scholarly journals Non-Wilms' Renal Tumors In Children:A 139 Cases Series

2021 ◽  
Author(s):  
Yi Wei Fang ◽  
Hong Cheng Song ◽  
Ning Sun ◽  
Wei Ping Zhang
Keyword(s):  
Cell Cancer ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Comprehensive Treatment ◽  
Malignant Rhabdoid Tumor ◽  
Cystic Nephroma ◽  
Single Center ◽  
Congenital Mesoblastic Nephroma ◽  
Lost To Follow Up

Abstract Background:Pediatric non-Wilms renal tumors (NWRTs) which comprise a small proportion of renal tumors,are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment.This study aimed to determine the clinical characteristics, management and prognosis of children with non-Wilms' renal tumors(NWRTs). Methods:Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01–2019.10) at a single center were reviewed retrospectively.Results:The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney(MRTK)(n: 30, 21.6%), renal cell cancer(RCC)(n: 26,18.7%), clear cell sarcoma of the kidney(CCSK)(n: 24,17.3%), congenital mesoblastic nephroma(CMN)(n: 21,15.1%), cystic nephroma(CN)(n: 16,11.5%),metanephric tumours(n: 12, 8.6%), renal angiomyoliporma(RAML)(n: 3, 2.2%), renal primitive neuroectodermal tumor(rPNET)(n: 2, 1.4%), renal hemangioma(n: 2, 1.4%), inflammatory myofibroblastic tumor(IMT)(n: 2, 1.4%), ossifying renal tumor of infancy(ORTI)(n: 1, 0.7%). 123 children were followed up with an average of 42 months. 16 children were lost to follow-up. Tumor-free survival was observed in 94 children. 28 children(22.8%) were died.Conclusions:Pediatric NWRTs comprises 19.1% of all renal tumors in our single center. Accurate diagnoses along with appropriate management are important factors in improving patients outcome. The mainstay treatment of malignant NWRTs including MRTK,CCSK,RCC and PNET is comprehensive treatment. The mainstay treatment of benign NWRTs including RAML,CN, ORTI, CMN,metanephric tumours, and renal hemangioma is surgical resection alone.

scholarly journals Cryoablation of Small Renal Tumors in Patients with Solitary Kidneys: Initial Experience

2008 ◽  
Vol 2008 ◽  
pp. 1-3 ◽  
Author(s):  
Ravi Munver ◽  
Grant I. S. Disick ◽  
Salvatore A. Lombardo ◽  
Vladislav G. Bargman ◽  
Ihor S. Sawczuk
Keyword(s):  
Tumor Location ◽  
Renal Tumors ◽  
Minimal Risk ◽  
Contralateral Kidney ◽  
Nephron Sparing ◽  
Feasible Alternative ◽  
Lost To Follow Up

Introduction. The purpose of this study was to evaluate the role of renal cryoablation in patients with solitary kidneys with the goals of tumor destruction and maximal renal parenchymal preservation.Methods. Eleven patients with single tumors were treated with cryoablation, of which 10 patients had solitary kidneys and 1 had a nonfunctioning contralateral kidney. All procedures were performed via an open extraperitoneal approach; ten tumors were treated with in-situ cryoablation and 1 tumor was treated with cryo-assisted partial nephrectomy.Results. Cryoablation was successfully performed without any preoperative complications. Mean patient age was 62.4 years (range 49–79), tumor location included: 6 (upper pole), 2 (mid-kidney), 3 (lower pole). The mean and median tumor size was 2.6 cm and 2.8 cm (range 1.2–4.3 cm), mean operative time 205 minutes (range 180–270 minutes), blood loss 98.5 ml (range 40–250 ml), and hospitalization 4.6 days (range 3–8 days). Creatinine values included: preoperative 1.43 mg/dL (range 1.2–1.9), postoperative 1.67 mg/dL (range 1.5–2.5), and nadir 1.57 mg/dL (range 1.3–2.1). All patients were followed postoperatively with magnetic resonance imaging for surveillance. At a median follow-up of 43 months, 9 patients had no evidence of recurrence, 1 patient has an enhancing indeterminate area, and 1 patient was lost to follow-up.Conclusion. Intermediate-term results suggest that renal cryoablation offers a feasible alternative for patients that require a maximal nephron-sparing effort with preservation of renal function and minimal risk of tumor recurrence.

