Child ; Humans ; Case-Control Studies ; East Asian People ; Genetic Predisposition to Disease/genetics* ; Methyltransferases/genetics* ; Polymorphism, Genetic ; Wilms Tumor/pathology*

Objective: To identify BRAF V600E mutations in adult Wilms tumor (WT) with overlapping histologic features of metanephric adenoma (MA) and to investigate the clinicopathological features of adult WT. Methods: The clinical features of adult WT diagnosed at the Fudan University Shanghai Cancer Center, Shanghai, China from 2012 to 2021 were reviewed. HE-stained slides of all cases were reviewed by 2 expert pathologists. Representative tissues were selected for BRAF V600E immunohistochemical (IHC) staining and gene sequencing. Results: In adult WT with MA-like areas (cohort Ⅰ, n=6), 5 of the 6 cases were composed of epithelial-predominant and were positive for WT-1 and CD56, respectively, and all were positive for CD57. All 6 cases revealed highly variable Ki-67 indices, ranging from 1% in some areas to 60% in others. 5 of the 6 cases harbored a BRAF V600E mutation. All cases in cohort I were followed up for 23 to 71 months, and all survived. In classical adult WT without MA-like areas cohort (cohort Ⅱ, n=13), all 7 cases with available material were negative for BRAF by IHC and none of them had any BRAF mutation. Conclusions: BRAF V600E mutations are frequently present in adult WT with overlapping morphologically features of MA, but not in those without. More importantly, adult WTs with overlapping histologic features of MA may be an intermediate entity between typical MA and WT that may have a favorable prognosis and possible therapeutic targets.
Adult ; Humans ; Proto-Oncogene Proteins B-raf/genetics* ; China ; Wilms Tumor/genetics* ; Kidney Neoplasms/pathology* ; Mutation ; Adenoma/genetics* ; Biomarkers, Tumor/genetics*

OBJECTIVETo evaluate the efficacy and safety of the WT-2009 chemotherapy protocol for Wilms' tumor (WT) in children.

METHODSThe clinical data of 34 children with newly-diagnosed WT between July 2009 and December 2013 were retrospectively analyzed. Among the 34 children, 2 died before treatment, 6 children did not accept therapy and 26 accepted the chemotherapy based on the WT-2009 chemotherapy protocol. Kaplan-Meier method was used to estimate the 2-year survival rate.

RESULTSThe pathological analysis revealed the favorable histology WT was common (88%, 30/34). The most common first manifestation was abdominal masses (56%, 19/34). Among the 26 patients who accepted the chemotherapy based on the WT-2009 protocol, complete remission was achieved in 24 cases (92%), partial remission was achieved in 1 case (4%), and disease relapse was found in 1 case (4%). Severe pulmonary infection occurred in 1 case in the course of treatment. The 2-year overall survival rate and event-free survival rate were 100% and 89.7% respectively.

CONCLUSIONSFavorable histology is most common pathological type in children with WT. The chemotherapy based on the WT-2009 protocol for WT can produce a favorable prognosis and a high tolerance.

Antineoplastic Agents ; adverse effects ; therapeutic use ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Kidney Neoplasms ; drug therapy ; mortality ; pathology ; Male ; Survival Rate ; Wilms Tumor ; drug therapy ; mortality ; pathology

Child ; Child, Preschool ; Female ; Humans ; Kidney Neoplasms ; pathology ; Logistic Models ; Male ; Prognosis ; Retrospective Studies ; Wilms Tumor ; pathology

OBJECTIVETo study the clinical and histopathologic features of metanephric adenoma (MA).

METHODSEight cases of recently diagnosed MA were retrieved from archival file. Immunohistochemical study was carried out. The clinical characteristics, pathologic parameters, differential diagnosis, treatment options and prognosis of MA were analyzed, with literature review.

