bromochloroacetic-acid and Wilms-Tumor

Topics: Adenoma, Oxyphilic; Carcinoma, Renal Cell; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Proto-Oncogene Proteins c-kit; Sensitivity and Specificity; Wilms Tumor; WT1 Proteins

Nephrogenic adenofibroma is a novel kidney tumor of young people (mean age of presentation, 13 years), who present with polycythemia, hypertension, or hematuria, which resolve following nephrectomy. The typical nephrectomy specimen contains a solitary, nonencapsulated, vaguely circumscribed, irregularly shaped or spherical, firm mass with either tan, gray-white, or pale yellow coloration. Cysts are sometimes present within the tumor. The histologic appearance is distinctive and characterized by a marked proliferation of spindled mesenchymal cells resembling the classical type of congenital mesoblastic nephroma, encasing discrete nodules of embryonal epithelium similar to the hyperplastic nephrogenic rests (nephroblastomatosis) usually associated with Wilms' tumor. The mesenchymal component consists of a fascicular proliferation of tightly interlaced, uniform, benign-appearing spindled cells that immunostatin for vimentin and fibronectin, but not desmin or actin. The epithelial component consists of discrete islands of blastemal cells that are partially or fully differentiated toward tubular, tubulopapillary, or papillary structures. Psammoma bodies are plentiful. Embryonal epithelium immunostains for cytokeratin but not epithelial membrane antigen. The overall histologic appearance of the mesenchymal and epithelial components is benign, and preliminary clinical data suggest that the tumor has a benevolent course. Two cases, however, contained small, well-circumscribed papillary lesions near the renal pelvis that resembled low-grade collecting duct carcinoma. The clinical implications of the latter finding are unclear.

Topics: Adenofibroma; Adolescent; Adult; Biopsy; Child; Child, Preschool; Diagnosis, Differential; Female; Fibronectins; Humans; Immunohistochemistry; Keratins; Kidney; Kidney Neoplasms; Male; Vimentin; Wilms Tumor

Desmoplastic small round cell tumor (DSRCT) and blastemal-predominant Wilms tumor (WT) share overlapping histologic features, yet accurate distinction is critical because of differing prognosis and treatment. We encountered a neck mass in a young adult with a concomitant abdominal mass. The neck mass was initially concerning for DSRCT on the basis of the poorly differentiated morphology, desmoplastic stroma, and immunoreactivity for desmin and cytokeratin. However, focal triphasic elements and glomeruloid bodies were identified on deeper sections, WT1 immunoreactivity was seen with antibodies to both the amino-terminus and carboxy-terminus, and EWSR1-WT1 rearrangement studies were negative, supporting the ultimate diagnosis of WT. On the basis of the overlapping histologic features of DSRCT and blastemal-predominant WT, we undertook a comparison study of desmin and cytokeratin reactivity patterns. We found that, whereas desmin reactivity was more frequent in DSRCT (11 of 12) than in WT blastema (11 of 22, P=0.024), dot-like perinuclear reactivity was seen with equal frequency in desmin-reactive DSRCT (10 of 11) and WT blastema (10 of 11), illustrating an important diagnostic pitfall. Moreover, we demonstrate coexpression of desmin and cytokeratin in both DSRCT (9 of 12) and WT blastema (11 of 22). Importantly, although dot-like desmin reactivity and coexpression of desmin and cytokeratin are historically associated with DSRCT, we demonstrate that these features can be seen in either DSRCT or blastemal-predominant WT. In these challenging cases, detection of an EWSR1-WT1 rearrangement and selective WT1 carboxy-terminus immunoreactivity (characteristic of DSRCT) or dual immunoreactivity for the WT1 amino-terminus and carboxy-terminus (characteristic of WT) remain the most discriminating diagnostic tools.

Topics: Biomarkers, Tumor; Biopsy; Desmin; Desmoplastic Small Round Cell Tumor; Diagnosis, Differential; Diagnostic Errors; Gene Rearrangement; Genetic Predisposition to Disease; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Oncogene Proteins, Fusion; Phenotype; Predictive Value of Tests; Prognosis; Wilms Tumor; WT1 Proteins

To study the clinicopathologic features and histologic differential diagnosis of small cell neuroendocrine carcinoma (SmCC) of kidney.. The clinicopathologic features of 12 cases of SmCC of kidney encountered during the period from 1999 to 2010 were retrospectively reviewed.. Six cases of primary and 6 cases of metastatic SmCC involving kidney were identified. Amongst the primary renal SmCC, 2 were located in renal parenchyma and 4 in renal pelvis. Chest X-ray showed negative findings. Five of them underwent radical nephrectomy. On gross examination, the tumor was located centrally around the renal pelvis in 4 cases and peripherally in renal parenchyma in 1 case. On the other hand, 4 of the 6 cases of metastatic SmCC were discovered during therapy for pulmonary SmCC. Two of these patients presented with abdominal pain and gross hematuria, with lung and renal tumor masses identified simultaneously. The diagnosis of all the 6 cases of metastatic SmCC was confirmed by fine needle aspiration biopsy. Microscopically, pure SmCC was demonstrated in the 2 cases of primary renal parenchymal SmCC and 6 cases of metastatic SmCC. The 4 primary renal pelvic SmCC coexisted with urothelial carcinoma component. On immunohistochemical study, all cases were positive for cytokeratin, synaptophysin and CD56. All metastatic cases and 4 primary cases were also positive for TTF-1. Of six patients with primary SmCC two died 4 and 9 months after operation, and two were alive with a follow-up of 25 and 138 months, respectively. Five of six cases with metastatic SmCC died 3 - 8 months after diagnosis. The other 3 cases were failed to follow-up.. Both primary and metastatic SmCC can be found in the kidney. Although rare, primary SmCC is located either in renal parenchyma or in pelvis. The diagnosis of SmCC relies on morphologic examination and immunohistochemical study. TTF-1 immunostaining cannot reliably distinguish primary from metastatic SmCC in kidney. Correlation with clinicoradiologic findings and demonstration of coexisting urothelial carcinoma component (if any) is helpful in delineation of the tumor origin.