scholarly journals Midterm results of consecutive periprosthetic femoral fractures Vancouver type A and B

2016 ◽  
Vol 6 (3) ◽  
Author(s):  
Klemens Trieb ◽  
Rainer Fiala ◽  
Christian Briglauer
Keyword(s):  
Femoral Fractures ◽  
Type A ◽  
Periprosthetic Femoral Fracture ◽  
Type B ◽  
Single Center ◽  
Midterm Results ◽  
Periprosthetic Femoral Fractures ◽  
Multimorbid Patients ◽  
Lost To Follow Up

Surgical treatment of periprosthetic femoral fractures has a high complication and mortality rate of more than 10%. The aim of this study is to report the outcome of a consecutive single center patient group. Thirty-four consecutive patients (mean age 81.2+/-8.5 years, 14 male, 20 female) with a periprosthetic femoral fracture Vancouver type A (n=5) or type B (n=29) were followed-up after 43.2 months, none of the patients were lost to follow- up. Nineteen of the patients were treated through change of the stem and cerclage fixation, five by plates and ten by cerclage cables. One successfully treated infection was observed. No further complications have been reported peri- or postoperatively, therefore resulting in 2.9% overall complication rate. These results demonstrate that precisely selected revision surgery protocol following periprosthetic femoral fractures within elderly multimorbid patients may lead to beneficial outcomes at a low risk of complications.

Adenocarcinoma Ex goblet cell: A retrospective experience.

2018 ◽  
Vol 36 (4_suppl) ◽  
pp. 847-847
Author(s):  
Satya Das ◽  
Jordan Berlin ◽  
Liping Du ◽  
Chanjuan Shi
Keyword(s):  
Goblet Cell ◽  
Single Center ◽  
Local Setting ◽  
Appendiceal Neoplasms ◽  
Metastatic Setting ◽  
Definitive Surgery ◽  
Group B ◽  
Lost To Follow Up ◽  
Peritoneal Spread

847 Background: Adenocarcinoma ex goblet cell tumors (AGCC) represent rare appendiceal neoplasms with neuroendocrine and adenocarcinoma features. They represent the most aggressive subset of goblet cell carcinoids (Group B and C) and are often categorized as chemotherapy unresponsive [Am J SurgPathol.2008;32(10):1429-43]. Despite this, most patients with AGCC are treated with colon cancer chemotherapy regimens. We explored outcomes in patients treated at our institution with these regimens. Methods: We performed a single center retrospective analysis of 22 biopsy-proven AGCC patients (13 men, 9 women, median age at diagnosis 54.5) at Vanderbilt who had undergone treatment for local or metastatic disease. PFS and OS were determined for each patient and stratified by features listed on Table 1. Patients who were lost to follow up were censored based on their last recorded clinic date. Results: 16 patients received chemotherapy in the metastatic setting and all 16 had peritoneal involvement; of these patients 4 underwent HIPEC. Median PFS and OS in HIPEC treated patients was 17 months and 33 months respectively. 8 patients received adjuvant chemotherapy with FOLFOX; 6 in the local setting and 2 in the metastatic setting. Patients with metastatic AGCC had a median PFS of 21.5 months and a median OS of 32.9 months. Patients with local disease had a median PFS of 32.8 months and an OS that was not reached (median duration of follow up 12.4 months). Conclusions: AGCC have a predilection for peritoneal spread. Patients who received either adjuvant FOLFOX post definitive surgery or FOLFOX/FOLFIRI in the metastatic setting post optimal cytoreduction had prolonged periods of PFS and OS. [Table: see text]

Functional and Gene Expression Analysis of the p53 Signaling Pathway in Clear Cell Sarcoma of the Kidney and Congenital Mesoblastic Nephroma

2002 ◽  
Vol 5 (3) ◽  
pp. 257-268 ◽  
Author(s):  
Noel A. Brownlee ◽  
Debra J. Hazen-Martin ◽  
A. Julian Garvin ◽  
Gian G. Re
Keyword(s):  
Tumor Tissue ◽  
Clear Cell ◽  
Wilms Tumor ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Mesoblastic Nephroma ◽  
Primary Tumors ◽  
Cell Sarcoma ◽  
Congenital Mesoblastic Nephroma ◽  
Mdr 1

