Wilms tumor (nephroblastoma) in the adult patient: clinical and radiologic manifestations (original) (raw)
Related papers
A rare case of adult Wilms tumor: case report and literature review from a tertiary centre
International Surgery Journal, 2021
Nephroblastoma (Wilms’ tumour) in adult population is extremely rarefied scenario and has a poorer prognosis than paediatric Wilms’ tumour with an incidence of about 0.2 per million per year in the USA and Europe. Clinical presentation is also distinct between children and adults. In adults, manifestation is usually hematuria and abdominal pain while paediatric cases frequently present with an asymptomatic abdominal lump. It is arduous to demarcate adult Wilms’ tumour from renal cell carcinoma based on radiology alone. The final diagnosis in adult cases is often fortuitous following radical nephrectomy for presumed adult Renal cell carcinoma (RCC). Due to the lack of published discussion, there are no standard protocols for the management of adult Wilms tumour (WT) hence it is managed as per paediatric WT. In view of this, we report a case of adult WT in a 32-years-old female, who was diagnosed propitiously following right radical nephrectomy for an assumed right renal mass.
Journal of Clinical Pathology, 2010
In the International Society of Paediatric Oncology renal tumour trials, preoperative chemotherapy has been successfully applied with resulting reduction of tumour rupture and increased favourable stage distribution of nephroblastoma. Postoperative treatment includes chemotherapy and sometimes radiotherapy in a riskadapted approach based on histological sub-classification and stage of the tumour. However, preoperative chemotherapy alters the tumour's histological features and distribution of subtypes, and makes staging more difficult. The paper highlights the most common practical diagnostic difficulties that a pathologist is faced with in dealing with pretreated nephroblastomas. It emphasises the importance of a systematic, step-by-step analysis based on adequately sampled material, in order to accurately sub-classify a nephroblastoma as a low, intermediate or high risk tumour and assign its genuine stage. Finally, it outlines the standard operating procedure for submission of renal tumours for rapid central pathology review which allows the treating oncologists to apply the optimal treatment protocol.
Adult Wilms' Tumour: Case Report and Review of Literature
Journal of Kidney Cancer and VHL, 2016
Wilms' tumour (nephroblastoma) is the most common renal tumour in children. Wilms' tumour in adults is extremely rare and has a poorer prognosis than paediatric Wilms' tumour. It is difficult to differentiate adult Wilms' tumour from renal cell carcinoma based on radiological findings alone. The diagnosis in adults is often serendipitous following nephrectomy for presumed renal cell carcinoma. Because of the paucity of literature, there are no standard protocols for the management of adult Wilms' tumour, and therefore, it is managed as per paediatric Wilms' tumour. Herein, we report the case of adult Wilms' tumour in a 43-year-old man, which was diagnosed unexpectedly following nephrectomy for presumed renal cell carcinoma.
Adult Wilms tumour: A rare entity
WILMS TUMOR (nephroblastoma) in adults is rare. Only 3% of Wilms tumors are reported in adults. Most adult patients are diagnosed unexpectedly following nephrectomy for presumed renal cell carcinoma. We present a 40 yr old female patient who came with complaints of lump and pain in left lumbar and hypochondrium region. After examination and workup, provisionally diagnosed as renal cell carcinoma of left kidney. Radical nephrectomy was done and histopathology revealed Wilms tumor.
Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology
Journal of the National Comprehensive Cancer Network
The NCCN Guidelines for Wilms Tumor focus on the screening, diagnosis, staging, treatment, and management of Wilms tumor (WT, also known as nephroblastoma). WT is the most common primary renal tumor in children. Five-year survival is more than 90% for children with all stages of favorable histology WT who receive appropriate treatment. All patients with WT should be managed by a multidisciplinary team with experience in managing renal tumors; consulting a pediatric oncologist is strongly encouraged. Treatment of WT includes surgery, neoadjuvant or adjuvant chemotherapy, and radiation therapy (RT) if needed. Careful use of available therapies is necessary to maximize cure and minimize long-term toxicities. This article discusses the NCCN Guidelines recommendations for favorable histology WT.
