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1.
J Urol ; 209(6): 1186-1193, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36821137

RESUMO

PURPOSE: Although Children's Oncology Group renal tumor protocols mandate lymph node sampling during extirpative surgery for pediatric renal tumors, lymph node sampling is often omitted or low yield. Concerns over morbidity associated with extended lymph node sampling have led to hesitancy in adopting a formal lymph node sampling template. We hypothesized that complications in children undergoing lymph node sampling for renal tumors would be rare, and not associated with the number of lymph nodes sampled. MATERIALS AND METHODS: A single-institution, retrospective review of patients aged 0-18 years undergoing extirpative renal surgery with lymph node sampling for a suspected malignancy between 2005 and 2019 was performed. Patients with 0 or an unknown number of lymph nodes sampled or <150 days of follow-up were excluded. A "clinically significant" complication was defined as any Clavien complication ≥III, small-bowel obstruction, chylous ascites, organ injury, or wound infection. The number of lymph nodes sampled and its influence on the odds of experiencing a clinically significant complication was examined. RESULTS: A total of 144 patients met inclusion criteria. Median patient age was 38 months. Twenty-one patients (15%) had a clinically significant complication, the most common of which was ileus/small-bowel obstruction (n=16). In a multivariable analysis, increased lymph node yield was not found to influence the odds of experiencing a clinically significant complication (P = .6). CONCLUSIONS: In this cohort, there was no statistically significant difference in clinically significant complications in patients who underwent more extensive lymph node sampling during surgery for a suspected malignant pediatric renal tumor. Future studies on protocol adherence, staging accuracy, and survival trends using a lymph node sampling template in these patients should be performed.


Assuntos
Neoplasias Renais , Humanos , Criança , Neoplasias Renais/patologia , Linfonodos/cirurgia , Linfonodos/patologia , Excisão de Linfonodo/efeitos adversos , Excisão de Linfonodo/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Estudos Retrospectivos , Estadiamento de Neoplasias
2.
J Urol ; 209(3): 582-590, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36445021

RESUMO

PURPOSE: The majority of children with unilateral renal masses suspicious for malignancy undergo radical nephrectomy, while nephron-sparing surgery is reserved for select cases. We investigated the impact of tumor size on the probability of histology. We hypothesized that pediatric small renal masses are more likely benign or non-Wilms tumor, thus potentially appropriate for nephron-sparing surgery. MATERIALS AND METHODS: The SEER (Surveillance, Epidemiology, and End Results) database was analyzed for patients aged 0-18 years diagnosed with a unilateral renal mass from 2000-2016. Statistical analysis was performed to help determine a tumor size cut point to predict Wilms tumor and assess the predictive value of tumor size on Wilms tumor histology. Additionally, a retrospective review was performed of patients 0-18 years old who underwent surgery for a unilateral renal mass at a single institution from 2005-2019. Statistical analysis was performed to assess the predictive value of tumor size on final histology. RESULTS: From the SEER analysis, 2,016 patients were included. A total of 1,672 tumors (82.9%) were Wilms tumor. Analysis revealed 4 cm to be a suitable cut point to distinguish non-Wilms tumor. Tumors ≥4 cm were more likely Wilms tumor (OR 2.67, P ≤ .001), but this was driven by the statistical significance in children 5-9 years old. From the institutional analysis, 134 patients were included. Ninety-seven tumors (72.3%) were Wilms tumor. Tumors ≥4 cm had higher odds of being Wilms tumor (OR 30.85, P = .001), malignant (OR 6.75, P = .005), and having radical nephrectomy-appropriate histology (OR 46.79, P < .001). CONCLUSIONS: The probability that a pediatric unilateral renal mass is Wilms tumor increases with tumor size. Four centimeters is a logical cut point to start the conversation around defining pediatric small renal masses and may help predict nephron-sparing surgery-appropriate histology.


