Does bilateral Wilms' tumor involving the collecting system in children have a worse prognosis?

BACKGROUND: The literature on nephron-sparing surgery (NSS) in children with bilateral Wilms' tumors (BWT) involving the collection system is mostly comprised of case reports. The present study aimed to summarize the clinical characteristics, treatments, and prognosis of children with BWT involving the collecting system admitted to our pediatric surgery center compared with those whose tumors did not involve the collecting system. A secondary aim was to discuss how to preserve more kidney parenchyma and prevent long-term renal failure under the premise of preventing tumor recurrence. METHODS: Patients with BWT admitted to our pediatric surgery center between January 2008 and June 2022 were reviewed. All included patients were grouped according to the relationship between the tumor and collecting system according to the intraoperative findings. Group I included children with tumor infiltrating the collecting system, group II included children with tumor growing into the collecting system, and group III included children whose tumor did not involve the collecting system. The clinical features, treatments and prognosis of the patients were analyzed. RESULTS: Seventy patients were enrolled, including 20 patients with 25 sides of tumors infiltrating the collecting system in group I,10 patients with 13 sides of tumors growing into the collecting system in group II, and 40 patients in group III. There was no significant difference in patients age and gender between group I and group II. In total, 20 patients in group I and 9 patients in group II had partial response (PR) after neoadjuvant chemotherapy. In group I, 22 of 25 sides of tumors underwent NSS; in group II, 11 of 13 sides of tumors underwent NSS. During an average follow-up of 47 months, in group I, 6/20 patients relapsed and 2/20 patients died; in group II, 3/10 patients relapsed and 1/10 patient died. There was no significant difference in 4-year overall survival (OS) rate among groups I, II and III (86.36% vs. 85.71%vs. 91.40%, P = 0.902). CONCLUSIONS: To preserve renal parenchyma, NSS is feasible for children with BWT involving the collecting system. There was no significant difference in postoperative long-term OS between patients with BWT involving the collecting system and not involving the collecting system.

Collecting duct carcinoma associated with oncocytoma

Collecting duct carcinoma (CDC) is a rare, highly aggressive malignant neoplasm that arises from the collecting duct epithelium of the kidney. CDC was reported to coexist with renal cell and transitional cell carcinomas. We report a rare case of CDC associated with oncocytoma, confirmed by the characteristic histological appearance and immunohistochemistry. We also review the epidemiological, histological and immunohistochemical criteria for diagnosis, in addition to the genetic and cytogenetic aberrations reported in the literature. Identification and reporting CDC is important for the establishment of treatment strategies and monitoring prognosis.

Caracterização de eventos na fibrose túbulo-intersticial tratada com micofenolato mofetil e/ou losartan no modelo de ablação renal / The characteriation of eventss in tubuloointersticial fibrosis attenuated bby losartan and/or micopphenollate mofetil therapy in the remnant kidnney

Para caracterizar os eventos na fibrose túbulo-intersticial renal, foi realizado estudo em ratos submetidos a nefrectomia 5/6 e tratados com micofenolato mofetil e/ou losartan. Cortes histológicos de rim foram submetidos a processamento histológico e imuno-histoquímico para a identificação e quantificação do colágeno e de vários marcadores de células epiteliais, mesenquimais e inflamatórias. O estudo morfométrico foi realizado utilizando-se imagens digitalizadas e método computadorizado de análise de imagem. Verificou-se redução da deposição de colágeno e da expressão daqueles marcadores celulares, principalmente nos grupos tratados com a associação micofenolato mofetil e losartan / In order to characterize the events of tubulointerstitial fibrosis, it was performed a study in 5/6 ablation rats receiving losartan and/or micophenolate mofetil therapy. Kidney sections were submitted to several histochemical and immunohistochemical procedures. Morphometric evaluation was performed using digitalized image and a computer assisted image analyzer...

Análise do padrão de expressão do produto de PKHD1, o gene mutado na doença renal policística autossômica recessiva / Analysis of the expression pattern of the PKHD1 gene product, mutated in autosomal recessive polycistic kidney disease

O gene PKHD1, mutado na doença renal policística autossômica recessiva, apresenta um padrão de splicing complexo associado a múltiplos transcritos alternativos. Neste trabalho estudamos o perfil de expressão de seu produto, poliductina. Análises por western blot revelaram produtos putativos de membrana de >440 kDa e aproximadamente 230 kDa, e de aproximadamente 140 kDa em frações solúveis de rim, fígado e pâncreas. Estudos imunoistoquímicos mostraram marcação em ductos coletores renais e porção ascendente espessa da alça de Henle, em epitélios ductais biliar e pancreático e, no período embrionário, em broto ureteral, ductos biliar e pancreático e glândula salivar. /PKHD1, the gene mutated in autosomal recessive polycystic kidney disease, presents a complex splicing pattern, associated with multiple alternative transcripts. In this work we have studied the expression profile of its product, polyductin. Western blot analysis revealed putative membrane products of >440 kDa and 230 kDa, and of about 140 kDa in soluble fractions in kidney, liver and pancreas. Immunohistochemistry studies showed staining in renal collecting duct and thick ascending limb of Henle, in biliary and pancreatic ductal epithelia and, in the embryonic period, in ureteric bud, biliary and pancreatic ducts and salivary gland...

Carcinoma de celulas de Bellini o carcinoma de los conductos colectores: aspecto por RM / Bellini's Cell Carcinoma or Carcinoma of collecting tubules: appearance by MR

Se presenta el caso de un paciente de 77 años con hematuria y antecedentes de carcinoma de próstata y carcinoma de colon a quien se le encontró masa renal central sólida, con realce heterogéneo con el medio de contraste en los estudios de TAC y RM. Su diagnóstico histopatológico es el de carcinoma de los conductos colectores. Se discuten los diagnósticos diferenciales por imágenes diagnósticas y su caracterización por estudios histopatológico