Topics: Adult; Aged; Carcinoma, Neuroendocrine; Carcinoma, Renal Cell; Carcinoma, Small Cell; CD56 Antigen; Diagnosis, Differential; Female; Follow-Up Studies; Humans; Keratins; Kidney Neoplasms; Lung Neoplasms; Lymphoma; Male; Middle Aged; Nephrectomy; Nuclear Proteins; Retrospective Studies; Sarcoma, Ewing; Synaptophysin; Thyroid Nuclear Factor 1; Transcription Factors; Treatment Outcome; Wilms Tumor

To analyse the cytomorphologic spectrum of Wilms tumour (WT) on aspirates, the largest series reported to date.. Adequate aspirates from paediatric renal tumours over a period of 17 years were reviewed and selected if subsequent excision showed WT or aspirates were diagnostic for WT and clinical/radiological evidence consistent with that diagnosis. Smears were re-examined for the proportion of components, degree of pleomorphism and mitosis.. Of 110 aspirates, smears were triphasic in 44 (40.0%), biphasic (blastema and tubules) in 36 (32.7%) and monophasic (blastema alone) in 30 (27.3%). Stromal predominance was seen in 11 aspirates (10.0%) and five showed rhabdomyoblastic differentiation; all 11 were triphasic. Mean mitotic rate was 9.3/5000 cells (range 4-39/5000). Nuclear atypia not amounting to anaplasia and without atypical mitoses was seen in 15 (13.6%); these presented diagnostic problems. Two aspirates (1.8%) were considered anaplastic (unfavourable), both having atypical mitoses. Criteria similar to histology (i.e. 3-fold or more variation in nuclear size, marked hyperchromasia with bizarre nuclei and atypical mitoses in a biphasic or triphasic aspirate) helped in distinguishing anaplastic WT. Histopathological correlation in 67 cases showed good correlation of blastemal predominance, stromal predominance and anaplastic histology with the corresponding cytology. However, 9/27 (33.3%) triphasic tumours had only blastemal cells on corresponding aspiration because of sampling error. Cytokeratin was positive in 4 of 20 aspirates with blastema alone.. Aspirates from WT were triphasic or biphasic in the majority (72.7%), permitting cytological diagnosis, which was improved by cytokeratin immunocytochemistry. Blastemal and stromal predominance on histology correlated well with cytology, but many triphasic tumours showed only blastema on aspiration. Anaplastic WT can be detected on aspirates using criteria similar to histology.

Topics: 12E7 Antigen; Adolescent; Anaplasia; Antigens, CD; Biopsy, Fine-Needle; Carcinoma, Renal Cell; Cell Adhesion Molecules; Cell Differentiation; Child; Child, Preschool; Chromosomal Proteins, Non-Histone; Diagnosis, Differential; DNA-Binding Proteins; Female; Follow-Up Studies; Humans; Infant; Keratins; Kidney; Kidney Neoplasms; Male; Peptide Fragments; Rhabdoid Tumor; SMARCB1 Protein; Staining and Labeling; Synaptophysin; Transcription Factors; Wilms Tumor; WT1 Proteins

Metanephric adenoma (MA), a well-described renal neoplasm, usually behaves in a benign fashion. It may have areas that are morphologically similar to papillary renal cell carcinoma (RCC) type, or epithelial (tubular predominant) type Wilms' tumor. Prior immunohistochemical studies of MA have reported variable staining patterns. Alpha-methylacyl-CoA racemase (AMACR), a molecular marker for prostate carcinoma, has subsequently been found to be overexpressed in breast, colorectal and ovarian cancers, among others. Recent microarray analysis of renal tumors has shown an increase of AMACR mRNA levels in papillary RCC but not in other subtypes. We investigated the utility of immunohistochemical staining for AMACR, cytokeratin 7(CK7), CD57 and WT1 to differentiate between the above-mentioned three neoplasms. Immunohistochemical stains were performed on paraffin-embedded tissue sections from 25 papillary RCC, 10 MAs and eight Wilms' tumors. AMACR was positive in one (10%) of 10 MAs and 24 (96%) of 25 papillary RCC, while it was negative in all Wilms' tumors. CK7 was positive in 20 of 25 papillary RCCs, focally positive in one Wilms' tumor and was negative in all MAs. CD57 was positive in all six MAs that were stained, focally positive in one of 25 papillary RCC and one of eight Wilms' tumors. WT1 was positive in seven of 10 MAs, three of 25 papillary RCCs and all eight Wilms' tumors. In conclusion, diffuse and strong immunoreactivity for AMACR may be useful in differentiating papillary RCC from MA but a panel which includes AMACR, CK7 and CD57 is better in this differential diagnosis. AMACR is not helpful in differentiating MA from Wilms' tumor, but CD57 is helpful in this differential diagnosis. WT1 may be useful in separating Wilms' tumor from MA and papillary RCC but is not helpful in differentiating MA from papillary RCC.

Topics: Adenoma; Biomarkers, Tumor; Carcinoma, Papillary; Carcinoma, Renal Cell; CD57 Antigens; Diagnosis, Differential; Humans; Immunohistochemistry; Keratin-7; Keratins; Kidney Neoplasms; Racemases and Epimerases; Wilms Tumor; WT1 Proteins

Topics: Adult; Chromosomes, Human, Pair 18; Chromosomes, Human, X; Humans; Immunohistochemistry; Karyotyping; Keratins; Lung; Lung Neoplasms; Male; Neoplasms, Radiation-Induced; Radiotherapy; Sarcoma, Synovial; Translocation, Genetic; Wilms Tumor

The occurrence of an extrarenal Wilms tumor in the lumbosacral region is an extremely uncommon condition.. We report a case of Wilms tumor in the lumbosacral region that was associated with diastematomyelia and occult spina bifida. An 18-month-old girl presented with a swelling over the lower back with a tuft of hair on it, which she had had since birth. Imaging of the spine revealed spina bifida, bony diastematomyelia, and tethered cord. Excision of the bony spur and detethering of the cord was done. After a year, she had recurrence of swelling at the same site, weakness of both lower limbs, and incontinence of bladder and bowel. Excision of the mass and bony spur and detethering of the spinal cord were done. Histopathological examination showed features of a Wilms tumor.

Topics: Female; Humans; Immunohistochemistry; Infant; Keratins; Kidney Neoplasms; Lumbosacral Region; Magnetic Resonance Imaging; Spina Bifida Occulta; Spinal Cord Neoplasms; Spinal Dysraphism; Staining and Labeling; Wilms Tumor

Wilms' tumor is one of the most common solid tumors in children and is an interesting model for understanding the pathogenesis of embryonal tumors. Cytokeratins are intracellular fibrous proteins present in tissue of epithelial origin. The immunoexpression of the pan-cytokeratin AE1AE3 was studied in paraffin-embedded tissue sections from 24 Wilms' tumors (12 with nephrogenic rests) and also tissue samples from 15 corresponding normal kidneys, to evaluate epithelial differentiation in the genesis of Wilms' tumor. We observed that the intensity of the expression of AE1AE3 in the epithelial component of Wilms' tumors directly correlated with the degree of maturity of the epithelial structures correspondent to the collecting ducts.