Assuntos
Neoplasias Renais , Tumor de Wilms , Criança , Humanos , Recém-Nascido , Lactente , Pré-Escolar , Adolescente , Neoplasias Renais/cirurgia , Neoplasias Renais/patologia , Néfrons/cirurgia , Néfrons/patologia , Tumor de Wilms/cirurgia , Nefrectomia/métodos , Estudos Retrospectivos
3.
Am J Cardiol ; 190: 48-53, 2023 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-36563458

RESUMO

The American College of Cardiology and the American Heart Association guidelines recommend treatment of patients with severe hypercholesterolemia (low-density lipoprotein cholesterol [LDL-C] ≥190 mg/100 ml) with a high-intensity statin. However, atherosclerotic cardiovascular disease (ASCVD) risk, even among those with severe hypercholesterolemia, is heterogeneous, and coronary artery calcium (CAC) scoring may be used to clarify risk. We sought to evaluate CAC in patients with severe hypercholesterolemia and measure its impact on real-world statin prescriptions. We identified patients with at least 1 LDL-C ≥190 mg100 ml who had a CAC scoring in the Community Benefit of No-Charge Calcium Score Screening Program (CLARIFY) study (NCT04075162) between 2014 and 2020. We explored the CAC distribution, factors associated with CAC >0, and ASCVD risk (myocardial infarction, stroke, revascularization, death). A total of 1,904 patients (1.257 women, aged 57.8 ± 9.3 years) with severe hypercholesterolemia were included. LDL-C ranged from 190 to 524 mg100 ml (mean 215.5 ± 27 mg100 ml). A total of 864 patients (45.4%) had CAC = 0 and 1,561 (82%) had CAC <100. In patients with LDL-C ≥250 mg100 ml, 67 (36.6%) had CAC = 0. Age, male gender, smoking, diabetes, systolic blood pressure, and obesity (ps ≤0.001) were associated with CAC >0. In patients with LDL-C ≥190 mg100 ml, CAC was associated with a higher risk for ASCVD events (CAC ≥100 vs CAC <100, hazard ratio 3.57 [1.81 to 7.04], p <0.001). A higher CAC category was associated with increased statin use after CAC scoring (p <0.001). In patients with severe hypercholesterolemia, 45% had CAC = 0, which was associated with a significantly lower ASCVD risk. CAC was associated with statin prescription and cholesterol lowering. In conclusion, CAC scoring may be used to clarify ASCVD risk in this heterogeneous population with severe hypercholesterolemia.


Assuntos
Aterosclerose , Doença da Artéria Coronariana , Inibidores de Hidroximetilglutaril-CoA Redutases , Hipercolesterolemia , Humanos , Masculino , Feminino , Estados Unidos/epidemiologia , Hipercolesterolemia/complicações , Hipercolesterolemia/tratamento farmacológico , Hipercolesterolemia/epidemiologia , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Doença da Artéria Coronariana/complicações , Cálcio , LDL-Colesterol , Medição de Risco , Aterosclerose/epidemiologia , Colesterol , Fatores de Risco
4.
Intensive Care Med ; 48(9): 1133-1143, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35831640

RESUMO

PURPOSE: Early accurate diagnosis of infection ± organ dysfunction (sepsis) remains a major challenge in clinical practice. Utilizing effective biomarkers to identify infection and impending organ dysfunction before the onset of clinical signs and symptoms would enable earlier investigation and intervention. To our knowledge, no prior study has specifically examined the possibility of pre-symptomatic detection of sepsis. METHODS: Blood samples and clinical/laboratory data were collected daily from 4385 patients undergoing elective surgery. An adjudication panel identified 154 patients with definite postoperative infection, of whom 98 developed sepsis. Transcriptomic profiling and subsequent RT-qPCR were undertaken on sequential blood samples taken postoperatively from these patients in the three days prior to the onset of symptoms. Comparison was made against postoperative day-, age-, sex- and procedure- matched patients who had an uncomplicated recovery (n =151) or postoperative inflammation without infection (n =148). RESULTS: Specific gene signatures optimized to predict infection or sepsis in the three days prior to clinical presentation were identified in initial discovery cohorts. Subsequent classification using machine learning with cross-validation with separate patient cohorts and their matched controls gave high Area Under the Receiver Operator Curve (AUC) values. These allowed discrimination of infection from uncomplicated recovery (AUC 0.871), infectious from non-infectious systemic inflammation (0.897), sepsis from other postoperative presentations (0.843), and sepsis from uncomplicated infection (0.703). CONCLUSION: Host biomarker signatures may be able to identify postoperative infection or sepsis up to three days in advance of clinical recognition. If validated in future studies, these signatures offer potential diagnostic utility for postoperative management of deteriorating or high-risk surgical patients and, potentially, other patient populations.