Mutation of p53 has been implicated in progression of classical Wilms tumor (WT) into the anaplastic variant (AWT), drug resistance, and poor prognosis. Because of prognostic similarities, clear cell sarcoma of the kidney (CCSK) has been classified with AWT and other aggressive pediatric renal tumors, apart from congenital mesoblastic nephroma (CMN), which is instead a relatively benign tumor of neonates. Initially, CCSK and CMN were assumed to be ontologically related, but the role of p53 in the pathogenesis of either disease has not been sufficiently evaluated as in AWT. We examined the status of p53 in CMN and CCSK in comparison to AWT by immunohistochemistry and mRNA analysis of p53, the downstream effector p21 WAF-1/CIP-1 ( p21), the multidrug resistance gene MDR-1, a putative target of p53, and the p53-antagonist Mdm-2. Surprisingly, strong p53 nuclear immunoreactivity was found in cultures from two CMN specimens, but not in frozen or fixed tumor tissue from five other CMN specimens, nor in cell lines or tumor tissue from CCSK. Sequence analysis excluded p53 mutations. The size of the p53 mRNA in CMN and CCSK primary tumors excluded gross deletions or rearrangements. Low levels of Mdm-2 mRNA in CCSK and CMN primary tumors and cultures did not support a role for Mdm-2. Absence of MDR-1 mRNA excluded MDR-1 in the drug-resistant phenotype of CCSK. Cisplatin-induced p21 transactivation assays and G1 cell cycle arrest analyses showed that p21 transactivation and G1 arrest occurred in both CCSK and CMN cultures, demonstrating integrity of the p53 signal transduction pathway. Absence of p53 functional abnormalities excluded relationships between CCSK and CMN as in AWT, supporting the association of cellular CMN with congenital fibrosarcomas as more recently proposed.

scholarly journals Renal tumors: Age spectrum and histological distribution across childhood

2020 ◽  
Author(s):  
Tingting Liu ◽  
Lika’a Fasih Y. Al-Kzayer ◽  
Yozo Nakazawa ◽  
lei chen
Keyword(s):  
Early Childhood ◽  
Age Of Onset ◽  
Wilms Tumor ◽  
Malignant Tumors ◽  
Age Distribution ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Benign Tumors ◽  
Congenital Mesoblastic Nephroma ◽  
The Relationship

Abstract BackgroundThe objective of this study was to identify the relationship between the histopathological distribution of renal tumors and the age parameter across childhood period and to compare that with the available literature.MethodsA total of 193 pediatric patients with renal tumors were classified into 5 groups as embryo, infant, early childhood, childhood, and adolescent. Age distribution, pathological types and clinical characteristics were summarized. ResultsAmong the 193 patients, 95.8% presented with malignant tumors, and 4.2% showed benign tumors. The prevalence of Wilms’ tumor (WT( and non-WT, were 63.7% and 36.3%, respectively. Non-WT patients included clear cell sarcoma of the kidney (CCSK), followed by rhabdoid tumor of the kidney (RTK), congenital mesoblastic nephroma (CMN), cystic partially differentiated nephroblastoma (CPDN), and renal cell carcinoma (RCC). Early childhood (89/193, 46.2%) was the most common period of onset, followed by infant (73/193, 37.9%), childhood (19/193, 9.8%), embryo (8/193, 4.1%) and adolescent (4/193, 2.0%). WT predominantly occurred in infant and early childhood and decreased among older age group. Non-WT occurred various period of onset and rather common during embryo. 10.9% of patients were asymptomatic at the time of diagnosis and that was significantly observed in non-WT (P=0.04). ConclusionsAge of onset is a highly significant factor for pediatric renal tumors. Relationship between age spectrum and pathological characteristics influences distinguishing the histopathological distribution of WT and non-WT.

scholarly journals Renal tumors: Age spectrum and histological distribution across childhood

2020 ◽  
Author(s):  
Tingting Liu ◽  
Lika’a Fasih Y. Al-Kzayer ◽  
Yozo Nakazawa ◽  
lei chen
Keyword(s):  
Early Childhood ◽  
Age Of Onset ◽  
Wilms Tumor ◽  
Malignant Tumors ◽  
Age Distribution ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Benign Tumors ◽  
Congenital Mesoblastic Nephroma ◽  
The Relationship