Topics: Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Wilms Tumor

Several podocyte-related markers are organized to express in glomerular differentiation. However, whether expression of them is virtually synchronized and a reliable indicator of the state of differentiation is unknown. The present study investigated, by immunohistochemistry, the divergent expression of several podocyte markers in the improperly differentiated glomeruloid bodies from four cases of Wilms tumors. The glomeruloid bodies were classified into immature (IGB) or mature forms (MGB) based on morphology and epithelial features. Podocytes in IGB expressed WT1, synaptopodin, podocalyxin, and nephrin, and their expression was stronger in MGB. In contrast, Pax2 was strong in IGB and diminished in MGB. p27 was first expressed in MGB. The expression pattern in each molecule mimics normal glomerulogenesis. Podocytes in MGB showed persistent expression of bcl-2 and cytokeratin with synaptopodin, podocalyxin, and nephrin by serial section, a finding unusual for normal glomerulogenesis. Moreover, parietal cells in MGB also occasionally expressed these podocyte markers. The ultrastructure revealed that podocytes in MGB showed tight junctions without foot process formations, which indicated incomplete differentiation. These results suggest that a set of podocyte differentiation markers are occasionally diversely expressed, and raise the possibility that expression of these markers is insufficient to determine the state of terminal differentiation in podocytes.

Topics: Biomarkers, Tumor; Female; Humans; Infant; Keratins; Kidney Glomerulus; Kidney Neoplasms; Male; Membrane Proteins; Microfilament Proteins; Muscle Proteins; Proteins; Proto-Oncogene Proteins c-bcl-2; Sialoglycoproteins; Tight Junctions; Wilms Tumor; WT1 Proteins

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors, including blastema-predominant Wilms' tumor (WT). Approximately 90% of ES/PNET have a specific t(11;22), which results in a chimeric EWS-FLI-1 protein. Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. WT-1, the WT-associated tumor suppressor gene, is overexpressed in WT but not in ES/PNET. No study has examined FLI-1 or WT-1 expression in renal ES/PNET. The clinicopathologic features of 11 renal ES/PNET were studied along with immunohistochemistry for cytokeratin, desmin, CD99, FLI-1, and WT-1. WT were also immunostained for CD99 (5 cases), FLI-1 (10 cases), and WT-1 (9 cases). The patients (6 men, 5 women) ranged from 18 to 49 years of age (mean, 34 yr). The mean tumor size was 11.8 +/- 3.8 cm (mean +/- standard deviation). Presenting symptoms included abdominal/flank pain and/or hematuria. Grossly, all tumors showed necrosis and hemorrhage, and 4 had cystic change. Microscopically, all tumors showed vaguely lobular growth, primitive round cells, and variable rosette formation. Epithelial, myogenous, or cartilaginous differentiation was not seen. Immunohistochemical results on the renal ES/PNET were cytokeratin (2/8 focal), desmin (0/9), CD99 (8/8), FLI-1 (5/8), and WT-1 (0/8). In comparison, the WT only rarely expressed CD99 (1/5) and did not express FLI-1 (0/10), but were usually WT-1-positive (7/9). Follow-up on 8 cases (mean, 28 mo; range, 6-64 mo) showed 4 lung and pleural metastases, 1 bone metastasis, liver metastasis, 2 local recurrences, and 5 deaths from disease (median time to death, 16.8 mo). No case had distant metastatic disease at presentation. Adjuvant therapy included chemotherapy (8 cases), radiation (3 cases), and bone marrow transplantation (1 case). Our study affirms a unique proclivity of renal ES/PNET for young adults and that it is a highly aggressive neoplasm, with rapid death in many cases, usually after the development of treatment-resistant lung metastases. These tumors must be distinguished from blastema-predominant WT and other primitive renal tumors that require different therapy. FLI-1 and WT-1 immunohistochemistry may be valuable in this differential diagnosis, given the known immunophenotypic overlap between ES/PNET and blastema-predominant WT with regard to CD99, cytokeratin, and

Topics: 12E7 Antigen; Adult; Aged; Antigens, CD; Cell Adhesion Molecules; Child; Combined Modality Therapy; Desmin; Diagnosis, Differential; DNA-Binding Proteins; Female; Genes, Tumor Suppressor; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Male; Middle Aged; Neoplasm Metastasis; Neuroectodermal Tumors, Primitive; Proto-Oncogene Protein c-fli-1; Proto-Oncogene Proteins; Sarcoma, Ewing; Trans-Activators; Wilms Tumor; WT1 Proteins

So far, there is no approved tumour marker for diagnosis or follow-up in Wilms tumour (WT). Tissue polypeptide-specific antigen (TPS), a cytokeratin 18 proteolytic fragment, has been suggested to be of value in the clinical management of WT patients. Cytokeratin 18 fragments are an early indicator of apoptosis and cytokeratin 18 might influence tumour cell behaviour. We investigated TPS expression in specimens of WT and other paediatric renal malignancies. Immunoreactivity of WT sections (n = 9), clear cell sarcomas (CCSK, n = 3), and a renal cell carcinoma (RCC), and two pediatric kidney tumour cell lines (WT: SK-NEP-1 and rhabdoid tumour of the kidney: G-401) were investigated using the monoclonal antibody M3. Additionally, immunoblotting and RT-PCR analysis were performed. Cell culture supernatants were evaluated for TPS release. Serum TPS was measured in five patients at diagnosis, during chemotherapy and after surgical resection.. Moderate to strong immunoreactivity for TPS was found in tubular and blastemal components of nearly all (8/9) WT specimens. This was confirmed by Western-blotting. Cystic and epithelial-like portions of CCSKs and RCC showed distinct reactivity (3/3). The supernatant of G-401 but not of SK-NEP-1 showed a time- and cell number-dependent increase of TPS release. Interestingly, TPS synthesis was demonstrated in SK-NEP-1 cells. Median preoperative serum TPS was elevated (293 U/l) compared to healthy children and lowest after surgical resection (49.5 U/l).. This is the first study demonstrating the synthesis and release of TPS by WTs and other paediatric renal malignancies. Considering the elevated levels of TPS in serum of these patients, a further investigation of this marker by larger clinical trials seems to be justified.