RESULTSThe patients included 6 females and 2 males. The age of patients ranged from 12 to 70 years (mean=43.6 years). Eight cases were located in renal cortex and showed well-defined borders. Histologically, the tumor was composed of tubules lined by small basophilic cells and embedded in an edematous stroma. Papillary structures and psammoma bodies were focally seen. Immunohistochemical study showed that the tumor cells were positive for PAX2 and vimentin in all the 8 cases. WT-1 was positive in 2 cases, focal and weak in 5 cases, and negative in 1 case. CK-Pan was positive in 3 cases. CK7 staining was mostly negative, with focal and weak positivity only in 1 case. The proliferative index, as highlighted by Ki-67 staining, was less than 2% in 7 cases and focally around 5% in 1 case. The expressions of CK20, CD10, RCC, epithelial membrane antigen, CD56, synaptophysin and chromogranin A were negative. Follow-up information from 7 to 57 months was available in all patients; and none of them developed local recurrence or distant metastasis.

CONCLUSIONSThe diagnosis of MA relies primarily on thorough histologic examination and immunohistochemical study (vimentin and PAX2 positive, WT-1 focally and weakly positive in some cases, and low proliferative index). Correlation with clinical and radiologic findings would also be helpful.

Adenoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adolescent ; Adult ; Aged ; Biomarkers, Tumor ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Nephrectomy ; methods ; PAX2 Transcription Factor ; metabolism ; Tomography, X-Ray Computed ; Vimentin ; metabolism ; WT1 Proteins ; metabolism ; Wilms Tumor ; pathology ; Young Adult

12E7 Antigen ; Adolescent ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Lymph Node Excision ; Lymphatic Metastasis ; Lymphoma ; metabolism ; pathology ; Male ; Neoplastic Cells, Circulating ; Nephrectomy ; Neuroblastoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism ; Venae Cavae ; pathology ; Vimentin ; metabolism ; Wilms Tumor ; metabolism ; pathology

OBJECTIVEMatrine, a major ingredient of sophora, has an anti-tumor activity, capable of suppressing the proliferation and metastasis and promoting apoptosis or differentiation of tumor cells. This study was designed to investigate the effects of matrine on survival and apoptosis of nephroblastoma cell line SK-NEP-1, reduction of drug-resistance of cisplatin and the mechanism(s) underlying these effects.

METHODSSK-NEP-1 cells were treated with matrine and cisplatin at various doses (0.5, 1.0 and 1.5 mg/mL), either each alone or in combination. The viability in treated SK-NEP-1 cells was assessed by MTT colorimetric assay, apoptosis by flow cytometry, and PDCD4 mRNA abundance by RT-PCR.

RESULTSAs compared with the non-treatment control, matrine and cisplation, regardless of combination and dosage, significantly reduced the viability (P<0.01), induced apoptosis (P<0.01), and increased PDCD4 mRNA abundance (P<0.01), in SK-NEP-1 cells. The above effects of matrine and cisplation were dose-dependent when they were used alone, and were more pronounced when they were used in combination (P<0.05).

CONCLUSIONSMatrine can significantly induce apoptosis and inhibit growth of SK-NEP-1 cells in a dose-dependent manner, thus increasing the chemotherapeutic sensibility of cisplatin. The observed effects of matrine may be a result of increased PDCD4 expression.

Alkaloids ; administration & dosage ; Antineoplastic Agents ; administration & dosage ; Apoptosis ; drug effects ; Apoptosis Regulatory Proteins ; genetics ; Cell Line, Tumor ; Cell Survival ; drug effects ; Cisplatin ; administration & dosage ; Dose-Response Relationship, Drug ; Flow Cytometry ; Humans ; Quinolizines ; administration & dosage ; RNA, Messenger ; analysis ; RNA-Binding Proteins ; genetics ; Wilms Tumor ; drug therapy ; pathology

A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.
Adenocarcinoma ; Adenoma, Islet Cell ; Adult* ; Carcinoma, Acinar Cell ; Child ; Humans ; Korea ; Neoplasms, Glandular and Epithelial ; Pancreas ; Pathology ; Retinoblastoma ; Wilms Tumor

Child, Preschool ; Chromosome Deletion ; Chromosomes, Human, Pair 11 ; Humans ; Magnetic Resonance Imaging ; Male ; WAGR Syndrome ; diagnosis ; genetics ; pathology ; Wilms Tumor ; genetics ; pathology ; surgery

Humans ; Infant ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Male ; Mucin-1 ; metabolism ; Nephrectomy ; Precancerous Conditions ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; WT1 Proteins ; metabolism ; Wilms Tumor ; metabolism ; pathology ; surgery