Genitourinary Tract Tumors in Children: An Update

2022 ◽  
Vol 18 ◽  
Author(s):  
Andrés Augusto González-Arboleda ◽  
Nicolás Fernandez ◽  
Herney Andrés García-Perdomo
Keyword(s):  
Spanish Literature ◽  
Wilms Tumor ◽  
Technological Development ◽  
Transitional Cell ◽  
Survival Rates ◽  
Genitourinary Tract ◽  
Adrenal Tumors ◽  
Tumor Type ◽  
Kidney Tumors ◽  
Successful Work

Background: Genitourinary tract tumors in children are less common than in adults. Most of these tumors have different genetic backgrounds, clinical presentation, and oncologic behavior than their adult counterpart. As a result of low prevalence in children, some of the treatment approaches and recommendations are based on treatment experience in adult patients. However, thanks to scientific and technological development, survival rates have risen considerably. Objective: This paper presents a review of the principal features of the tumors involving the genitourinary tract in children and an update in genetic background, diagnosis, and treatment. Methods: A narrative review was performed on published literature about genitourinary tract tumors in pediatric patients. Papers presented in English and Spanish literature were reviewed. PubMed, Science Direct, and SciELO databases were used to collect information and present this article. Results: Kidney tumors are the most common type of genitourinary tumors in children. Among those, Wilms tumor represents the majority of cases and shows the successful work of clinical trial groups studying this tumor type. Other tumors involving the genitourinary tract in children include Rhabdomyosarcoma, Transitional cell carcinoma, Testicular, and Adrenal tumors. Conclusion: Genitourinary tract tumors in children represent significant morbidity and economic burden, so awareness in early diagnosis represents improvement in treatment, clinical and oncological outcomes.

A rare case of xanthogranulomatous pyelonephritis in a child with a doubling of the upper urinary tract (clinical case)

2019 ◽  
pp. 79-82 ◽  
Author(s):  
N. B. Kireeva ◽  
D. A. Alyautdinova ◽  
N. Yu. Orlinskaya
Keyword(s):  
Acute Pyelonephritis ◽  
Clinical Case ◽  
Wilms Tumor ◽  
Left Kidney ◽  
Kidney Injury ◽  
Xanthogranulomatous Pyelonephritis ◽  
Lower Segment ◽  
Morphological Examination ◽  
Kidney Tumors ◽  
Single Observation

Xanthogranuomatous pyelonephritis (CP) is an aggressive form of interstitial nephritis, including purulentdestructive and proliferative processes in the kidney to form granulomatous tissue. The difficulty of diagnosis of CP, low illumination of this problem in the literature are the reason for the publication of even a single observation. The exact diagnosis of the disease can be established only with morphological examination and, as a rule, after surgical treatment. The clinical observation of the treatment of the child 15 years xanthogranulomatous pyelonephritis, inpatient Nizhny Novgorod regional children's teaching hospital. A boy M. at the age of 15 years entered the clinic of acute pyelonephritis (fever up to 38–39°С, abdominal pain and left side). From anamnesis it is known that at the age of one year the child was diagnosed with recurrent leukocyturia. Urological examination revealed hydronephrosis of the lower half of the double kidney on the left. At the age of 7 he was operated on (due to negative dynamics), reconstructive surgery was performed – prilohanochnaya pyeloplasty on the lower segment of the double kidney on the left. In the subsequent exacerbations of pyelonephritis was not. 6 years after hepatitis and kidney injury (contusion), there was a significant increase in dilation of the abdominal system of the lower segment of the left kidney with exacerbation of secondary pyelonephritis. According to intravenous urography, MSCT, MRI (performed to exclude Wilms tumor), the absence of the function of the lower segment of the kidney with a sharp increase in its size was determined. After the preoperative examination, the left kidney was examined, in which the signs of xanthogranulomatous pyelonephritis (renal tissue of bright yellow color), lower left heminephroureterectomy were found, the Diagnosis was confirmed by morphological examination. In the postoperative period, a cyst of the lower pole of the kidney was formed, cured by percutaneous drainage, sclerosing under ultrasound control, followed by recovery. Thus, this clinical case demonstrates the complexity of the diagnosis of CP, which often occurs under the mask of a variety of diseases, including acute pyelonephritis and kidney tumors. Reliable diagnosis of xanthogranulomatous pyelonephritis is possible only with the help of histological examination of pathological tissues.