Topics: Adolescent; Base Sequence; Biomarkers, Tumor; Biopsy, Needle; Blotting, Western; Cell Line; Child; Child, Preschool; Female; Fluorescent Antibody Technique; Humans; Immunohistochemistry; Infant; Infant, Newborn; Keratins; Kidney Neoplasms; Male; Molecular Sequence Data; Peptides; Prognosis; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger; Sensitivity and Specificity; Wilms Tumor

Metanephric adenoma is a recently described benign renal neoplasm with distinctive histologic features. The cytologic appearance and fluorescence in situ hybridization (FISH) studies of this tumor have not been described. We present a case from a 48-yr-old woman. Cytologically, the cells were arranged in tight, short papillae and loose sheets. The cells had scant cytoplasma, round monotonous nuclei with fine even chromatin and rare small nucleoli. Immunohistochemistry revealed no reactivity for epithelial membrane antigen (EMA), keratins (AE1/AE3, callus, 34BE12), or carcinoembryonic antigen (CEA). FISH showed a disomic pattern for chromosomes 7, 17, and for the chromosome 3 short arm. The differential diagnosis includes Wilms' tumor, renal adenoma, papillary renal cell carcinoma, and metastatic tumors. Both immunohistochemistry and FISH may be of help in distinguishing some of these lesions.

Topics: Adenoma; Carcinoma, Papillary; Diagnosis, Differential; Female; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Keratins; Kidney Neoplasms; Middle Aged; Mucin-1; Neoplasm Metastasis; Tomography, X-Ray Computed; Wilms Tumor

A candidate tumor suppressor gene, WT-1, is believed to have an important role in the pathogenesis of Wilms' tumor, especially that occurring in patients with congenital aniridia.. To obtain a stable tumor line to work with, Wilms' tumor tissue was serially transplanted in athymic nude mice. Biopsied Wilms' tumor tissue, derived from an aniridia patient, was transplanted subcutaneously to an athymic nude mouse, and then transplanted serially. Histopathologic and molecular biologic studies were performed on the xenotransplants.. The aniridia patient showed partial deletion in one short arm of chromosome 11, which bears the WT-1 gene. The tumor was successfully transplanted in the nude mouse. Although the tumor contained blastemic, organoid, and stromal histologic elements, the organoid element began to decrease after more than 20 passages. Cytogenetic analysis revealed an additional abbreviation of one long arm of chromosome 6. Dot blot analysis showed that the copy number of WT-1 gene was decreased to half the amount in the tumor, in spite of the WT-1 transcript with normal size detected by Northern blotting.. The tumor is expected to bear one WT-1 gene with minute abnormalities as well as one congenitally deleted gene. This tumor line is useful when examining the effect caused by introduction of WT-1 gene to Wilms' tumor in vivo.

Topics: Alleles; Animals; Aniridia; Biopsy; Chromosome Deletion; Chromosome Mapping; Chromosomes, Human, Pair 11; Genes, Wilms Tumor; Humans; Immunohistochemistry; Infant; Keratins; Kidney Neoplasms; Male; Mice; Mice, Nude; Microscopy, Electron; Mucin-1; Mucoproteins; Neoplasm Proteins; S100 Proteins; Transplantation, Heterologous; Uromodulin; Vimentin; Wilms Tumor

A new cell line, designated NB-YK, was established from a transplantable rat nephroblastoma (NB-Y) which was derived from a spontaneous nephroblastoma in an aged Fischer 344 rat. NB-YK grew in a piling-up and noncohesive pattern on the plastic surface and formed colonies in a soft agar. The main cell type of NB-YK represented morphology of mesenchymal phenotype and most of the cells contained several secretory granules in their cytoplasm. Immunocytochemically the cells were positive for vimentin, cytokeratin, and laminin. Coexpression of vimentin and cytokeratin in the cells was confirmed by the one-dimensional gel electrophoresis and immunoblotting for intermediate filament proteins. NB-YK cells were tumorigenic and produced fibrosarcoma-like tumor when inoculated subcutaneously or intraperitoneally into syngeneic rats and nude mice. NB-YK seems to be a useful model for studying biological properties of nephroblastoma.

Topics: Animals; Blotting, Western; Cell Line; Electrophoresis, Polyacrylamide Gel; Gene Expression; Immunoenzyme Techniques; Immunohistochemistry; Keratins; Kidney Neoplasms; Laminin; Mice; Mice, Nude; Microscopy, Electron; Neoplasm Transplantation; Rats; Ribosomes; Tumor Cells, Cultured; Vimentin; Wilms Tumor

Wilms tumors (WTs) are embryonic neoplasms of the kidney that are believed to arise from primitive metanephrogenic blastema. Our previous reports and those of others indicate that WTs show an increased expression of insulin-like growth factor II (IGF-II) mRNA. However, the precise role of IGF-II on Wilms tumorigenesis is not known. A central question is to determine whether the increased IGF-II expression in WTs simply reflects the fetal nature of WTs (effect), or is induced by specific changes in gene expression (cause).. This study included 31 sporadic WTs, 7 fetal and 3 adult kidneys and 1 yolk sac tumor. Clinical and histologic summaries of WT cases are shown in Table 1. In WTs, the relative area of blastemal, epithelial, poorly differentiated spindle cell and heterologous cell components were assessed. Dot and Northern blot hybridization, using cDNA probes, were done to assess the level of IGF-II mRNA expression. In situ RNA hybridization was employed to localize IGF-II transcripts. Immunohistochemistry was applied to frozen sections to demonstrate cytokeratin and type-IV collagen. These results were then correlated with the histology of WTs and their precursor lesions, i.e., nephrogenic rests (NRs).. Dot blot hybridization indicated that IGF-II transcripts were 32- to 64-fold more abundant in WTs than in the adjacent uninvolved kidneys. In situ hybridization showed that WTs, NRs, and fetal kidney shared a common feature in which IGF-II transcripts were predominantly associated with blastema. However, WTs and NRs differed from fetal kidney in that occasional epithelial structures and dense blastema showed aberrant, sustained IGF-II expression.. The data indicate two points. 1) There is an inverse correlation between nephroblastic differentiation and IGF-II expression in developing fetal kidney. 2) The IGF-II expression in WTs and NRs does not simply reflect the embryonal nature of the tumor but is rather significantly altered, suggesting a role as a transforming growth factor in Wilms tumorigenesis.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Blotting, Northern; Cell Transformation, Neoplastic; Child; Collagen; Endodermal Sinus Tumor; Epithelium; Female; Gene Expression Regulation, Neoplastic; Humans; Immunohistochemistry; In Situ Hybridization; Insulin-Like Growth Factor II; Keratins; Kidney; Kidney Neoplasms; Male; Middle Aged; Precancerous Conditions; RNA, Messenger; Statistics as Topic; Transcription, Genetic; Transforming Growth Factors; Wilms Tumor

A 19-month-old black girl had a radical nephrectomy for a Wilms' tumor that contained areas of epithelium indistinguishable from renal cell carcinoma. She was treated with chemotherapy but subsequently had pulmonary metastases develop and massive abdominal recurrence. The recurrent tumor was histologically renal cell carcinoma with no identifiable Wilms' tumor elements. The child died with recurrent and metastatic tumor 13 months after nephrectomy. Pathologic, immunoperoxidase, and flow cytometric studies of this unusual case are presented.