Assuntos
Sepse , Transcriptoma , Biomarcadores , Humanos , Inflamação/complicações , Insuficiência de Múltiplos Órgãos , Complicações Pós-Operatórias/diagnóstico
5.
Clin Med (Lond) ; 21(1): 73-75, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33479071

RESUMO

Hypercalcaemia is a common electrolyte abnormality with 90% of cases due to either primary hyperparathyroidism or malignancy. Other causes of hypercalcaemia often require careful consideration. We describe an approach to the assessment of hypercalcaemia, particularly where preliminary tests are inconclusive.This approach is illustrated by a case which posed a diagnostic challenge: a patient with significant hypercalcaemia due to acute atypical isolated sarcoid myositis. This case highlights an under-recognised clinical syndrome with distinct biochemical and radiological findings.


Assuntos
Hipercalcemia , Hiperparatireoidismo Primário , Neoplasias , Humanos , Hipercalcemia/diagnóstico , Hipercalcemia/etiologia , Hiperparatireoidismo Primário/complicações , Hiperparatireoidismo Primário/diagnóstico
6.
Urol Pract ; 8(6): 682-691, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37145509

RESUMO

INTRODUCTION: Patients with differences in sex development represent a complex pediatric population with varying psychosocial and medical needs. Due to the complexity of care, families likely benefit from multidisciplinary care allowing for coordination of psychosocial and health services. Unfortunately, there are few data on the experiences of families of patients with differences of sex development in a multidisciplinary setting, such as their satisfaction with health care or their level of shared decision making. METHODS: Patients and guardians seen in a multidisciplinary, differences in sex development clinic were asked to participate in an anonymous online survey of their satisfaction with health care delivery (Patient Satisfaction Questionnaire Short Form) and involvement in shared decision making (Shared Decision Making Questionnaire). Welch's t-test was used to compare mean survey scores to historical and contemporary control populations. RESULTS: In all, 22 guardians and 1 young adult patient completed surveys. Median patient age was 36 months. Patient diagnoses were diverse, with the most common diagnosis beings 46, XY DSD (34.8%). At the time of their clinic visit, 7 patients had undergone surgery. Mean satisfaction scores were higher than a primary care population and comparable to patients and patients' caregivers with chronic medical conditions. The mean shared decision making score was well above adult populations and similar to caregiver reports in pediatric outpatient clinics. CONCLUSIONS: Despite the complex treatment associated with differences of sex development, families are very satisfied with the quality of care they receive in a multidisciplinary setting and appear to feel personally invested in the decision making process.

7.
Urology ; 149: 199-205, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33189736

RESUMO

OBJECTIVE: To describe 5 cases with complete urinary bladder duplication, their associated conditions, and their respective treatment. Urinary bladder duplication is an extremely rare congenital anomaly of the urinary system. So far about 70 cases have been published in the English literature, most of them as case reports and a few case series. METHODS AND RESULTS: All consecutive patients with bladder duplication treated at our institution between 2000 and 2015 were included. Patient records were retrospectively analyzed, and 5 patients with urinary bladder duplication were identified (see Summary Figure). Two patients were male. All duplications were recognized by health care providers. In 1 case recognition was prenatal (MRI in utero at 22 weeks of gestation), the latest recognition was at 12 months of age. A voiding cystourethrography was performed in 4 patients to confirm the diagnosis. In 4 patients the bladder duplication could be classified according to Abrahamson with 3 complete reduplications and one complete sagittal septum. All patients suffered from associated congenital diseases, but only one patient had urinary tract infections. Surgical treatment was only performed in one patient. Median follow-up was 34 months. DISCUSSION: Urinary bladder duplications reflect extremely seldom disorders that are almost always associated with other congenital anomalies. Treatment depends on patients' symptoms and associated conditions and hence needs to be individualized to each patient.