scholarly journals Prognostic factors and a new scoring system for survival of patients irradiated for bone metastases

BMC Cancer ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Dirk Rades ◽  
Rapha Haus ◽  
Steven E. Schild ◽  
Stefan Janssen
Keyword(s):  
Multivariate Analysis ◽  
Bone Metastases ◽  
Primary Tumor ◽  
Scoring System ◽  
Survival Rates ◽  
Karnofsky Performance Score ◽  
Tumor Type ◽  
Factor Scores ◽  
Survival Times ◽  
Primary Tumor Type

Abstract Background Personalized therapy for bone metastases should consider the patients’ remaining lifespan. Estimation of survival can be facilitated with scoring tools. A new tool was developed, specifically designed to estimate 12-month survival. Methods In 445 patients irradiated for bone metastases, radiotherapy regimen plus 13 factors (age, gender, Karnofsky performance score (KPS), primary tumor type, interval between cancer diagnosis and RT of bone metastases, visceral metastases, other (non-irradiated) bone metastases, sites of bone metastases, number of irradiated sites, pathological fracture, fractionation of RT, pre-RT surgery, pre-RT administration of bisphosphonates/denosumab, pre-RT systemic anticancer treatment) were retrospectively analyzed for survival. Factors achieving significance (p < 0.05) or borderline significance (p < 0.055) on multivariate analysis were used for the scoring system. Twelve-month survival rates were divided by 10 (factor scores); factor scores were summed for each patient (patient scores). Results On multivariate analysis, survival was significantly associated with KPS (hazard ratio (HR) 1.91, p < 0.001) and primary tumor type (HR 1.12, p < 0.001); age achieved borderline significance (HR 1.14, p = 0.054). These factors were used for the scoring tool. Patient scores ranged from 8 to 17 points. Three groups were designated: 8–9 (A), 10–14 (B) and 15–17 (C) points. Twelve-month survival rates were 9, 38 and 72% (p < 0.001); median survival times were 3, 8 and 24 months. Conclusions This new tool developed for patients irradiated for bone metastases at any site without spinal cord compression allows one to predict the survival of these patients and can aid physicians when assigning the treatment to individual patients.

scholarly journals PS1 - 176 Where Have All the Fat Cells Gone? A Comparative Analysis of Adiposity Patterns in Childhood Brain Tumor Survivors and Non-Cancer Controls

2016 ◽  
Vol 43 (S4) ◽  
pp. S11-S11 ◽  
Author(s):  
K.W. Wang ◽  
E. Kearsley ◽  
N. Falzone ◽  
A. Fleming ◽  
S. Burrow ◽  
...  
Keyword(s):  
Physical Activity ◽  
Brain Tumor ◽  
Brain Tumors ◽  
Survival Rates ◽  
Visceral Adiposity ◽  
Activity Levels ◽  
Tumor Type ◽  
Technological Advances ◽  
Cardiometabolic Disorders ◽  
Fried Foods

Brain tumors are the most common solid tumors in children in Canada. While technological advances have increased their survival rates, survivors of childhood brain tumors (SCBT) often develop obesity, which can reduce lifespan and quality of life. While adiposity is a known factor for cardiometabolic disorders in the general population, adiposity patterns in SCBT have not been determined. This study aims to investigate how adiposity patterns differ between SCBT and non-cancer controls, and how lifestyle and treatment factors may contribute to these patterns. Methods: Fifty-nine SCBT and 108 non-cancer controls were recruited from the clinics at McMaster Children’s Hospital. Sociodemographic and lifestyle details were collected using standardized tools to assess diet, physical activity, and sleep. Brain tumor type, location and treatment details were obtained from medical records. Total and visceral adiposity were determined by total fat mass (FM) as well as waist-to-hip (WHR) and waist-to-height ratio (WHTR). Results: SCBT have higher total and visceral adiposity, while BMI is similar to controls. Female SCBT who received radiotherapy and/or chemotherapy have higher adiposity. A dietary pattern of white bread and fried foods with low dark bread was positively associated with adiposity. Lower physical activity levels, but not sleep durations, were associated with higher adiposity. Conclusion: SCBT have higher visceral and total adiposity than non-cancer controls. Sex, chemoradiotherapy, high fat diet, and physical inactivity, can contribute to these adiposity patterns. These results provide multiple points of entry to design interventions that reduce adiposity, and may improve long-term outcomes in SCBT.

