Renal Cell Carcinoma In Children Research Articles

Renal cell carcinoma in children: the results of retrospective analysis

Renal cell carcinoma in children: the results of retrospective analysis

Nephron-sparing surgery for renal cell carcinoma in children and young adults: A systematic review.

To determine the patient characteristics and role of nephron-sparing surgery (NSS) in the treatment of children and young adults with renal cell carcinoma (RCC). A systematic search of Embase, MEDLINE, and Scopus databases was conducted in December 2021 according to Cochrane collaboration recommendations. All included manuscripts were assessed for patient characteristics and all reported outcomes for patients undergoing partial nephrectomy (PN), and radical nephrectomy (RN) outcomes were abstracted as a comparison group. Primary outcomes included surgical outcomes, overall survival, kidney outcomes. Outcomes were pooled with weighted mean and ranges. Meta-analysis was not performed given study quality. This systematic review was prospectively registered on PROSPERO (CRD42022300261). We found a total of 16 studies describing 119 and 559 unique patients undergoing PN and RN, respectively, with a mean age of 12.2 years and mean follow-up of 59.1 months. The mean tumor size for patients undergoing PN was 3.5 cm. Of the 113 patients undergoing PN with available data, 109 were alive at follow-up (98%). No studies reported long-term kidney outcomes, and four studies reported surgical outcomes. All studies had at least moderate risk of bias. The use of NSS in children and young adults with RCC is feasible in selected patients. However, small sample sizes, confounding, and low study quality limit clinical recommendation on NSS in this population. There are significant opportunities for future research on the use of NSS in RCC, especially with systematic reporting of oncological, kidney, and surgical outcomes.

Renal cell carcinoma in children and adolescents: Single-center experience and literature review.

Renal cell carcinoma (RCC) is infrequent in the pediatric population. In addition, till date, only a few reports have summarized the characteristics of pediatric RCC and differences between pediatric and adult RCC. Therefore, the current study aimed to investigate the clinical characteristics of RCC in children and adolescents, and identify the differences between children and adolescent patients and adult patients through literature retrieval.The data of 13 pediatric patients diagnosed with RCC at the Children's Hospital of Zhejiang University School of Medicine between 2005 and 2019 were retrospectively analyzed.Three patients were aged <5 years, 2 were aged 6 to 10 years, and 8 were aged 11 to 18 years. Among the 13 patients, common clinical manifestations included abdominal pain in 5 patients, gross hematuria in 4, and an abdominal mass in 1, while the other 3 patients were incidentally detected after an abdominal contusion. The pathological types were microphthalmia family translocation RCC in 9 patients, clear-cell RCC in 2, papillary RCC in 1, and unclassified in 1. All the children underwent radical nephrectomy, including 2 patients with advanced disease who underwent preoperative transcatheter arterial chemoembolization. The mean follow-up time was 58.6 months. Two patients died after 4 and 17 months of follow-up, respectively.In conclusion, microphthalmia family translocation renal cell carcinoma is the predominant type of pediatric RCC associated with advanced tumor stage. The early diagnosis and treatment of pediatric patients is important for improving prognosis. Nevertheless, future studies are urgently needed to determine the treatment for pediatric advanced RCC to increase the survival rate.

Renal Cell Carcinoma in Children: A Report of Three Cases

Renal Cell Carcinoma in Children: A Report of Three Cases

Clinicopathological characterisation of renal cell carcinoma in young adults: a contemporary update and review of literature.

Renal cell carcinomas are relatively rare in children and young adults. While well characterised in adults, the morphological and molecular characterisation of these tumours in young patients is relatively lacking. The objective of this study was to explore the spectrum of renal cell carcinoma (RCC) subtypes in children and young adults and to determine their clinico-pathological, immunohistochemical and molecular characteristics by evaluating a large retrospective cohort of renal cell carcinoma patients age 30years or younger. Sixty-eight cases with confirmed diagnosis of renal cell carcinoma at age 30years or younger were identified at our institution. Clear cell carcinoma accounted for the most common subtype seen in this age group. Translocation renal cell carcinoma and rare familial syndrome subtypes such as succinate dehydrogenase deficient renal cell carcinoma and tuberous sclerosis complex-associated renal cell carcinoma were found relatively more frequently in this cohort. Despite applying the 2016 WHO classification criteria, a high proportion of the tumours in our series remained unclassified. Our results suggest that renal cell carcinoma in children and young adults is a relatively rare disease that shares many histological similarities to renal cell carcinoma occurring in adults and yet demonstrate some unique clinical-pathological differences. Microphthalmia-associated transcription (MiT) family translocation RCC and rare familial syndrome subtypes are relatively more frequent in the paediatric and adolescent age groups than in adults. Clear cell RCC still accounted for the most common subtype seen in this age group. MiT family translocation RCC patients presented with advanced stage disease and had poor clinical outcomes. The large and heterogeneous subgroup of unclassified renal cell carcinoma contains phenotypically distinct tumours with further potential for future subcategories in the renal cell carcinoma classification.