A rare case of a Wilms tumor: case report
2018
The nephroblastoma or Wilms tumor (WT) is the most common renal tumor in childhood, representing approximately 6-7% of all pediatric cancers, with a yearly incidence of 10 cases in one million children less than 15 years old, and continues to arouse interest by remarkable actual therapeutic successes, consecutive to the multidisciplinary approach. Its maximum incidence is around the age of 3-3.5 years old, having an equal frequency in males and females. We present the case of a child, aged three years and five months, who was diagnosed with WT (nephroblastoma) with triphasic pattern, stage II tumor, and admitted to the Department of Oncopediatry for chemotherapeutic treatment and clinico-biological investigations.
Adult Wilms' tumor. Report of two cases and review of the literature
Cancer, 1995
Background. Wilms' tumor is rare in adults. The recommended treatments for patients with Stage I1 adult Wilms' tumor with favorable histology (FH) nephroblastomas are conflicting. Methods. Two patients with Stage 11, favorable histology, adult nephroblastomas are described. Current treatment modalities are discussed and the literature is reviewed. Results. The first patient, a 52-year-old woman, probably had a late local relapse of a Wilms' tumor 21 years after nephrectomy because of a renal tumor originally diagnosed as reticular sarcoma. In this case, a recurring or an extrarenal Wilms' tumor should have been considered. After the tumor was removed, the patient received adjuvant chemotherapy with dactinomycin gnd vincristine and was disease free 44 months after diagnosis. The Wilms' tumor in the second patient, a 33-year-old woman, was discovered accidentally and classified as Stage II/FH based on preoperative biopsy. She was treated with radical nephrectomy and adjuvant chemotherapy with dactinomycin and vincristine. This patient was disease free 24 months after surgery. Conclusions. Surgery and two-drug chemotherapy with dactinomycin and vincristine is suggested for patients with Stage I1 adult Wilms' tumor with FH nephroblastomas. Cancer 1995; 75:545-51.
2010
In the International Society of Paediatric Oncology renal tumour trials, preoperative chemotherapy has been successfully applied with resulting reduction of tumour rupture and increased favourable stage distribution of nephroblastoma. Postoperative treatment includes chemotherapy and sometimes radiotherapy in a riskadapted approach based on histological sub-classification and stage of the tumour. However, preoperative chemotherapy alters the tumour's histological features and distribution of subtypes, and makes staging more difficult. The paper highlights the most common practical diagnostic difficulties that a pathologist is faced with in dealing with pretreated nephroblastomas. It emphasises the importance of a systematic, step-by-step analysis based on adequately sampled material, in order to accurately sub-classify a nephroblastoma as a low, intermediate or high risk tumour and assign its genuine stage. Finally, it outlines the standard operating procedure for submission of renal tumours for rapid central pathology review which allows the treating oncologists to apply the optimal treatment protocol.
Wilms' tumor: a 10 year retrospective study
Archives of Iranian medicine, 2007
Wilms' tumor (nephroblastoma) is the most common renal malignancy of childhood. The aim of the study was to evaluate the characteristics of Wilms' tumor and the results of combined modality treatment obtained in our center in Tehran. Fifty-five patients diagnosed as having Wilms' tumor were studied in the period between February 1992 and March 2002. Demographic features, mode of presentation, associated anomalies, the stage of tumor, histopathologic results, and the survival rates were evaluated. Of these 55 patients, 31 were males and 24 were females (M/F = 1.2). The mean age at the time of diagnosis was 45.2 months. The distribution of 54 operated patients according to the surgical stage was: stage I 32.7%, stage II 16.36%, stage III 38.1%, stage IV 9%, and stage V 1.8% (one patient (1.8%) has not been operated). Favorable histology was diagnosed in 54.5% and unfavorable histology in 43.6% of the patients. The patients were treated according to National Wilms' Tumo...