Assuntos
Bexiga Urinária/anormalidades , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/terapia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
8.
J Pediatr Urol ; 16(5): 576-582, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32564942

RESUMO

OBJECTIVE: To describe the rates of GCNIS-free and GCT-free pathology based on age at gonadal surgery and to describe long-term oncologic outcomes in patients with DSD who have GCNIS or GCT at the time of gonadal surgery. STUDY DESIGN: A systematic review was conducted using MEDLINE to identify patients with DSD who underwent gonadal surgery. DSD diagnoses were stratified based on malignancy risk. GCNIS/GCT and GCT-free survival by age of gonadal surgery, RFS and OS were calculated using the Kaplan-Meier method, with groups compared using log-rank testing. RESULTS: 386 articles from 1951 to 2017 were included (2037 patients). Median age at gonadal surgery was 17 years (y) (IQR 11-20), median follow-up was 60 months (m) (IQR 30-68.1). GCNIS/GCT- and GCT-free survival at the time of gonadal surgery was lowest for those in the high/intermediate risk group (p < 0.001) but decreased sharply around age 15y, regardless of risk category. 5y RFS and OS was similar for those with no GCNIS/GCT and GCNIS and was worse for those with GCT (p < 0.001). DISCUSSION: When patients undergo gonadal surgery, regardless of indication (i.e. prophylactic vs. tumor), it appears that GCTs are more commonly found when surgery is done around age 15 y or older, despite risk category. This is similar to ovarian and testicular GCTs. Patients with GCNIS can be reassured that long-term oncologic outcomes are excellent. While RFS and OS for GCTs are not as good as for ovarian and testicular GCTs (95%), they are still >80%. This similar trend was found in a COG review of 9 patients with DSD and ovarian GCT. There were several limitations to this study. This is a retrospective analysis that included aa wide time frame of publications. The indication for surgical intervention was not addressed in the majority of publications. Thus these data provide pathologic outcomes based on age at gonadal surgery rather than the age at which GCNIS/GCT develops over a lifetime, if at all. CONCLUSIONS: The risk of GCNIS or GCT at the time of gonadal surgery appears to increase with age, accelerating between 15 and 20y regardless of risk category. 5y RFS and OS for those with GCNIS is equivalent to those without GCNIS/GCT but is worse for those with GCT. These data may be used when counseling families on timing of gonadal surgery and quantification of outcomes should GCNIS or malignancy be identified.


Assuntos
Neoplasias Embrionárias de Células Germinativas , Neoplasias Testiculares , Adolescente , Gônadas , Humanos , Masculino , Neoplasias Embrionárias de Células Germinativas/epidemiologia , Neoplasias Embrionárias de Células Germinativas/cirurgia , Estudos Retrospectivos , Desenvolvimento Sexual , Neoplasias Testiculares/epidemiologia , Neoplasias Testiculares/cirurgia
9.
J Pediatr Surg ; 55(7): 1334-1338, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31128844

RESUMO

BACKGROUND/PURPOSE: While many children with renal tumors require long term venous access (VA) for adjuvant chemotherapy, certainly not all do. This study develops and tests a VA decision tree (DT) to direct the placement of VA in patients with renal tumors. METHODS: Utilizing data readily available at surgery a VADT was developed. The VADT was tested retrospectively by 2 independent reviewers on a historic cohort. The ability of the VADT to appropriately select which patients would benefit from VA placement was tested. RESULTS: 160 patients underwent renal tumor surgery between 2005 and 2018. 70 (43.8%) patients met study criteria with median age of 45.1 months (range 1.1-224); 73% required VA. Using the VADT, VA placement was "needed" in 67.1% of patients and "deferred" in 32.9%. Interrater reliability was very high (kappa = 0.97, 95% CI 0.91-1, p < 0.001). The sensitivity and specificity of the VADT to correctly decide on VA placement were 0.92 (0.8-0.98) and 1 (0.79-1). Using the VADT, no patient would have undergone unnecessary VA placement. In reality, 4.3% of patients had an unnecessary VA placed which required a subsequent removal. CONCLUSIONS: These preliminary data support the continued study of this VADT to guide intraoperative decisions regarding VA placement in patients with renal tumors. LEVEL OF EVIDENCE: III - Study of diagnostic test.