Pediatric renal cell carcinoma.

To review the presentation, natural history and treatment of renal cell carcinoma in children and young adults with renal cell carcinoma (RCC). Complete resection of lymph nodes at the time of tumor resection can improve clinical outcomes and limit the need for adjuvant chemotherapy. Genetic alterations that lead to translocation tumors are a therapeutic target of receptor tyrosine kinase inhibitors. The incidence of RCC increases with age. Unlike adult patients, young patients with RCC present symptomatically and at higher stage and grade. Translocation tumors predominate RCC in children with biologic activity characterized by early spread to lymph nodes with small primary tumors. Preoperative imaging is poorly sensitive for positive lymph nodes; as such, surgeons should have a low threshold for lymph node sampling during tumor resection. Despite the advanced stage at presentation, the prognosis in children is more favorable than their adult counterparts. Complete resection of lymph nodes at the time of surgical resection improves patient prognosis. Chemotherapy targeting the PI3/AKT pathway has demonstrated clinical benefit.

Renal Cell Carcinoma in Children: 5 years experience from a Regional Cancer Centre from South India

Renal Cell Carcinoma in Children: 5 years experience from a Regional Cancer Centre from South India

Renal Cell Cancer in a 6 Years Old Patient: Rare Clinical Case

Background . According to the data of the world literature and own data of observations renal cell carcinoma (RCC) in children is a rare pathology. There are mainly localized and locally common forms of the disease, characterized by a good prognosis and rare reccurence. The tactic of the treatment in this group of patients based on radical surgery. If there are distant metastases, adjuvant chemotherapy is performed. The drug of choice in metastatic forms is a tyrosine kinase ingibitor. Description of a Clinical Case . A review of the world literature on the problem of RCC in children and also the clinical observation of RCC in a 6 years old patient, who treated in N.N. Blokhin National Medical Research Center of Oncology (Moscow), are presented. Now the patient is healthy and is under the dynamic observation. Conclusion . The rarity of RCС requires careful examination and observation of patients, morphological and molecular-genetic study of the tumor for features of biology and develop of «adult» cancer in children.

A clinical study on the expression of PTEN in renal cell carcinoma in children.

The expression pattern of tumor suppressor gene phosphatase and tensin homolog deleted on chromosome ten (PTEN) and phosphatase and tensin homolog deleted on chromosome ten/phosphatidylinositol3-kinase/protein kinase B (PTEN/PI3K/AKT) cell signaling pathway in renal cell carcinoma (RCC) were investigated in children. A total of 5 cases of RCC (observation group) in children and 10 cases of benign kidney tumor (control group) diagnosed by pathological examinations were included to obtain tumor samples. Expression of PTEN mRNA was detected by reverse transcription-quantitative polymerase chain reaction (RT-qPCR). The protein expression of PTEN, PI3K and AKT was detected by western blotting; relationships between the expression level of PTEN mRNA and the clinical features of RCC were analyzed. It turned out that expression level of PTEN mRNA in the observation group was significantly lower than that in the control group. The protein expression levels of PTEN, PI3K and AKT were significantly lower in the observation group than in the control group (P<0.05). The expression level of PTEN mRNA decreased with the increased clinical stage of RCC (P<0.05), and was not related to sex, age and maximum tumor diameter (P>0.05). The results showed that downregulation of the tumor suppressor gene PTEN expression and the inhibition of PTEN/PI3K/AKT cell signaling pathway may be involved in the occurrence and development of RCC in children.

Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade: From the Radiologic Pathology Archives.

Malignant renal tumors account for 7% of childhood cancers, and Wilms tumors are by far the most common-but not in older children and adolescents. Among individuals in the latter half of their 2nd decade of life, renal cell carcinoma (RCC) is more common than Wilms tumor. The histopathologic spectrum of RCCs in children differs from that in adults. The most common subtype of RCC in children and adolescents is Xp11.2 translocation RCC, which is distinguished by hyperattenuation at nonenhanced computed tomography, a defined capsule, and associated retroperitoneal lymphadenopathy. Papillary RCC is the second most common histologic subtype. It enhances less intensely compared with the adjacent renal parenchyma and has a propensity for calcification. Clear cell RCC is seen in patients with von Hippel-Lindau disease and is distinguished by its relatively hypervascular nature. Medullary carcinoma affects adolescents with the sickle cell trait and is characterized by an infiltrative growth pattern and extensive metastasis at presentation. Angiomyolipoma is seen in children with tuberous sclerosis complex and is often multifocal and hypervascular, with macroscopic fat. Metanephric tumors are central, circumscribed, and typically calcified. Lymphoma usually manifests as multifocal masses, but it may involve a solitary mass or infiltrative pattern. Extensive adenopathy and involvement of the gastrointestinal tract or other organs also may be seen. Primitive neuroectodermal tumor is an aggressive neoplasm that is typically quite large at diagnosis. Knowledge of the clinical, biologic, and histopathologic features of renal tumors in older children and adolescents and their effects on the imaging appearance can help the radiologist offer a useful preoperative differential diagnosis.

Kidney Cancers in Adolescents &amp; Young Adults

Kidney Cancers in Adolescents &amp; Young Adults

Renal Cell Carcinoma in Children: About Two Case Report

Introduction Renal cell carcinoma (RCC) is extremely rare in children. Several factors could influence prognosis, including stage, grade, histology, symptomatic presentation, and performance status. Among these, tumor stage is the most important predictor of disease prognosis for RCC. We report our experience with two cases of children with renal cell carcinoma in children. Cases Case 1: A 13-year-old girl presented macroscopic hematuria after a right lumbar trauma. The renal ultrasonography and CT urography showed a very limited rounding medio renal mass. Fine needle renal aspiration objectified RCC. Patient underwent transabdominal radical nephrectomy. Histopathology revealed RCC grade 2 of Furhrman T1b N0 M0. The postoperative course was uneventful. Case 2: A 9-year-old girl presented macroscopic hematuria. The renal ultrasonography showed a horseshoe left kidney with limited rounding mass. CT urography showed limited left renal mass of 31 × 25 mm. Fine needle renal aspiration objectified RCC. Patient underwent transabdominal radical nephrectomy with regional lymphadenectomy. Histopathology revealed RCC associated with translocation Xp11.2 grade 2 of Furhrman T1a N1M0. The postoperative course was uneventful. Conclusion RCC is rare in children but in children older than 5 years with renal masses it is very important to suspect diagnosis. Surgery is the best treatment and prognosis is favorable when the tumor is localized and completely eradicated.

Clinicohistological characteristics of renal cell carcinoma in children: A multicentre study.

In this retrospective multicentre study, we compared the clinicohistological characteristics of renal cell carcinoma (RCC) between pediatric and adult patients. Data for patients who underwent radical or partial nephrectomy for RCC between 1988 and 2014 at multiple institutions were collected. Patients were divided into 2 groups according to age at diagnosis: pediatric patients (age ≤18 years) and adult patients (age ≥40 years). The groups were compared for clinical and pathologic variables, and survival analysis was performed. The median follow-up period was 64 (range: 30-91) months for pediatric patients versus 44 (range: 19-59) months for adult patients (p = 0.026). Pediatric patients were mostly female (p = 0.003), had symptoms at presentation (p < 0.001), and had a high-stage tumour (p = 0.014) than adult patients. Among the symptomatic patients, gross hematuria was the most common symptom. The median tumour size was not different between groups. Regarding histologic types, pediatric patients had more papillary tumours (p < 0.001), more unclassified tumours (p < 0.001), and fewer clear cell carcinomas (p < 0.001). Five-year cancer-specific survival rates were 85% and 87.4% in pediatric and adult patients, respectively (log rank p = 0.901). Recurrence-free survival was better in adult patients, although this did not reach statistical significance (log rank p = 0.272). This study has several limitations, including its retrospective nature and the relatively small number of pediatric RCC cases. RCC in children is rare and is characterized by features that differ from those in adult RCC. Prognosis did not differ between groups.

