pemphigoid antigen, three distinct protein components of basement membrane. Aggregates of basal cell carcinoma in the dermis were surrounded by a continuous basement membrane containing laminin and type IV collagen; however, bullous pemphigoid antigen was either completely undetectable or faint and discontinuous rather than linear. In contrast to this specific defect in bullous pemphigoid antigen found in basal cell carcinoma, well-differentiated superficially invasive epidermal squamous cell carcinoma and several benign epidermal tumors (trichoepithelioma, wart, keratocanthoma, seborrheic keratosis, cylindroma) displayed all three antigens in the basement membrane that surrounded epithelial cell aggregates. Cylindroma displayed in addition, broad areas of type IV collagen in the hyalinized material between epithelial islands. Basal cell carcinoma was the only tumor examined in which there was a specific antigenic defect in the basement membrane. This defect in bullous pemphigoid antigen may be due to abnormal synthesis by the tumor cells and could be related to the absence of differentiation of these cells.
Antigens, Neoplasm↗
Pathogenesis of congenital tumors and malformations of the sacrococcygeal region.
Skin-covered lesions in the sacrococcygeal region include an unusual array of congenital tumors and malformations. Excluding the easily recognized meningomyelocele, there are over 50 other conditions that occur, many of which seem to be important in linking the disciplines of teratology and oncology. While it is impossible to precisely determine the manner in which these arise, there is suggestive evidence that early structures incorporated within the caudal cell mass of the embryonic tail may play an important role. The present paper reviews the basis for this speculation from both the literature and a series of 136 cases.
Ependyma↗
Parasitic conjoined twins, two cases, and their relation to limb morphogenesis.
Reports of parasitic conjoined twins have appeared rarely in the scientific literature and only one study has included a detailed description of the parasitic extremities. We report here two specimens consisting for the most part of normal hosts with ectopic legs attached to the thorax (case 1, thoracoparasitus) or the abdomen (case 2, gastroparasitus). These two specimens are in agreement with several others reported in the literature in having no sign of vertebral columns. These specimens also exhibit a marked decrease in innervation (case 1) or complete absence of somatic nerves (case 2). This finding is in complete contradiction with the idea of the dependence of limb skeletal structure upon peripheral nerves. The hypothesis is presented that this type of parasitic twin may result from duplication of the Wolffian ridge.
Bone and Bones↗
Therapy and outcome in 51 children with mesoblastic nephroma: a report of the National Wilms' Tumor Study.
Mesoblastic nephroma is a distinctive pathologic renal tumor with unique clinical, therapeutic, and prognostic patterns. This report reviews 51 patients (2.8% of 1905 patients submitted to NWTS with renal tumors) with this diagnosis from the NWTS contrasting this disease entity with Wilms' tumor. There were 33 males and 18 females, predominantly term babies born after uncomplicated pregnancies. The mean age at tumor excision was 3.44 +/- 0.6 mo (one child 9 yr). A palpable mass was the predominant presentation in 48 patients and in addition hematuria (9), hypertension (2), vomiting (3), and jaundice (1) were noteworthy. Diagnostic studies included IVP (49 positive, 2 negative) and ultrasound (15 positive, 1 negative). Adequate operative excision was achieved in 43 of 51 patients while 8 children had local extension and 10 had tumor spillage. Operation alone (23), predominantly since 1978, surgery plus chemotherapy (24), prior to 1978, and surgery, chemotherapy, and radiation therapy (4), prior to 1976, were the modes of therapy. The follow-up ranges from 4 mo to 11.5 yr. Survival was excellent; 50 patients survive (98%), only 1 dying of sepsis. These data suggest that mesoblastic nephroma contrasts with Wilms' tumor in that it occurs in a younger age group, has a benign biologic behavior, and a more favorable outcome. Aggressive multimodal treatment though utilized in the early years of the study is not essential to achieve this outcome.
Age Factors↗
Elevated values of tri-iodothyronine in victims of sudden infant death syndrome.
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Humans↗
Indirect immunofluorescent localization of benzo[a]pyrene adducted to nucleic acids in cultured mouse keratinocyte nuclei.
The localization of benzo[a]pyrene-deoxyguanosine adducts was studied by indirect immunofluorescence in cultured BALB/c epidermal cells exposed to (+/-) 7 alpha, 8 alpha-dihydroxy-9 alpha, 10 alpha-epoxy-7,8,9,10-tetrahydrobenzo[a]pyrene (the anti-isomer) utilizing an antiserum specific for the major benzo[a]pyrene-deoxyguanosine adduct in DNA. This antiserum does not cross-react with benzo[a]pyrene or DNA alone. When cultured keratinocytes were incubated with the carcinogen for 1 h, the immunofluorescence was localized in the nucleus as intense spots on a background of diffuse fluorescence. Fluorescence was absent from cells not exposed to carcinogen and from carcinogen-exposed cells incubated with normal rabbit serum in place of the antiserum. Fluorescence was abolished when the specific antiserum was absorbed with the immunogen DNA prior to incubation with cells, and substantially diminished when exposed cells were preincubated with deoxyribonuclease before the application of the specific antiserum. Incubation of exposed cells with ribonuclease prior to incubation with the specific antiserum removed the bright fluorescent spots and resulted in fluorescent nuclei containing dark sports in similar frequency. Dose-response studies in which benzo[a]pyrene-deoxyguanosine adducts were quantified by enzyme-linked immunosorbent assay and compared with intensity of immunofluorescence demonstrated that decreasing doses of the carcinogen resulted in fewer numbers of adducts as well as proportionally less fluorescence. When cells were exposed to non-toxic doses of the activated carcinogen for 1 h, nuclear fluorescence was detectable in immediately-fixed cells but faded to non-detectable levels when cells were washed and cultured for an additional 24-48 h before fixation.
Animals↗