Topics: Carcinoma, Renal Cell; Female; Humans; Immunoenzyme Techniques; Infant; Keratins; Kidney Neoplasms; Lung Neoplasms; Ploidies; Recurrence; Vimentin; Wilms Tumor

Ten renal cell carcinomas in children under 15 years were investigated. The average age was 122.5 months and the girls predominated in our cases (7 girls, 3 boys). By using the classification of Thoenes et al., Pathol Res Pract 181: 125-143, 1986 a predominance of clear cell-eosinophilic tumor cell type and of the tubulopapillary growth pattern was found. Immunohistochemistry revealed a heterogeneity of cytokeratin expression. By using the monoclonal antibodies Cam 5.2 and KL 1, cytokeratins were found in 7 cases each. The other 4 cytokeratin antibodies used were less sensitive. The expression of cytokeratin 13 in 3 cases suggested a more complex histogenesis than assumed. Vimentin was found in 3 tumors, but an association to a higher grade (G) of malignancy was not found in these cases. One tumor expressed the Tamm-Horsfall-protein, which is predominantly found in the distal tubule of the normal kidney. In summary the results of immunohistochemistry characterized the great heterogeneity of these tumors. Follow-up information was available in 9 cases. All patients with G I- and G II-tumors were free of disease after an average time of 39.6 months (mean 27 months). Two of the 3 cases with G III-tumors died after 9 and 15 months, despite additional chemo- or radiotherapy. Therefore tumors of grade I and II of the Thoenes classification seem to have a good prognosis.

Topics: Adenocarcinoma; Adolescent; Antibodies; Carcinoma, Renal Cell; Child; Child, Preschool; Eosine Yellowish-(YS); Female; Follow-Up Studies; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Lectins; Male; Prognosis; Staining and Labeling; Wilms Tumor

Adult nephroblastoma is a rare tumor. The authors report a new case observed in a 35 year old woman in whom the diagnosis was made by histopathology. The authors study the particular features of this tumor. Its prognosis appeared to be poorer than that of renal adenocarcinoma.

Topics: Adult; Cytoplasm; Female; Humans; Keratins; Kidney Neoplasms; Wilms Tumor

Wilms' tumour (nephroblastoma), a childhood embryonal kidney tumour, is believed to arise from malignant transformation of abnormally persistent metanephric blastemal cells. At a histological level, tumours show a remarkable mimicry of the normal nephrogenic pathway. There is histological and epidemiological evidence for at least two pathogenetic groupings within Wilms' tumour which may reflect different timings of the tumorigenic insult in this pathway and/or involvement of different genes. Tumorigenesis is thought to result from loss of function of a so-called tumour-suppressor gene which has an essential role in control of normal genitourinary development. Such a candidate, Wilms' tumour gene (WT1) mapping to chromosome 11p13, has been isolated and is known to be mutated in some tumours. We have examined the cell types expressing this gene in 32 Wilms' tumours and in nephroblastomatosis by in situ mRNA hybridization. Our results show that WT1 is expressed only in neoplastic structures whose normal counterparts also express the gene and that abnormally persistent high levels of expression are common in both these lesions. Thus, WT1 expression is a good marker for tumour differentiation and reveals how the normal pattern of differentiation is disrupted in Wilms' tumours. We postulate that mutation of the WT1 gene at the 11p13 locus results in Wilms' tumours associated with intralobar nephrogenic rests, which frequently show stromal-predominant histology. We have used our results and ideas to reinterpret current theories on tumour histogenesis and propose a model which explains how patterns of epithelial differentiation are disrupted in Wilms' tumour and how malignant stroma can result from mutation in WT1.

Topics: Antibodies, Monoclonal; Cell Differentiation; Chromosomes, Human, Pair 11; DNA Probes; Genes, Tumor Suppressor; Humans; Immunohistochemistry; Keratins; Kidney; Kidney Neoplasms; Laminin; Models, Biological; RNA, Messenger; Wilms Tumor

Ultrasound-guided percutaneous needle biopsy proved to be a reliable and safe method to obtain material for histopathological and immunohistochemical diagnosis prior to treatment in childhood malignancies. A principal tumour identification could be obtained by a combined morphological and phenotypic examination of 38 small-sized tumour biopsy specimens using a fairly limited panel of immunological reagents, including antibodies to leucocyte common antigen (CD 45), certain B- and T-cell markers, various intermediate filaments (cytokeratin, desmin and vimentin), and neuroblastoma cells (UJ 167.11, A2B5, and UJ 13A; the latter recognizes NCAM). Five undifferentiated neuroblastomas were all positive with the neuroblastoma antibodies but negative for the other markers, including vimentin. The negative reactivity for desmin and vimentin was the major immunohistochemical distinction between neuroblastomas and rhabdomyosarcomas. In addition, limited reactivity with the neuroblastoma antibodies was seen in blastematous parts of Wilms' tumour, duct-like structures in a hepatoblastoma, and in tumour cells in a few undifferentiated myelo- and lympho-proliferative lesions. This study shows the importance of a combined evaluation of morphology and the pattern of immunoreactivity employing multiple markers.