Wilm's tumor with pulmonary metastases at diagnosis: the significance of primary chemotherapy. International Society of Pediatric Oncology Nephroblastoma Trial and Study Committee.

1990 ◽  
Vol 8 (7) ◽  
pp. 1187-1190 ◽  
Author(s):  
J de Kraker ◽  
J Lemerle ◽  
P A Voûte ◽  
J M Zucker ◽  
M F Tournade ◽  
...  
Keyword(s):  
Pulmonary Metastases ◽  
Wilms Tumor ◽  
Survival Rates ◽  
Disease Free Survival ◽  
Local Therapy ◽  
Stage Iv ◽  
Actuarial Survival ◽  
Preoperative Ct ◽  
Lung Irradiation ◽  
Whole Lung Irradiation

Data from patients with pulmonary metastases (PM) from Wilms' tumor at diagnosis (stage IV) were collected from six European centers. All patients were pretreated with a chemotherapy (CT) regimen consisting of vincristine (VCR), dactinomycin (AD), and Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH). After nephrectomy, local therapy for residual pulmonary disease was considered to avoid whole-lung irradiation. Only four of 36 patients still had multiple inoperable metastases after preoperative CT. Thirty patients survived. Four of them were irradiated. Of the six patients who died, four died of PM, one died of abdominal recurrence, and one of therapy-related disease. Disease-free survival and actuarial survival rates are 83% with a mean follow-up of 4 years postnephrectomy.

Outcomes in low-risk babies treated with half-dose chemotherapy according to the Third National Wilms' Tumor Study.

1992 ◽  
Vol 10 (8) ◽  
pp. 1305-1309 ◽  
Author(s):  
B W Corn ◽  
J W Goldwein ◽  
I Evans ◽  
G J D'Angio
Keyword(s):  
Wilms Tumor ◽  
Survival Rates ◽  
Chemotherapeutic Agents ◽  
Low Risk ◽  
Hematologic Toxicity ◽  
Older Children ◽  
The Third ◽  
Favorable Histology ◽  
Tumor Study ◽  
Half Dose

PURPOSE To determine whether the 50% dose reduction of all chemotherapeutic agents recommended for babies (less than or equal to 12 months of age) by the Third National Wilms' Tumor Study (NWTS-3) produced acceptable toxicity without sacrificing any survival benefit. MATERIALS AND METHODS The 365 babies enrolled in NWTS-3 had tumors of varying histologies and stages. The present analysis was restricted to the 256 infants who had tumors of favorable histology, were free of metastasis at diagnosis, and received treatment according to NWTS-3 guidelines. RESULTS Despite the recommended attenuation of drug doses observed in 75% of the chemotherapy courses received, outcomes for these babies were comparable to those obtained in older children given full doses of chemotherapy. Four-year survival rates for 256 babies with stages I (n = 199), II (n = 38), and III (n = 19) favorable-histology tumors were 96%, 95%, and 90%, respectively. The figures for 498 stage I, 342 stage II, and 373 stage III older children with favorable-histology lesions were 92%, 94%, and 91% in that order. There were no deaths from hematologic toxicity or infection among babies who received half-dose chemotherapy. The death rates for their older NWTS-3 counterparts was 1%. CONCLUSION Less aggressive therapies advocated for babies in NWTS-3 provide acceptable levels of morbidity without compromising the excellent results previously reported for low-risk patients of all ages.

A 20-Year Prospective Study of Wilms Tumor and Other Kidney Tumors

2014 ◽  
Vol 36 (6) ◽  
pp. 445-450 ◽  
Author(s):  
Ching Ching Chan ◽  
Ka Fai To ◽  
Hui Leung Yuen ◽  
Alan Kwok Shing Chiang ◽  
Siu Cheung Ling ◽  
...  
Keyword(s):  
Prospective Study ◽  
Wilms Tumor ◽  
Kidney Tumors

scholarly journals Single cell derived mRNA signals across human kidney tumors

2020 ◽  
Author(s):  
Matthew D Young ◽  
Thomas J Mitchell ◽  
Lars Custers ◽  
Thanasis Margaritis ◽  
Francisco Morales ◽  
...  
Keyword(s):  
Single Cell ◽  
Human Cancer ◽  
Human Kidney ◽  
Cell Types ◽  
Renal Tumors ◽  
Tumor Type ◽  
Kidney Tumors ◽  
Cell Renal Cell Carcinoma ◽  
Cellular Origin ◽  
Normal Tissues

