Cystic Wilms Research Articles

Multilocular Cystic Nephroma: Is Radical Nephrectomy The Best Treatment In All Cases? Our Experience After 45 Years Working on a Tertiary Care Center

Introduction: Multilocular cystic nephroma (MCN) is a type of benign renal tumor that is sometimes misdiagnosed with cystic Wilms Tumor. Given that each one has a different treatment, it is essential to make a proper diagnosis. Objective: Our aim was to find the differential characteristics of MCN and to be able to identify these renal masses with low probability of malignancy, which are subsidiaries of a more conservative treatment. Material and Methods: We conducted a retrospective descriptive study were we analyzed the patients with MCN treated in our center between 1971 and 2016. Results: We found 13 patients with a histological diagnosis of MCN, mostly males, with an average age of 46.8 months at the time of surgery. Clinically, 2 cases (15.4%) were asymptomatic, 6 had an abdominal mass (46.1%), 3 had abdominal pain (23.1%) and 2 had hematuria (15.4%). The maximum diameter of the mass was 7.3 cm (mean). 9 nephrectomies (69.2%) and 4 tumorectomies (30.8%) were performed. In two of these (15.4%), neoadjuvant chemotherapy was administered due to presurgical suspicion of Wilms' tumor, which was discontinued after the pathological analysis. In another 4 patients with suspicion of MCN, all of them asymptomatic and with a tumor size <3cm, non-surgical treatment was decided. At the present time all patients (operated or not) stay alive. No. cases of distant metastasis, local recurrence or mass progression (in the non-operated patients) have been recorded. Conclusion: Given its good prognosis, MCN can be treated with a non-radical surgery or even non-surgical conservative treatment in selected cases. In our experience, the clinical and radiological characteristics are the pillars to identify those patients with low risk of malignancy in which this attitude could be carried out.

Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken.

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.

Cystic variant of favorable-histology Wilms′ tumor presenting with osteolytic metastasis to the ribs

Cystic neoplasms of the kidney presenting in childhood always present a therapeutic dilemma. Low-risk renal tumors of childhood cover a wide spectrum: cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor. There are difficulties in differentiating these tumors on preoperative imaging. The present case presented such a therapeutic dilemma and was finally diagnosed as a cystic variant of favorable-histology Wilms' tumor with osteolytic metastasis to the ribs. The case is presented in view of its rare presentation and the therapeutic and diagnostic challenges involved.

Unusual Case of Multilocular Cystic Nephroma Treated with Nephron Sparing Technique: A Case Report

Multilocular cystic nephroma is an unusual entity of uncertain etiology that can affect children and adults equally. This lesion is believed to be part of a spectrum of renal cystic disorders with multilocular cyst and cystic Wilms' tumor representing the two extremes and usually presents with a benign character. We report a 22-year-old female who presented with a painful right renal mass and gross hematuria. After a series of examinations including abdominal ultrasound, computed tomography, and angiography, she underwent partial nephrectomy under the impression of benign lesion. The pathologic examination showed that it was a multilocular cystic nephroma. At the 11-month follow-up, the patient was completely asymptomatic and free of recurrence and metastasis.

Cystic partially differentiated nephroblastoma

Cystic partially differentiated nephroblastoma is a relatively rare tumour of the kidney usually affecting infants. Cystic Wilms' tumour and multilocular cystic nephroma should be distinguished from cystic partially differentiated nephroblastoma. Multilocular cystic nephroma is a benign tumour whereas cystic Wilms' tumour is at the malignant end of the range of classification of such tumours. Cystic partially differentiated nephroblastoma may undergo local recurrence but there is no report of metastasis.