Assuntos
Cateterismo , Neoplasias Renais/tratamento farmacológico , Neoplasias Renais/cirurgia , Adolescente , Criança , Pré-Escolar , Árvores de Decisões , Humanos , Lactente , Rim/cirurgia , Estudos Retrospectivos
10.
Urology ; 137: 168-172, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31794814

RESUMO

Neurofibromatosis-1 has a known increased risk of malignancy with rhabdomyosarcoma occurring in up to 6% of patients. Here we report on an 8-year-old male with a history of Neurofibromatosis-1 and previously treated stage 3, group III bladder/prostate embryonal rhabdomyosarcoma (diagnosed at 18 months old) who presented with penile swelling concerning for priapism. Imaging and subsequent biopsy confirmed embryonal rhabdomyosarcoma of the penile corporal bodies. Penile rhabdomyosarcoma is exceedingly rare, with less than 15 case reports in the literature. Our patient received chemoradiation per D9803 with organ preserving local control and is doing well 3 months after treatment.


Assuntos
Quimiorradioterapia/métodos , Recidiva Local de Neoplasia , Neurofibromatose 1 , Neoplasias Penianas , Priapismo/diagnóstico , Neoplasias da Próstata , Rabdomiossarcoma Embrionário , Neoplasias da Bexiga Urinária , Biópsia/métodos , Criança , Diagnóstico Diferencial , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Neurofibromatose 1/patologia , Neurofibromatose 1/terapia , Neoplasias Penianas/diagnóstico , Neoplasias Penianas/tratamento farmacológico , Neoplasias Penianas/patologia , Neoplasias Penianas/radioterapia , Pênis/diagnóstico por imagem , Pênis/patologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Neoplasias da Próstata/patologia , Neoplasias da Próstata/terapia , Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/terapia , Resultado do Tratamento , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/terapia
11.
J Pediatr Urol ; 16(1): 81-88, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31791906

RESUMO

BACKGROUND: Lymph node (LN) involvement is an important prognostic indicator for patients with Wilms tumor (WT), and there have been previous reports of utilizing LN density (LND = positive LN/LNs examined) as an advanced metric to risk-stratify patients with WT. OBJECTIVE: The purpose of this study was to describe patient characteristics that affect LN yield and assess the effect of LND on the overall survival (OS) in patients with WT, with the expectation that patients with LNDs above a critical cut-point would demonstrate lower OS. STUDY DESIGN: The Surveillance, Epidemiology, and End Result (SEER) database was queried for all patients diagnosed with unilateral WT from 2004 to 2015. Patient and disease characteristics were collected, and Poisson regression was used to identify characteristics correlated with LN yield. LND was calculated for LN-positive patients, and multivariable survival analysis was performed, including patient demographics and LND as variables. RESULTS: 1489 patients with unilateral WT were identified for analysis, 231 (15.51%) of whom were LN-positive. Median patient age at diagnosis was three years (IQR 1-5). On Poisson regression, the year of diagnosis, patient age, tumor size and laterality, and stage were found to impact LN yield. For patients with positive LNs, five-year OS of patients with LNDs above 0.4 was worse than those below 0.4 (76.1% vs 89.6%, p = 0.041). On multivariable analysis, tumor size and LND remained significant predictors of OS. DISCUSSION: Administrative databases such as SEER provide an excellent resource for studying conditions where large patient numbers for analysis are difficult to obtain. Unfortunately, the SEER database is unable to account for every factor that could affect LN sampling patterns. Additionally, favorable vs unfavorable histology is not available in SEER, and SEER utilizes its own staging system, which makes comparison to Children's Oncology Group staging difficult. Despite these limitations, the findings of this study are similar to those previously published using administrative databases analyzing LN sampling patterns and the effect of LND on OS in WT. CONCLUSIONS: Analysis of the SEER database confirms that there are several patient- and disease-specific factors that affect the number of LNs sampled during nephrectomy for WT, and that LND may be a predictor of OS. These findings highlight the need for standardization of LN sampling patterns for pediatric renal tumors and support the investigation of LND in future studies to further risk-stratify WT patients to tailor therapy intensity.