Topics: Antibodies, Monoclonal; Antigens, CD; Biomarkers, Tumor; Biopsy, Needle; Child; Child, Preschool; Desmin; Diagnosis, Differential; Fluorescent Antibody Technique; Histocompatibility Antigens; Humans; Immunohistochemistry; Keratins; Leukocyte Common Antigens; Lymphoproliferative Disorders; Neuroblastoma; Rhabdomyosarcoma; Vimentin; Wilms Tumor

Using an indirect immunoperoxidase technique, we tested frozen specimens from 12 Wilms' tumors with monoclonal antibodies (MoAbs) reacting against a large panel of molecules including laminin, fibronectin, cytokeratin, vimentin, villin, CD24, CALLA/CD10, CR1, CD26, class I and class II major histocompatibility complex (MHC) molecules, and endothelium factor VIII. These molecules were chosen because they are markers of specific segments of the mature kidney and because their loss or acquisition is indicative of different steps of human nephrogenesis. KI67 MoAb was used to evaluate the proliferating activity of the cells. The blastemal component (cell compact areas) of Wilms' tumors consisted of vimentin-positive cells with a fibronectin network. However, signs of epithelial maturation were present in compact areas where cytokeratin-positive cells producing laminin were observed. The cells exhibited a high degree of proliferating activity. The tubule formations consisted of cytokeratin-positive cells and had a defined laminin border. All the cells, whether in compact areas or in tubules, were strongly CD24-positive. Some tubular formations showed signs of proximal maturation with the presence of CALLA, CD26, and even villin. In four cases class I-MHC molecules were expressed by some tubular cells. Large cystic cavities present in five cases were edged by cytokeratin, CD24-positive cells, or by vimentin, CALLA, CR1-positive cells. Some glomeruloid bodies, present in two cases, were also composed of vimentin, CALLA, and CR1-positive cells which correspond to the mature podocyte phenotype. The interstitial tissue contained mainly laminin and fibronectin network with macrophages and few CD3 lymphocytes. The presence of large cells with muscular differentiation was noted; round vimentin and CD26-positive cells were also seen. The endothelial cells of the vessels exhibited vimentin, factor VIII, and class I and class II MHC molecules as do mature cells, but in some cases the endothelial cells lacked class II molecule expression and were CALLA-positive. These results which confirmed and extended those previously described show that cell differentiation in Wilms' tumor mimics that observed during metanephros development. Moreover, this study shows that tumoral cells in nephroblastoma share several antigens with cells from lymphoid lineage (CD24, CALLA, and CD26) as do developing and mature kidney cells. Such cell phenotype dissection provides a useful and reliable

Topics: Antigens, CD; Antigens, Differentiation; Antigens, Differentiation, T-Lymphocyte; Antigens, Neoplasm; CD24 Antigen; Cell Differentiation; Cell Nucleus; Cytoplasm; Dipeptidyl Peptidase 4; Factor VIII; Fibronectins; Histocompatibility Antigens Class I; Histocompatibility Antigens Class II; Humans; Immunoenzyme Techniques; Immunohistochemistry; Keratins; Kidney Neoplasms; Membrane Glycoproteins; Neprilysin; Receptors, Complement; Receptors, Complement 3b; Vimentin; Wilms Tumor

The case of a 27-year-old female patient presenting with a Wilms' tumour is reported. The tumour was dominantly of blastemal type showing minor epithelial (tubular) areas and anaplastic portions. Thorough immunohistochemical examinations were performed to solve differential diagnostic problems. Keratin positivity was found to be of differential diagnostic value in the distinction of this tumour from the so called small round cell tumours. Prognostic aspects of Wilms' tumour are also discussed.

Topics: Adolescent; Female; Humans; Immunohistochemistry; Keratins; Kidney; Kidney Neoplasms; Wilms Tumor

Sixteen cases of malignant rhabdoid tumor (MRT) were studied by conventional light microscopy, immunohistochemistry, electron microscopy and flow cytometry. The age of the 16 patients varied from two months to 25.9 years. There were 11 males and five females. Eleven tumors were located in the kidney. The remaining five were found in the chest wall (n = 2) and the head and neck (n = 3). Particular histopathological findings included myxoid, pseudoalveolar and hyalinized areas. By immunohistochemistry, 15/15 cases stained positively for vimentin, 9/14 for cytokeratin, 6/15 for desmin, 9/14 for epithelial membrane antigen (EMA), 10/14 for neuron specific enolase (NSE) and 10/15 for protein S-100. Stains for neurofilaments, myoglobin and Ulex europaeus aggl. I (UEA I) were negative. The characteristic finding by electron microscopy in three cases were large numbers of intermediate filaments arranged either randomly or in concentric whorls. None of the 11 cases studied revealed aneuploid DNA stem lines as determined by flow cytometry. Of the 16 patients 12 died, one is living with disease and three are living without evidence of disease. Postoperative treatment consisted of chemotherapy, in some cases combined with radiotherapy. Two patients developed a medulloblastoma in addition to a renal and extrarenal MRT, respectively. Our findings demonstrate that MRT may present more histopathological patterns than hitherto recognized. In addition, they show that MRT may express a wide range of antigenic "markers", similar to epithelioid sarcoma with which it may be confused on cytological grounds. Despite aggressive postoperative chemotherapy prognosis is still poor.

Topics: Adolescent; Adult; Age Factors; Aneuploidy; Cell Nucleus; Child; Child, Preschool; Desmin; DNA; Female; Flow Cytometry; Head and Neck Neoplasms; Humans; Immunohistochemistry; Infant; Keratins; Kidney Neoplasms; Male; Microscopy, Electron; Phosphopyruvate Hydratase; Rhabdomyosarcoma; S100 Proteins; Sex Factors; Thoracic Neoplasms; Vimentin; Wilms Tumor

A nephroblastoma (Wilms' tumor) with morphological, histochemical, immunohistochemical and ultrastructural evidence of neuroendocrine differentiation is described. Whereas areas of neural differentiation and occasional argyrophilic cells in cases of Wilms' tumor have been previously reported, the unique characteristic in this case was the extent of the neuroendocrine differentiation, as shown by a strong Grimelius stain of over 90% of the blastematous cells. Immunoperoxidase studies employing antibodies to neuron-specific enolase and ultrastructural data were also in favor of the neuroendocrine differentiation and suggested the existence, in addition to the already reported variant of Wilms' tumor with neural differentiation, of a neuroendocrine variant which may be part of the histologic spectrum of this neoplasm.