This Month in Pediatric Urology

No AccessJournal of UrologyThis Month in Pediatric Urology1 Apr 2015This Month in Pediatric Urology Michael L. Ritchey Michael L. RitcheyMichael L. Ritchey More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2015.01.035AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "This Month in Pediatric Urology." The Journal of Urology, 193(4), pp. 1079–1080 References 1 : Postnatal germ cell development during mini-puberty in the mouse does not require androgen receptor: implications for managing cryptorchidism. J Urol2015; 193: 1361. Link, Google Scholar 2 : Canalization of the urethral plate precedes fusion of the urethral folds during male penile urethral development: the double zipper hypothesis. J Urol2015; 193: 1353. Link, Google Scholar 3 : Bladder volume at onset of vesicoureteral reflux is an independent risk factor for breakthrough febrile urinary tract infection. J Urol2015; 193: 1342. Link, Google Scholar 4 : Renal cell carcinoma in children, adolescents and young adults: a National Cancer Database study. J Urol2015; 193: 1336. Link, Google Scholar 5 : A pilot randomized controlled trial evaluating the effectiveness of group vs individual urotherapy in decreasing symptoms associated with bladder-bowel dysfunction. J Urol2015; 193: 1347. Link, Google Scholar © 2015 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 193Issue 4April 2015Page: 1079-1080 Advertisement Copyright & Permissions© 2015 by American Urological Association Education and Research, Inc.MetricsAuthor Information Michael L. Ritchey More articles by this author Expand All Advertisement PDF downloadLoading ...

Pediatric Papillary Renal Cell Carcinoma in a Horseshoe Kidney: A Case Report with Review of the Literature.

Renal cell carcinoma is the most common malignancy of the kidney in adults. In children, however, it only accounts for an estimated 1.8 to 6.3% of all pediatric malignant renal tumors. Papillary renal cell carcinoma is the second most common type of renal cell carcinoma in children. We present the case of a 12-year-old boy with a 2-month history of abdominal pain, unexplained weight loss, and gross hematuria. Computed tomography revealed a horseshoe kidney and a well-defined mass of 4 cm arising from the lower pole of the right kidney. Microscopically the tumor was composed of papillae covered with cells with abundant eosinophilic cytoplasm and high-grade nuclei with prominent nucleoli. Immunohistochemistry was performed; EMA, Vimentin, and AMACR were strongly positive while CK7, CD10, RCC antigen, TFE3, HMB-45, and WT-1 were negative. Currently, 10 months after the surgical procedure, the patient remains clinically and radiologically disease-free.

Renal Cell Carcinoma in Children, Adolescents and Young Adults: A National Cancer Database Study

We compared the presentation and outcomes of patients younger than 21 years with renal cell carcinoma and determined risk factors associated with mortality. We searched the National Cancer Database for patients diagnosed with renal cell carcinoma between 1998 and 2011. We evaluated patients younger than 30 years with renal cell carcinoma, including clear cell, chromophobe, papillary and not otherwise specified subcategories. We used logistic regression to compare presenting cancer, demographics and treatment variables in patients 0 to 15 years, 15 to 21 years and 21 to 30 years old. Cox regression analysis was used to determine risk factors for mortality in patients younger than 21. Of 3,658 patients younger than 30 years included in the study 161 were younger than 15 and 337 were 15 to 21 years old. A higher proportion of younger patients had renal cell carcinoma not otherwise specified and papillary histology compared to those 21 to 30 years (p < 0.001). Younger patients presented with higher stage (p < 0.0001), higher grade (p < 0.0001) and larger tumors (p < 0.0001) than those 21 to 30 years. A higher percentage of younger patients underwent lymph node dissection (p < 0.0001) or chemotherapy as first-line treatment (p < 0.0001) compared to those 21 to 30 years. Cox regression analysis demonstrated that stage 4 presentation, government insurance status, nonchromophobic pathology results and not undergoing surgery as first-line treatment were independently associated with increased mortality in patients younger than 21 years. Children and adolescents with renal cell carcinoma present with more advanced disease than those 21 to 30 years old. In patients younger than 21 years mortality was associated with the nonchromophobe histological subtype, stage 4 disease, government insurance and not undergoing surgery as first-line therapy.