Assuntos
Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Linfonodos/patologia , Manejo de Espécimes/métodos , Tumor de Wilms/mortalidade , Tumor de Wilms/patologia , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Lactente , Masculino , Programa de SEER , Análise de Sobrevida
12.
Urology ; 137: 164-167, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31733273

RESUMO

Pediatric extrarenal malignant rhabdoid tumors (MRTs) are rare, aggressive tumors with a poor prognosis (20% 5-year survival). There are currently fewer than 10 published case reports of primary MRT of the bladder. We report the case of an 18-month-old female with an isolated MRT of the bladder which was initially misdiagnosed as an inflammatory myofibroblastic tumor on biopsy. We review the history, tumor biology, histology, and current management of extrarenal MRT, along with lessons learned from the difficulty with the patient's initial diagnosis.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Biópsia/métodos , Cistectomia/métodos , Neoplasias de Tecido Muscular , Radioterapia/métodos , Tumor Rabdoide , Neoplasias da Bexiga Urinária , Diagnóstico Diferencial , Erros de Diagnóstico , Feminino , Humanos , Hidronefrose/diagnóstico , Hidronefrose/etiologia , Lactente , Neoplasias de Tecido Muscular/diagnóstico , Neoplasias de Tecido Muscular/patologia , Prognóstico , Tumor Rabdoide/complicações , Tumor Rabdoide/diagnóstico , Tumor Rabdoide/patologia , Tumor Rabdoide/cirurgia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Ultrassonografia/métodos , Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/complicações , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias da Bexiga Urinária/patologia , Neoplasias da Bexiga Urinária/cirurgia
13.
Urology ; 133: 243-244, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31404584

RESUMO

OBJECTIVE: To present the surgical approach to a multifocal Wilms tumor found on screening ultrasound in an asymptomatic 2-year-old female. MATERIALS: A 2-year-old female with hemihypertrophy underwent screening imaging every 3 months with renal ultrasound. A solitary, incidental renal mass was detected. Physical exam was unremarkable except for left leg hemihypertrophy. Laboratory workup was largely normal other than an elevated lactate dehydrogenase. Staging imaging revealed multiple masses on the right kidney and a normal left kidney. There were no distant metastases. The most likely diagnosis was Wilms tumor. After receiving chemotherapy for 6 weeks, imaging revealed an excellent response to chemotherapy and surgery was performed. RESULTS: The patient underwent open partial nephrectomy of the 3 tumors on the right. Prior to beginning, cystoscopy and ureteral stent placement was performed as it was felt the collecting system would likely be entered during resection. The renal hilum was not clamped throughout this resection and manual parenchymal compression was used to minimize global ischemia. Retroperitoneal lymph node dissection was also performed. The patient recovered well and was discharged home 5 days after surgery. Her stent was removed at home without complications. Final pathology revealed nephrogenic rests with all 7 lymph nodes were negative for malignancy. CONCLUSION: Management of multifocal, unilateral Wilms tumor with open partial nephrectomy after neoadjuvant chemotherapy is an important part of protocol management for patients with Wilms tumor with predisposition syndromes.


Assuntos
Neoplasias Renais/cirurgia , Nefrectomia/métodos , Tumor de Wilms/cirurgia , Pré-Escolar , Feminino , Humanos , Hiperplasia/complicações , Neoplasias Renais/complicações , Neoplasias Renais/patologia , Tumor de Wilms/complicações , Tumor de Wilms/patologia
14.
Urology ; 133: 241-242, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31419431

RESUMO

OBJECTIVE: To present the surgical approach to a cystic renal mass suspicious for malignancy in a 22-month-old female. MATERIALS: The patient was a healthy female was found to have an abdominal mass by her parents. Her mother underwent a nephrectomy as a child for unknown pathology. Physical examination was otherwise unremarkable and laboratory workup was normal. Imaging workup revealed a large cystic renal mass suspicious for malignancy. Surgical resection was planned. RESULTS: A nonsyndromic 22-month-old female underwent open radical nephrectomy on the left with regional lymph node dissection. A transverse abdominal incision was used, followed by medial reflection of the colon and complete renal mobilization. The distal ureter and renal vasculature were identified and controlled and the kidney removed. A retractor was then assembled and regional lymph node dissection performed. Final pathology was cystic nephroma with 10 benign lymph nodes. The patient recovered well and was discharged home 3 days after surgery. CONCLUSION: Open radical nephrectomy is an important part of protocol management for patients with renal masses suspicious for malignancy.