Topics: Cell Differentiation; Child, Preschool; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Male; Microscopy, Electron; Wilms Tumor

The biological nature of human sarcomatous Wilms' tumor (SWT) was studied by analyzing newly established SWT lines, both heterotransplantable in nude mice and cultured in vitro. Five lines in nude mice include two from clear cell sarcoma of the kidney (CCSK), two from malignant rhabdoid tumor of the kidney (MRTK), and one from unclassified sarcoma. Five in vitro lines include three from CCSK, one from MRTK, and one from unclassified sarcoma. All of these in vitro cell lines produced tumors when innoculated in nude mice. Most of lines, especially of MRTK and unclassified sarcoma, well maintained their original morphological characteristics. However, CCSK lines, both heterotransplantable and in vitro, often showed unique morphological changes such as the increase of cells with eosinophilic cytoplasms and the production of mucin. Ultrastructurally, clusters of intermediate filaments, twisted sheaves of filaments resembling tonofilaments, intermediate junctions, and intracellular canaliculi were found in these cells. These findings suggested that CCSK had the latent epithelial nature which became obvious in the cell lines. This was confirmed by immunohistochemical and immunoblotting analyses with anticytokeratin antibodies. The result proved that CCSK expressed cytokeratin 8 (Mr 52,000) and 19 (Mr 40,000) as well as nephroblastic Wilms' tumor and strongly indicated that there was a close relationship between CCSK and nephroblastic Wilms' tumor.

Topics: Animals; Blotting, Western; Cell Differentiation; Child, Preschool; Epithelium; Female; Humans; Infant; Intermediate Filaments; Keratins; Kidney Neoplasms; Male; Mice; Mice, Nude; Sarcoma; Tumor Cells, Cultured; Vimentin; Wilms Tumor

The Wilms' tumour is a solid childhood tumour of the kidney, consisting of blastema, tubules and mesenchyme. Embryonic tumours, such as Wilms', may arise as a result of a developmental disturbance in differentiation. The expression of class I and II major histocompatibility complex (MHC) antigens was investigated on 6 Wilms' tumours and related to that in the developing human kidney in this immunohistological study, using a panel of monoclonal antibodies. The Wilms' tumour blastemal cells were class I MHC antigen negative, but differentiated structures were positive. Class II MHC antigens were not observed in Wilms' tumours. In the developing human kidney class I MHC antigen expression was observed on glomeruli from 8 weeks and on tubules from 13 weeks gestational age. Class II MHC antigen expression was observed on glomeruli from 11 weeks and on tubules from 13 weeks gestation. These results suggest that the blastemal cells within the Wilms' tumour may reflect an early stage of development with respect to the expression of MHC antigens.

Topics: Adult; Child; Fetus; Gestational Age; Histocompatibility Antigens Class I; Histocompatibility Antigens Class II; Humans; Keratins; Kidney; Kidney Glomerulus; Kidney Neoplasms; Kidney Tubules; Vimentin; Wilms Tumor

Topics: Aged; Carcinoembryonic Antigen; Combined Modality Therapy; Female; Humans; Keratins; Kidney Neoplasms; Neoplastic Cells, Circulating; Wilms Tumor

Two cases of malignant rhabdoid tumor of the kidney (MRT) and two cases of bone metastasizing renal tumor (BMRT) were studied using electron microscopy and immunohistochemistry, in an attempt to evaluate the histogenetic relation to classical Wilms' tumor (2 cases). The two cases of MRT showed ultrastructural features of intermediate filament clusters in the cytoplasm. Vimentin, keratin, laminin, and peanut (PNA) lectin were immunohistochemically demonstrated in two cases of MRT. Tissue polypeptide antigen (TPA) was detected in one case. As for BMRT, the metastatic lesion exhibited numerous rosette-like structures of tumor cells. Vimentin was immunohistochemically demonstrated in one case, but no other antibodies were stained in two cases. Two cases of classical Wilms' tumor immunohistochemically demonstrated vimentin, keratin, PNA lectin, and TPA. MRT and BMRT fall within the broad spectrum of Wilms' tumors from the histogenetic aspect, but their clinical behavior is distinct.

Topics: Bone Neoplasms; Child; Creatine Kinase; Humans; Immunoenzyme Techniques; Isoenzymes; Keratins; Kidney; Kidney Neoplasms; Laminin; Lectins; Microscopy, Electron; Myoglobin; Peanut Agglutinin; Rhabdomyosarcoma; Sarcoma; Vimentin; Wilms Tumor

The distribution of cytokeratin antigens during embryogenesis of the kidney and in 57 renal tumours has been studied using immunocytochemical techniques. A polyclonal antiserum to epidermal prekeratins and the monoclonal antibodies CAM 5.2 and PKK1 have been used to identify cytokeratins of different molecular weights. The ureteric bud-derived structures expressed large molecular weight cytokeratins. The tubular component of the kidney expressed cytokeratins detected by CAM 5.2 and PKK1. During glomerular development there was transient expression of low molecular weight cytokeratins by the visceral glomerular epithelium but in the adult kidney only the parietal epithelium expressed cytokeratins. Tubules in nephroblastomas contained low molecular weight cytokeratins but the blastema did not. Some ureteric bud-derived structures were identified in six nephroblastomas. Renal carcinomas expressed low molecular weight cytokeratins. Four collecting duct carcinomas were studied; these all expressed the large molecular weight cytokeratins found in collecting duct epithelium. These results indicate that the cytokeratin phenotype of renal tumours is unchanged from that of the normal epithelial cells.

Topics: Antibodies, Monoclonal; Carcinoma; Cytoskeletal Proteins; Fetus; Humans; Immunologic Techniques; Keratins; Kidney; Kidney Neoplasms; Wilms Tumor

A cell line (T3/73) from a Wilms' tumour has been established from a 9 month-old boy with aniridia. The tumour was removed in 1973. On histological examination a diagnosis of Wilms' tumour was made which showed undifferentiated areas, marked tubule formation and abundant striped muscle fibres. The tumour cells, which are fusiform, grew rapidly in culture without the addition of growth factors, and have undergone over 100 passages. Approximately 95% and 5% were positive for desmin and cytokeratin, respectively. The cell doubling time was 28 hr. Cytogenetic studies revealed a karyotype of 46,XY,del(11) (p12::p14). Although the cells stained very intensely with a monoclonal antibody that detects oncogene ras p 21 antigen, Southern blot analysis using c-Ha-ras as a probe failed to reveal an obvious deletion or amplification of either Ha-ras allele.