AbstractThe cellular transcriptome may provide clues into the differentiation state and origin of human cancer, as tumor cells may retain patterns of gene expression similar to the cell they derive from. Here, we studied the differentiation state and cellular origin of human kidney tumors, by assessing mRNA signals in 1,300 childhood and adult renal tumors, spanning seven different tumor types. Using single cell mRNA reference maps of normal tissues generated by the Human Cell Atlas project, we measured the abundance of reference “cellular signals” in each tumor. Quantifying global differentiation states, we found that, irrespective of tumor type, childhood tumors exhibited fetal cellular signals, thus replacing the long-held presumption of “fetalness” with a precise, quantitative readout of immaturity. By contrast, in adult cancers our assessment refuted the suggestion of dedifferentiation towards a fetal state in the overwhelming majority of cases, with the exception of lethal variants of clear cell renal cell carcinoma. Examining the specific cellular phenotype of each tumor type revealed an intimate connection between the different mesenchymal populations of the developing kidney and childhood renal tumors, whereas adult tumors mostly represented specific mature tubular cell types. RNA signals of each tumor type were remarkably uniform and specific, indicating a possible therapeutic and diagnostic utility. We demonstrated this utility with a case study of a cryptic renal tumor. Whilst not classifiable by clinical pathological work-up, mRNA signals revealed the diagnosis. Our findings provide a cellular definition of human renal tumors through an approach that is broadly applicable to human cancer.

Bilateral Wilms' tumor: long-term survival and some epidemiological features.

1989 ◽  
Vol 7 (3) ◽  
pp. 310-315 ◽  
Author(s):  
M J Coppes ◽  
J de Kraker ◽  
P J van Dijken ◽  
H J Perry ◽  
J F Delemarre ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Survival Rates ◽  
Tumor Stage ◽  
Advanced Tumor Stage ◽  
Older Children ◽  
Term Survival ◽  
Contralateral Kidney ◽  
Advanced Tumor ◽  
Bilateral Wilms Tumor

Sixty-seven children with a bilateral Wilms' tumor (BWT) who were registered to the International Society of Pediatric Oncology (SIOP) nephroblastoma trial and studies 1, 2, and 5, conducted between 1971 and 1980, were analyzed. The overall 10-year survival was 64%. While most deaths due to tumor occurred within 3 years after diagnosis of bilateral disease, five patients died after 3 years (20%), three with synchronous and two with metachronous BWT. The survival rates for the 42 children with synchronous BWT (follow-up time, 6 1/2 to 14 years) and the 25 children with metachronous BWT (follow-up time, 5 to 13 years) were 69% and 56%, respectively. Due to an improvement in the synchronous group, overall survival improved over the years: 47%, 72%, and 70%, in SIOP 1, 2, and 5, respectively. Age at diagnosis and most advanced tumor stage affected prognosis. Children presenting a tumor manifestation before the age of 2 years had better prognosis than older children. This difference is significant in synchronous BWT. Prognosis for children with local stage 1 or 2 was better than for those with local stage 3. Median age at initial presentation in BWT was lower than in unilateral nephroblastoma and lower in metachronous BWT than in synchronous BWT. Young children presenting with unilateral nephroblastoma should have a careful follow-up of the contralateral kidney for at least the next 3 1/2 years, as most contralateral tumors will develop during this period.

scholarly journals Activity of Vincristine and Irinotecan in Diffuse Anaplastic Wilms Tumor and Therapy Outcomes of Stage II to IV Disease: Results of the Children’s Oncology Group AREN0321 Study

2020 ◽  
Vol 38 (14) ◽  
pp. 1558-1568 ◽  
Author(s):  
Najat C. Daw ◽  
Yueh-Yun Chi ◽  
John A. Kalapurakal ◽  
Yeonil Kim ◽  
Fredric A. Hoffer ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Survival Rates ◽  
Stage Iv ◽  
High Response Rate ◽  
Stage Ii ◽  
Therapy Outcomes ◽  
Free Survival ◽  
Nonhematologic Toxicity ◽  
Measurable Disease ◽  
To Receive