Abstract Background: The objective of this study was to identify the relationship between the histopathological distribution of renal tumors and the age parameter across childhood period and to compare that with the available literature. Methods: A total of 193 pediatric patients with renal tumors were classified into 5 groups as embryo, infant, early childhood, childhood, and adolescent. Age distribution, pathological types and clinical characteristics were summarized. Results: Among the 193 patients, 95.8% presented with malignant tumors, and 4.2% showed benign tumors. The prevalence of Wilms’ tumor (WT( and non-WT, were 63.7% and 36.3%, respectively. Non-WT patients included clear cell sarcoma of the kidney (CCSK), followed by rhabdoid tumor of the kidney (RTK), congenital mesoblastic nephroma (CMN), cystic partially differentiated nephroblastoma (CPDN), and renal cell carcinoma (RCC). Early childhood (89/193, 46.2%) was the most common period of onset, followed by infant (73/193, 37.9%), childhood (19/193, 9.8%), embryo (8/193, 4.1%) and adolescent (4/193, 2.0%). WT predominantly occurred in infant and early childhood and decreased among older age group. Non-WT occurred various period of onset and rather common during embryo. 10.9% of patients were asymptomatic at the time of diagnosis and that was significantly observed in non-WT (P=0.04). Conclusions: Age of onset is a highly significant factor for pediatric renal tumors. Relationship between age spectrum and pathological characteristics influences distinguishing the histopathological distribution of WT and non-WT.

scholarly journals 500 penile prostheses implanted by a surgeon in Italy in the last 30 years

2015 ◽  
Vol 87 (3) ◽  
pp. 216 ◽  
Author(s):  
Diego Pozza ◽  
Mariangela Pozza ◽  
Marco Musy ◽  
Carlotta Pozza
Keyword(s):  
Erectile Dysfunction ◽  
Penile Prosthesis ◽  
Great Majority ◽  
Public Hospitals ◽  
Single Center ◽  
Experienced Surgeon ◽  
Penile Prostheses ◽  
Lost To Follow Up ◽  
Prosthetic Surgery

Objectives: The aim of our study was to report our experience with patients affected by Erectile Dysfunction (ED) and undergoing penile prosthetic implantation (PPI) in a single center by a single surgeon. Material and Methods: We retrospectively evaluated the clinical outcome of 500 patients (mean age: 51.5 years, range: 20-86 years) affected by ED and referred to our private andrological center from January 1984 to December 2013 who underwent penile prosthesis implantation, including the reported level of patient satisfaction. Results: 182 silicone, 180 malleable, 18 monocomponent hydraulic and 120 multicomponents hydraulic prostheses were implanted by the same experienced surgeon. All patients were hospitalized for the procedure. All patients were evaluated immediately, 1 month (496 patients) and, for the great majority, every year after implantation. One hundred twenty five patients were lost to follow-up. Twenty two patients underwent revision surgery for complications in the postoperative period. The most serious postoperative complications were mechanical problems (45 patients, 9.0%) and infection (15 patients, 3%). Forty two (8.4%) prostheses were explanted. Overall, 80% (400/500) of patients were able to have sexual intercourse and were fully satisfied with the results. Conclusions: In our experience prosthetic surgery should be considered a good solution for men affected by ED and not responsive to other therapeutic solutions. Prosthetic surgery can be performed not only in large public hospitals but also in smaller private facilities.

scholarly journals Chiari-related scoliosis: a single-center experience with long-term radiographic follow-up and relationship to deformity correction

2018 ◽  
Vol 21 (2) ◽  
pp. 185-189 ◽  
Author(s):  
Vijay M. Ravindra ◽  
Kaine Onwuzulike ◽  
Robert S. Heller ◽  
Robert Quigley ◽  
John Smith ◽  
...  
Keyword(s):  
Cobb Angle ◽  
Univariate Analysis ◽  
Deformity Correction ◽  
Single Center ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Single Center Experience ◽  
Lost To Follow Up