Topics: Antibodies, Monoclonal; Cell Line; Chromosome Deletion; Chromosomes, Human, Pair 11; Desmin; Gene Amplification; Humans; Infant; Karyotyping; Keratins; Male; Oncogenes; Proto-Oncogene Proteins; Proto-Oncogene Proteins p21(ras); Wilms Tumor

Wilms' tumor has been proposed to originate from a developmental abnormality of the metanephric blastema. This undifferentiated component of Wilms' tumors has previously eluded efforts for in vitro growth. Blastema from a "classical" Wilms' tumor was transplanted into nude mice and passaged through 12 generations of heterotransplantation. Tumors from heterotransplants were grown for 12 serial passages in a serum-free growth medium supplemented with hormones and conditioned media from human kidney proximal tubule cells. The blastema initially grew on a collagen-fetal calf serum matrix as multicellular spheroids, and the cells proliferating from the rim of the spheroids had a flattened shape. Pulse-labeling with bromodeoxyuridine (BrdU) identified the proliferating cell population as blastemal in origin. Except for a loss of extracellular matrix, ultrastructural studies demonstrated morphologic similarities in the cultured cells, compared with the primary tumor and heterotransplants. Lectin histochemical stains for the peanut lectin (PNA) and immunohistochemical stains for cytokeratin (CYTO), vimentin (VIM), and epithelial membrane antigen (EMA) were performed on the original tumor, successive heterotransplants, and cells grown in vitro. The PNA stained the surface of the blastemal cells after sialidase digestion in the original tumor, heterotransplants, and cultured cells. The blastema of the original tumors was negative for CYTO and EMA but reactive for vimentin. This lack of differentiation was maintained in heterotransplants through 12 passages. However, blastemal cells demonstrated coexpression of CYTO and VIM intermediate filaments when grown in a serum-free medium on a matrix material. These studies demonstrate that the blastemal component of Wilms' tumor can be successfully grown in culture, passaged in nude mouse heterotransplants, and shown to undergo early stages of blastemal differentiation in vitro by growth in serum-free medium. This in vitro system provides a model for testing the factors that influence the growth and differentiation of the blastemal component of Wilms' tumors.

Topics: Animals; Antigens, Surface; Cell Division; Cells, Cultured; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Male; Mice; Mice, Nude; Microscopy, Electron; Neoplasm Transplantation; Transplantation, Heterologous; Vimentin; Wilms Tumor

Three cases of clear cell sarcoma of the kidney (CCSK) and 5 cases of Wilms' tumor were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, and desmin) and myoglobin. In CCSK, tumor cells were negative for cytokeratin, except for occasional tubular structures, and vimentin was demonstrated in only one case. In Wilms' tumor, epithelial components were positive for cytokeratin and stromal cells were positive for vimentin, while no staining was found in blastemal cells for either. Both desmin and myoglobin were negative in all tumor cells except for skeletal muscle cells in Wilms' tumor. In the current study, some neoplastic cells in CCSK were revealed to be of mesenchymal nature, but blastemal cells in Wilms' tumor were not.

Topics: Antibodies, Monoclonal; Child; Child, Preschool; Desmin; Humans; Immunoenzyme Techniques; Infant; Keratins; Kidney Neoplasms; Male; Myoglobin; Sarcoma; Staining and Labeling; Vimentin; Wilms Tumor

The distribution of cytokeratins, desmosomal-plaque proteins (desmoplakins), and vimentin in nephroblastoma tissue was studied by immunofluorescence microscopy using specific antibodies. In undifferentiated blastema cells, desmosomes, as revealed by antibodies to desmoplakins, preceded the advent of significant amounts of cytokeratins, indicating that desmosomes are early and sensitive markers of epithelial differentiation. Cytokeratin-positive tumor cells were seen in the following distribution patterns: groups of loosely arranged and scattered cells containing only scant cytokeratin fibrils surrounded by negative stroma cells; focal accumulation of cytokeratin-positive cells with cytokeratin-specific cytoplasmic fibril meshwork staining; rosettes of cytokeratin-positive cells without formation of distinct lumina, showing concentration of cytokeratin staining in the center; tubules with distinct lumina made up of cytokeratin-positive cells, with cytokeratin staining concentrated in the subapical cell portions. In cytokeratin-positive cells, the numbers of desmoplakin-positive dots were generally increased; in well-formed tubules, enrichment of desmoplakin-positive spots, corresponding to the subapical skeletal disks, was most conspicuous. Vimentin was demonstrated in stromal areas, but also in blastema cells showing coexpression of desmosomes and vimentin filaments. Moreover, in certain blastema cells, an overlap of cytokeratin and vimentin immunostaining was observed. Epithelial cells of nephroblastoma tubules did not react with vimentin antibodies. Our results show that the appearance of desmosomal plaques, as demonstrated by antibodies to desmoplakins, may be a very early feature of epithelial differentiation, and they also emphasize the value of antibodies to desmoplakins in tumor cell typing and diagnosis.

Topics: Cell Differentiation; Child, Preschool; Cytoskeletal Proteins; Desmoplakins; Desmosomes; Electrophoresis, Polyacrylamide Gel; Epithelial Cells; Fluorescent Antibody Technique; Humans; Keratins; Kidney Neoplasms; Male; Membrane Proteins; Microscopy, Electron; Vimentin; Wilms Tumor

Nine surgically resected Wilms' tumors (WIT) and nude mouse heterotransplants from one WIT were studied by histochemistry and immunohistochemistry. Histochemistry showed acid phosphatase in all cells, while alkaline phosphatase and gamma-glutamyl transpeptidase were present in only some tubules. Using immunohistochemistry, antibodies to the intermediate filaments cytokeratin and vimentin distinguished tubular epithelium and mesenchyme, respectively. WIT tubules were also identified using antibody against a structural component (epithelial membrane antigen) and a secretory product (uromucoid) associated with distal convoluted tubules of normal kidney. Basement membrane surrounding the tubules of WIT was demonstrated using antibody to type IV collagen plus laminin. Different blastema subpopulations were negative or stained positively with antibodies to cytokeratin and vimentin. Production of basement membrane by blastema was also shown. Fetal antigen expression in WIT was examined using the monoclonal PI 153/3 and J5 antibodies. The blastema and tubules of WIT were strongly stained by PI 153/3, which did not label normal adult kidney, and weakly stained by J5, which strongly labeled glomeruli and proximal convoluted tubules of normal kidney. These studies show that WIT blastema is heterogeneous in intermediate filament subtypes, while WIT tubules more closely resemble distal than proximal convoluted tubules of adult kidneys but also retain expression of fetal antigens.

Topics: Acid Phosphatase; Alkaline Phosphatase; Animals; Histocytochemistry; Humans; Immunoenzyme Techniques; Keratins; Kidney Neoplasms; Membrane Proteins; Mice; Mice, Nude; Mucin-1; Neoplasm Transplantation; Rabbits; Transplantation, Heterologous; Vimentin; Wilms Tumor

Topics: Female; Humans; Infant; Keratins; Kidney; Kidney Neoplasms; Polycystic Kidney Diseases; Wilms Tumor