Assuntos
Neoplasias Renais/cirurgia , Nefrectomia/métodos , Feminino , Humanos , Lactente
15.
Pediatr Surg Int ; 35(9): 1005-1008, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31278478

RESUMO

PURPOSE: The literature about ectopic ureters in anorectal malformations is limited. Repair of an anorectal malformation may require dissection near the normal or abnormal insertion of the ureters. Knowledge of the presence and location of ectopic ureters may prevent intraoperative injury. We aim to describe the incidence and location of ectopic ureters in patients with anorectal malformations and to characterize associated renal anomalies. METHODS: This is an IRB-approved retrospective study of patients with anorectal malformations and ectopic ureters identified in our colorectal database. RESULTS: Of 2283 patients with anorectal malformation, 79 (3.5%) had ectopic ureter(s). Of those, 29% had bilateral ectopic ureters. Nearly all (87%) of bilateral ectopic ureters occurred in females. Ectopic ureters most commonly inserted into the bladder neck (33%), vagina (15%), or urethra (13%). Renal dysfunction was noted in a high proportion of patients. The majority (59%) of ectopic ureters were associated with dysfunction of the ipsilateral kidney. Interestingly, 29% of patients with unilateral ureteral ectopia had an abnormal contralateral kidney. Only 22 patients (28%) had two normal kidneys, and 5 (6%) had documented renal failure with 2 (2.5%) requiring renal transplant. This compares to a transplant rate of 0.6% among anorectal malformation patients without ectopic ureter. CONCLUSIONS: The incidence of ectopic ureter is 3.5% among anorectal malformation patients. Cloaca and recto-bladder neck fistula are the types of anorectal malformation with higher incidence. Ureteral ectopia seems to confer an increased risk of renal failure. Identifying ectopic ureters is important for surgical planning and monitoring renal function vigilantly.


Assuntos
Malformações Anorretais/cirurgia , Ureter/anormalidades , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Rim/anormalidades , Masculino , Estudos Retrospectivos
16.
Urology ; 130: 151-154, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30807774

RESUMO

Due to the rarity of Wilms tumor (WT) and the relative urgency with which pediatric renal tumors are treated, there is little reported data on the natural history and growth of WTs. Historical reports of estimated doubling times of WTs were based on time to disease recurrence after initial diagnosis and treatment, and were published before the current advancements in molecular biomarker testing. We compare 2 cases of WT with sequential imaging, and postulate how the growth parameters of these tumors may be associated with differing chromosomal traits.


Assuntos
Neoplasias Renais/patologia , Tumor de Wilms/patologia , Adolescente , Pré-Escolar , Humanos , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/cirurgia , Masculino , Fatores de Tempo , Carga Tumoral , Tumor de Wilms/diagnóstico por imagem , Tumor de Wilms/cirurgia
17.
Urology ; 129: 200-205, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30597169

RESUMO

Adult Wilms tumor (WT) is a well-known, albeit rare entity and has historically been associated with worse overall clinical outcomes when compared to younger patients. Because WT is uncommon in adult patients, it is often misdiagnosed and treated off standardized pediatric protocols. WT associated with pregnancy is even more rare, and there is not a standardized approach to this small subset of patients. We present a case of an adult WT discovered and managed during the perinatal period and review prior published cases.


