RESUMEN
Introduction and importance: Cystic partially differentiated nephroblastoma (CPDN) is a rare cystic tumor that affects the kidney. It has a low potential for malignancy. It usually presents as an abdominal mass. It may be difficult to confirm the diagnosis of CPDN without a histopathological study. Case presentation: The authors report a case of an 18-month-old girl with abdominal distention, which was noticed by her parents. An abdominal computed tomography scan showed a large multilocular cystic mass arising from the lower pole of the left kidney. A left total nephrectomy was performed. Immature blastemal elements without evidence of malignant cells were observed on histological analysis. Conclusion: The authors report a case of an 18-month-old girl with CPDN managed by total nephrectomy. CPDN should be considered in the differential diagnosis of patients with cystic renal lesions. The authors would also like to affirm that partial or total nephrectomy should be done in all cases of CPDN and other cystic renal tumors.
RESUMEN
Cases of patients with cystic partially differentiated nephroblastoma (CPDN) have been reported to date, which presented as polycystic renal tumor in all of them. It is a special pathological type of nephroblastoma. Here, we report the first case of a unicystic CPDN in a child. The patient was diagnosed with a simple renal cyst and underwent laparoscopic decortication. The naive nephron was found in the pathological section, and the diagnosis of CPDN was confirmed. The patient then underwent a radical nephrectomy and six cycles of postoperative chemotherapy. There was no recurrence or metastasis after 2 years of follow-up. Pediatric CPDN presenting as a unicystic renal tumor poses a new challenge to the diagnosis, differential diagnosis, and treatment of unicystic renal tumor.
RESUMEN
Pediatric cystic nephroma is a rare, clinically benign, renal tumor. Pediatric renal cystic lesions are complex. Imaging findings and tumor appearance are often nonspecific, and careful pathological examination is necessary. We discuss diagnosis of pediatric cystic nephroma and how to differentiate it from multicystic dysplastic kidney and cystic partially differentiated nephroblastoma.
Asunto(s)
Enfermedades Renales Quísticas , Neoplasias Renales , Riñón Displástico Multiquístico , Neuroblastoma , Tumor de Wilms , Niño , Humanos , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Tumor de Wilms/diagnóstico , Tumor de Wilms/patología , Riñón/diagnóstico por imagen , Riñón/patología , Enfermedades Renales Quísticas/diagnóstico por imagen , Enfermedades Renales Quísticas/patología , Riñón Displástico Multiquístico/diagnóstico por imagen , Riñón Displástico Multiquístico/patología , Neuroblastoma/patologíaRESUMEN
INTRODUCTION: Cystic partially differentiated nephroblastoma is a multilocular cystic variant of Wilms tumor that always presents in children. However, we encountered an elderly patient with cystic partially differentiated nephroblastoma. Therefore, we report it. CASE PRESENTATION: A 74-year-old male presented with a left renal tumor detected with ultrasonography. Contrast-enhanced computed tomography and magnetic resonance imaging revealed a 4 cm multilocular cystic tumor with septa, which suggested multilocular cystic renal cell carcinoma. Therefore, we performed a radical nephrectomy. The definitive diagnosis of cystic partially differentiated nephroblastoma was made with histopathological findings. After the surgical resection, no recurrence has occurred in the past 13 years. CONCLUSION: Cystic partially differentiated nephroblastoma can develop in adults, regardless of age. Furthermore, surgical resection can be used as an established treatment option in adult cystic partially differentiated nephroblastoma cases.
RESUMEN
In children presenting with a predominantly cystic renal tumor, the most likely diagnoses include cystic partially differentiated nephroblastoma (CPDN) and cystic nephroma (CN). Both entities are rare and limited information on the clinical and molecular characteristics, treatment, and outcome is available since large cohort studies are lacking. We performed an extensive literature review, in which we identified 113 CPDN and 167 CN. The median age at presentation for CPDN and CN was 12 months (range: 3 weeks-4 years) and 16 months (prenatal diagnosis-16 years), respectively. No patients presented with metastatic disease. Bilateral disease occurred in both entities. Surgery was the main treatment for both. Two/113 CPDN patients and 26/167 CN patients had previous, concomitant, or subsequent other tumors. Unlike CPDN, CN was strongly associated with somatic (n = 27/29) and germline (n = 12/12) DICER1-mutations. Four CPDN patients and one CN patient relapsed. Death was reported in six/103 patients with CPDN and six/118 CN patients, none directly due to disease. In conclusion, children with CPDN and CN are young, do not present with metastases, and have an excellent outcome. Awareness of concomitant or subsequent tumors and genetic testing is important. International registration of cystic renal tumor cohorts is required to enable a better understanding of clinical and genetic characteristics.
RESUMEN
Cystic partially differentiated nephroblastoma is a rare renal tumor of childhood. It is part of a spectrum of multicystic renal tumors that also includes cystic nephroma and cystic Wilms' tumor. We present a case of cystic partially differentiated nephroblastoma, highlighting the clinical and imaging diagnostic challenge. Although the histological diagnostic criteria for all these 3 entities are well established, they are clinically and radiologically indistinguishable. Cystic partially differentiated nephroblastoma is often observed in male children under 2 years old. Typical clinical presentations include abdominal masses, abdominal pain and/or hematuria. Patients should be treated according to tumor histology and stage.
RESUMEN
BACKGROUND: Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumors is still riddled with controversy as a definitive preoperative differentiation between the three has not been possible. METHODS: A retrospective evaluation was performed of the treatment strategies employed with nine cases of multicystic renal tumors treated from 2005 to 2015. RESULTS: The median age at presentation was 12 months with all except one being boys. All except two children underwent primary surgery. The median follow-up was 50 months with six children having long-term survival. One child succumbed to the disease process, one died due to an unrelated cause and another was lost to follow-up. Although there was no ambiguity with cases of cystic nephroma (CN) and cystic Wilms tumor, three of the four cases of CPDN had problems. CONCLUSION: Primary surgery for multicystic renal tumors is safe and should be seriously considered as it prevents overtreatment in cases of CN and early stage CPDN. Further studies are needed to fully understand the biological behavior of CPDN.
RESUMEN
Cystic partially differentiated nephroblastoma (CPDN) is extremely rare in adults. Only 2 cases have been documented in the English literature. Herein, we present a third case of CPDN with unique morphological and immunohistochemical features. A 45-year-old man had a multicystic right renal mass, with a maximum diameter of 3 cm on magnetic resonance imaging. Being unable to rule out malignancy, partial nephrectomy was performed. The surgically resected specimen contained a multicystic mass, 3 × 3 × 2.5 cm in size, without an expansile solid nodule. Histopathological examination revealed nephroblastomatous elements without identifiable blastema; transition from cap-mesenchyme-like cells to an immature glomerulus was observed and maturing tubules and a glomerulus were present. Despite the lack of a blastema, the diagnosis of CPDN was the most appropriate. Immunohistochemical WT1 expression imitated the pattern of ongoing normal nephrogenesis. Therefore, we believe that the blastema disappeared because of maturation.