OBJECTIVEPrevious reports have addressed the short-term response of patients with Chiari-related scoliosis (CRS) to suboccipital decompression and duraplasty (SODD); however, the long-term behavior of the curve has not been well defined. The authors undertook a longitudinal study of a cohort of patients who underwent SODD for CRS to determine whether there are factors related to Chiari malformation (CM) that predict long-term scoliotic curve behavior and need for deformity correction.METHODSThe authors retrospectively reviewed cases in which patients underwent SODD for CRS during a 14-year period at a single center. Clinical (age, sex, and associated disorders/syndromes) and radiographic (CM type, tonsillar descent, pBC2 line, clival-axial angle [CXA], syrinx length and level, and initial Cobb angle) information was evaluated to identify associations with the primary outcome: delayed thoracolumbar fusion for progressive scoliosis.RESULTSTwenty-eight patients were identified, but 4 were lost to follow-up and 1 underwent fusion within a year. Among the remaining 23 patients, 11 required fusion surgery at an average of 88.3 ± 15.4 months after SODD, including 7 (30%) who needed fusion more than 5 years after SODD. On univariate analysis, a lower CXA (131.5° ± 4.8° vs 146.5° ± 4.6°, p = 0.034), pBC2 > 9 mm (64% vs 25%, p = 0.06), and higher initial Cobb angle (35.1° ± 3.6° vs 22.8° ± 4.0°, p = 0.035) were associated with the need for thoracolumbar fusion. Multivariable modeling revealed that lower CXA was independently associated with a need for delayed thoracolumbar fusion (OR 1.12, p = 0.0128).CONCLUSIONSThis investigation demonstrates the long-term outcome and natural history of CRS after SODD. The durability of the effect of SODD on CRS and curve behavior is poor, with late curve progression occurring in 30% of patients. Factors associated with CRS progression include an initial pBC2 > 9 mm, lower CXA, and higher Cobb angle. Lower CXA was an independent predictor of delayed thoracolumbar fusion. Further study is necessary on a larger cohort of patients to fully elucidate this relationship.

scholarly journals Malignant renal tumors in children

2015 ◽  
Vol 2 (3) ◽  
pp. 84-89 ◽  
Author(s):  
Justin Scott Lee ◽  
Thomas Ray Sanchez ◽  
Sandra Wootton-Gorges
Keyword(s):  
Clear Cell ◽  
Wilms Tumor ◽  
Pediatric Population ◽  
Medullary Carcinoma ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Malignant Rhabdoid Tumor ◽  
Renal Masses ◽  
The Common ◽  
Prompt Diagnosis

Renal malignancies are common in children. While the majority of malignant renal masses are secondary to Wilms tumor, it can be challenging to distinguish from more aggressive renal masses. For suspicious renal lesions, it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment. This review article will discuss the common differential diagnosis that can be encountered when evaluating a suspicious renal mass in the pediatric population. This includes clear cell sarcoma of the kidney, malignant rhabdoid tumor, renal medullary carcinoma and lymphoma. 

scholarly journals Primary Small Cell Carcinoma in Nasal Cavity and Paranasal Sinuses: 15 Cases From a Single Center

2021 ◽  
pp. 014556132110498
Author(s):  
Jian Wang ◽  
Yingying Fan ◽  
Xiao-dong Chen ◽  
Tao Xue ◽  
Fu-quan Chen
Keyword(s):  
Overall Survival ◽  
Nasal Cavity ◽  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Tumor Recurrence ◽  
Small Cell ◽  
Comprehensive Treatment ◽  
Single Center

Small cell carcinoma (SCC) in the nasal cavity and sinuses is extremely rare. The clinical data of 15 patients with primary SCC in nasal cavity and sinuses were analyzed retrospectively. All patients were treated with surgery, radiotherapy, and chemotherapy. Of the 15 patients, 2 patients are alive for more than 6 years, and 5 patients died after the median follow-up period (11 months). Most of our patients represent the later stage (73% presented at stage III or IV) and had surgery combined with radiotherapy and chemotherapy; however, nearly half of patients have tumor recurrence and/or distant metastasis. SCC of nasal cavity and sinuses often invades surrounding tissues, and the long-term curative rate is generally low. Early diagnosis and comprehensive treatment are key to improve survival. Although the overall survival time of SCC is not optimistic, it is still recommended that patients take comprehensive treatment.

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