VCL-ALK Renal Cell Carcinoma in Children With Sickle-cell Trait

We report the third case of a renal cell carcinoma bearing a fusion of the vinculin (VCL) and anaplastic lymphoma kinase (ALK) genes. Like the 2 other reported cases, this neoplasm occurred in a young patient (6 y old) with sickle-cell trait and demonstrated distinctive morphologic features including medullary epicenter, discohesive polygonal or spindle-shaped cells with prominent cytoplasmic vacuoles, and prominent lymphocytic infiltrate. The neoplastic cells demonstrated focal membranous labeling for ALK protein by immunohistochemistry, ALK gene rearrangement by fluorescence in situ hybridization, and a specific VCL-ALK gene fusion by reverse transcriptase polymerase chain reaction. VCL-ALK renal cell carcinoma may represent the eighth sickle-cell nephropathy.

Metastatic Renal Cell Carcinoma in Children and Adolescents

Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents. The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients. Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5%) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively. Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20%) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.

Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect.

The concept of Xp11.2 renal cell carcinoma (RCC) was recently established as a tumor affecting 15% of RCC patients <45 years. Many patients present with advanced stage with frequent lymph node metastases. Histologically, Xp11.2 RCC is characterized by mixed papillary nested/alveolar growth pattern and tumor cells with clear and/or eosinophilic, voluminous cytoplasm. Neoplastic cells show intense nuclear immunoreactivity to TFE3, while focal immunostaining for melanocytic markers, including melanosome-associated antigen or Melan A in some cases, are also noted. Alpha smooth muscle actin and TFEB are consistently negative. Ultrastructurally, the ASPL-TFE3 RCC variant contains rhomboid crystals in the cytoplasm, similar to that observed in alveolar soft part sarcoma. The fusion of the TFE3 gene with several different genes, including ASPL(17q25), PRCC(1q21), PSF(1q34), NonO (Xq12) and CLTC (17q23) have been identified to date. The behavior of Xp11.2 RCC in children and young adults is considered as indolent even when diagnosed at advanced stage, including lymph node metastasis. However, Xp11.2 RCC in older patients behaves in a more aggressive fashion. Therapy includes nephrectomy with extended lymphadenectomy. There may be a role for new protease inhibitors in advanced inoperable disease. Further research is required to correlate clinical behavior with the expanding genetic spectrum of this tumor, and to establish standard therapy protocols for primary and metastatic lesions.

Diagnosis and Treatment of Renal Cell Carcinoma in Children: A Report from the Polish Pediatric Rare Tumor Study Group

Renal cell carcinoma (RCC) in children is rare, accounting for approximately 1.9-6% of all pediatric renal malignancies. The aim of this study was to transmit our experience in the treatment of RCC in Polish children. Clinical data from 21 children (6.3-18 years old) with RCC treated between 1992 and 2009 at Polish pediatric oncological centers were analyzed. In 2 patients, RCC developed as a second malignancy after neuroblastoma or astrocytoma fibrillare, respectively. In 6 patients, initial diagnoses based on imaging studies were unilateral Wilms' tumor, leading to preoperative chemotherapy. The remaining patients underwent surgery at the beginning of treatment. According to the AJCC/TNM staging system, 14 patients had stage I, 5-II, 1-III, and 1-IV. Nephrectomy was performed in 19 patients, heminephrectomy in one, and biopsy in another. Histopathological diagnoses were clear-cell RCC (18 patients), papillary RCC (2 patients), and chromophobe RCC (1 patient). 10 patients were treated with chemotherapy, with or without IL-2, INFα, and antiangiogenic agents. 2 patients died due to disease progression. RCC in children is mostly operable at diagnosis, resulting in good prognosis. The role of adjuvant chemo- and immunotherapies is unclear. Neoadjuvant chemotherapy proven for children with Wilms' tumors is ineffective, but the delay in adequate therapy did not worsen the outcome if complete nephrectomy is done subsequently.