A Rare Case of Bilateral Multilocular Renal Cysts

Several names have been used to describe a rare multiloculated cystic lesion in the kidney, including benign multilocular cyst, multilocular cystic nephroma, cystic nephroma and others. The abundance of names is partially attributable to an incomplete understanding of the etiology and natural history of this condition. Most authorities believe that this lesion is part of a spectrum of renal cystic disorders with multilocular cyst and cystic Wilms tumor representing the 2 extremes. Whatever the etiology, multilocular cysts have a typical radiographic appearance, are almost uniformly unilateral and are treated with curative nephrectomy. We report a unique case of bilateral multilocular renal cysts treated with bilateral partial nephrectomy. CASE REPORT A 15-month-old Hispanic boy presented with a 3-month history of progressively enlarging bilateral flank masses, and nausea, vomiting and anorexia 1 week in duration. There was no other significant medical history. Physical examination revealed blood pressure of 100/60 mm. Hg and abdominal examination demonstrated bilateral 12 to 14 cm., nontender, nonfixed, firm flank masses. Serum electrolytes, urea nitrogen, creatinine, hemoglobin and urinalysis were normal. Abdominal ultrasound and computerized tomography showed 10 X 12 cm. cystic renal masses with multiple septa (fig. 1). A rim of normal appearing renal parenchyma (right side greater than left side) was displaced inferiorly and medially. 3-Mercaptoacetyltriglycine nuclear renal scan revealed that 80% of renal function was by the right kidney. Abdominal exploration was planned but moderate hypertension developed, which was controlled with oral calcium channel blockers. Subsequently exploration was performed through a transperitoneal midline incision. The left renal mass was approached first, revealing a firm mobile cystic renal mass contiguous with the inferior pole of normal appearing renal tissue. Because of the benign appearance of this mass and bilateral involvement, a nephron sparing procedure was performed involving partial nephrectomy with renal reconstruction. The mass was completely excised en bloc (fig. 2). However, the dilated renal pelvis was opened to extrude the inferior aspect of the mass, which had herniated into the collecting system. Frozen section was consistent with a multilocular renal cyst without evidence of malignancy. Exploration of the right side revealed similar fmdings and an identical procedure was performed. Postoperatively the patient recovered well. He is currently asymptomatic and thriving, and hypertension has completely resolved. Followup renal scan and ultrasound 6 months postoperatively demonstrated excellent function of both remaining renal units with no evidence of any recurrent cystic lesions. The final pathological diagnosis was consistent with bilateral benign multilocular renal cysts.

Multilocular cysts of kidney

Multilocular renal cyst is a distinct renal tumor whose gross external appearance and absence of normal renal tissue within the septa of loculi distinguish it clearly from other renal cystic lesions. Interlocular septa may contain either (1) fibrous tissue alone or (2) embryonic tissue separating adjacent loculi. Of 29 patients with multilocular renal cysts, 24 underwent a renal-sparing procedure, and only 5 had radical nephrectomy. None of the histologic specimens showed evidence of immature renal tissue or neoplasia. Patients were followed from three months to eight years (mean, 39 months), and no evidence of local recurrence or metastatic disease was found. Because it is difficult to distinguish multilocular renal cyst from cystic Wilms tumor and multicystic clear cell carcinoma on the basis of imaging studies alone, surgical intervention is the only effective method to differentiate multilocular renal cyst from a malignant lesion of the kidney.

Cystic mesoblastic nephroma

Mesoblastic nephroma is the most common solid renal neoplasm in the first few months of life. It has been recognized only recently as a distinct entity, having been classified previously as Wilms tumor. There are relatively few ultrasonic descriptions of this entity in the literature. We present an additional case featuring a large peripheral cystic component with a solid central core demonstrated on ultrasonography. This feature is not specific for mesoblastic nephroma and occurs in cystic Wilms tumor.

Wilms' tumor in a multilocular cyst of the kidney (cystic Wilms' tumor) : S. H. Chang. So. Med. J. 69:1623–1625 (December), 1976

Wilms' tumor in a multilocular cyst of the kidney (cystic Wilms' tumor) : S. H. Chang. So. Med. J. 69:1623–1625 (December), 1976

Wilmsʼ Tumor in a Multilocular Cyst of the Kidney (Cystic Wilmsʼ Tumor)

A 2-year-old boy had a Wilms' tumor of the left kidney that appeared during physical examination as an abdominal mass and on gross examination as a multilocular cyst. Multilocular cyst of the kidney is a rare but distinctive congenital lesion. Its true biologic behavior is unknown. Although simple nephrectomy is sufficient for most lesions, more aggressive procedures are recommended for those lesions that contain Wilms' tumor (cystic Wilms' tumor).