Assuntos
Neoplasias Renais/diagnóstico , Complicações Neoplásicas na Gravidez , Tumor de Wilms/diagnóstico , Feminino , Humanos , Recém-Nascido , Neoplasias Renais/cirurgia , Imageamento por Ressonância Magnética , Nefrectomia , Gravidez , Resultado da Gravidez , Tomografia Computadorizada por Raios X , Tumor de Wilms/cirurgia , Adulto Jovem
18.
Urology ; 119: 140-142, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29545044

RESUMO

Pheochromocytoma is a rare chromaffin cell tumor that may be associated with a genetic predisposition, such as von Hippel-Lindau (VHL) disease. VHL is an autosomal dominant disorder that is characterized by a predisposition to multiple tumors, including retinal and central nervous system hemangioblastomas, renal cell carcinoma, and pheochromocytomas. The classic presentation of pheochromocytoma is episodic hypertension, headaches, palpitations, and diaphoresis. In the pediatric population, 40% of pheochromocytomas have a hereditary basis. We present a case of metastatic pheochromocytoma in a child with VHL and discuss the relevant current medical literature.


Assuntos
Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias Pulmonares/secundário , Feocromocitoma/secundário , Doença de von Hippel-Lindau , Neoplasias das Glândulas Suprarrenais/etiologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Doenças Assintomáticas , Criança , Humanos , Neoplasias Pulmonares/etiologia , Neoplasias Pulmonares/cirurgia , Masculino , Feocromocitoma/etiologia , Feocromocitoma/cirurgia , Doença de von Hippel-Lindau/complicações
19.
BMC Urol ; 17(1): 26, 2017 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-28381270

RESUMO

BACKGROUND: Ureteral herniations are a rare occurrence, generally found incidentally on cross sectional imaging or during surgical intervention for unrelated processes. Several locations of ureteral herniations can occur including the inguinal, femoral, sciatic, obturator, and thoracic regions. While few reports of ureteral hernias are reported in the literature overall, the vast majority of those reported are inguinoscrotal herniations found during evaluation and treatment of inguinal hernias. Pelvic outlet ureteral herniations intrinsically are more common secondary to their dependent locations. Intrathoracic ureteral herniations through diaphragmatic defects are an exceptionally rare subset of ureteral herniations and have only been described sparingly. Fewer than ten case reports of diaphramatic ureteral herniations have been reported and none have described both cystoscopic management and open reconstruction. CASE PRESENTATION: We report the case of a 81 year old female with flank pain who was found to have idiopathic diaphragmatic hernia with incarcerated proximal ureter. She had no prior injury or surgery that explained her clinical presentation. She was initially observed and then managed conservatively with ureteral stent exchanges. Ultimately she underwent open surgical repair of her diaphragmatic hernia, reduction, resection and anastomosis of redundant proximal incarcerated ureteral segment, and nephropexy for a hypermobile right renal unit. This case report illustrates the pre- and post-operative imaging studies of a very rare intrathoracic ureteral herniation as well as surgical approach to repair. CONCLUSION: A herniated ureter is a potential source of serious renal and ureteral complications. The thoracic herniation of ureter is the rarest of the ureteral herniations. When discovered, they should be managed to preserve renal function and prevent strangulation of the affected segment of ureter. This case report documents the treatment of a thoracic ureteral herniation with observation, conservative endoscopic management, and finally open surgical reconstruction.


Assuntos
Hérnia Diafragmática/cirurgia , Ureter/cirurgia , Doenças Ureterais/cirurgia , Idoso de 80 Anos ou mais , Endoscopia , Feminino , Hérnia Diafragmática/complicações , Hérnia Diafragmática/diagnóstico por imagem , Humanos , Tomografia Computadorizada por Raios X , Ureter/diagnóstico por imagem , Doenças Ureterais/diagnóstico por imagem , Obstrução Ureteral/diagnóstico por imagem , Obstrução Ureteral/etiologia
20.
Indian J Surg Oncol ; 8(3): 403-406, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30429637

RESUMO

Renal cell carcinoma accounts for 3-4% of adult malignant neoplasms and over 65,000 new cases of kidney cancer were diagnosed in the USA in 2013 [1, 2]. Widespread use of abdominal imaging is leading to an increased incidence in the detection of small renal masses (SRMs) among other causes [1-4]. In light of recent literature on the role of percutaneous renal mass biopsy and retrospective data analysis, surveillance for renal masses ≤4 cm is likely to become more common especially in patients with less aggressive pathology, advanced age and multiple medical comorbidities.

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