PURPOSE AREN0321 evaluated the activity of vincristine and irinotecan (VI) in patients with newly diagnosed diffuse anaplastic Wilms tumor (DAWT) and whether a regimen containing carboplatin (regimen UH1) in addition to regimen I agents used in the National Wilms Tumor Study 5 (NWTS-5; vincristine, doxorubicin, cyclophosphamide, and etoposide plus radiotherapy) would improve patient outcomes. PATIENTS AND METHODS Patients with stage II to IV DAWT without measurable disease received regimen UH1. Patients with stage IV measurable disease were eligible to receive VI (vincristine, 1.5 mg/m2 per day intravenously on days 1 and 8; irinotecan, 20 mg/m2 per day intravenously on days 1-5 and 8-12 of a 21-day cycle) in an upfront window; those with complete (CR) or partial response (PR) had VI incorporated into regimen UH1 (regimen UH2). The study was designed to detect improvement in outcomes of patients with stage II to IV DAWT compared with historical controls treated with regimen I. RESULTS Sixty-six eligible patients were enrolled. Of 14 patients with stage IV measurable disease who received VI, 11 (79%) achieved CR (n = 1) or PR (n = 10) after 2 cycles. Doses of doxorubicin, cyclophosphamide, and etoposide were reduced midstudy because of nonhematologic toxicity. Four patients (6%) died as a result of toxicity. Four-year event-free survival, relapse-free survival, and overall survival rates were 67.7% (95% CI, 55.9% to 79.4%), 72.9% (95% CI, 61.5% to 84.4%), and 73.7% (95% CI, 62.7% to 84.8%), respectively, compared with 57.5% (95% CI, 47.6% to 67.4%; P = .26), 57.5% (95% CI, 47.6% to 67.4%; P = .048), and 59.2% (95% CI, 49.4% to 69.0%; P = .08), respectively, in NWTS-5. CONCLUSION VI produced a high response rate in patients with metastatic DAWT. AREN0321 treatment seemed to improve outcomes for patients with stage II to IV DAWT compared with NWTS-5, but with increased toxicity. The UH2 regimen warrants further investigation with modifications to reduce toxicity.

The rise in appendix cancer incidence: 2000-2009.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e12506-e12506
Author(s):  
Schelomo Marmor ◽  
Pamela R. Portschy ◽  
Todd M. Tuttle ◽  
Beth A. Virnig
Keyword(s):  
Tumor Size ◽  
Survival Rates ◽  
Signet Ring Cell Carcinoma ◽  
Signet Ring Cell ◽  
Tumor Type ◽  
Appendiceal Cancer ◽  
Distant Disease ◽  
Appendix Cancer ◽  
Signet Ring ◽  
Ring Cell

e12506 Background: Cancer of the appendix is a rare and potentially aggressive malignancy. The objectives of this study were to characterize secular demographic patterns of disease and to determine survival by using a population-based data source. Methods: Using the Surveillance Epidemiology and End Results database we identified all appendiceal neoplasms reported to the registry between 2000 and 2009.Tumors were classified as carcinoid, adenocarcinoma, mucinous and signet ring cell carcinoma. We compared overall incidence, age distribution by tumor type and overall survival rates by extent of disease at diagnosis. To assess whether the incidence rate, tumor size, and cancer stage changed over time, the Cochrane-Armitage trend test was used. Logistic regression models were used to determine the odds of distant disease at diagnosis and Cox proportional-hazards modeling was used to identify the factors associated with overall survival rates. Results: We identified 4,765 patients with appendix cancer. The incidence of appendiceal cancer increased by 54% from 2000 (0.63 per 100,000) to 2009 (0.97 per 100,000). Incidence rates increased across all tumor types, stages, age groups and gender. The most common malignancies were mucinous (38%), followed by carcinoids (28%) adenocarcinoma (27%), and signet ring cell carcinoma (7%). Distant stage at diagnosis was most common among, signet ring cell tumors (60%). Larger tumor size and older patient age were significantly associated with higher relative odds of distant disease at diagnosis (P < .0001). Tumor type, older age, black race, males, tumor size of 2 cm and greater, cancer stage and registry were significantly associated with higher relative hazard of death (P< .0001). Mean 5-year survival was 55% for carcinoid patients, 49% for adenocarcinoma, 50% for mucinous and 38% for the signet ring cell cohort. Conclusions: Although appendiceal cancer is rare, the incidence increased significantly in the U.S. from 2000 to 2009. The cause of this trend is not obvious. We did not see increases differentially associated with stage, histology, registry or demographic characteristics. Further investigation is needed to examine factors underlying this increase. This research was generously supported by the Betti Boers Maloney Appendix Cancer Research Fund at the University of Minnesota Foundation.

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