Multilocular cystic nephroma: a radiographic-pathologic correlation of 58 patients.

Abstract

Clinical radiographic, and pathologic findings of multilocular cystic nephroma (MLCN) in 58 patients are presented. The lesion that affects predominantly boys in childhood and women in adulthood is usually solitary but rarely can be multiple, and it commonly occurs as an asymptomatic mass, occasionally with hematuria. Tumors may grow slowly over years or rapidly within months. A mass that is usually identified on plain radiographs occasionally has curvilinear calcification. Excretory urography and retrograde pyelography are helpful when pelvic herniation of the tumor is recognized, or when septae are noted with total body opacification. Angiography showed an avascular mass (six lesions), a hypovascular mass (14 lesions), or a hypervascular mass three lesions). Ultrasonography is helpful when multiple circumscribed sonolucent areas are identified. Similarly, a multilocular mass with septae is usually identified by computed tomography (six of seven cases). Our findings and a review of the literature indicate that this lesion is a neoplasm that can be strongly suspected preoperatively if pelvic herniation or a multilocular pattern is noted on imaging studies.

Multicystic Nephroma-Report of Two Rare Cases in a Span Of Seven Years

“…The tumor was originally described by Edmunds in 1892 as a "cyst adenoma" and since then, the spectrum of histologic findings and multiple theories of the pathogenesis of cystic nephroma have given rise to many synonymous terms, including benign multilocular cystic nephroma and cystic nephroblastoma. 5,6 Less than 200 cases have been reported till date in the international literature. 4 Studies have confirmed a biphasic age and sex distribution: two-thirds of multi-locular cystic renal tumors occur in a predominantly male pediatric population between three months and two years of age; approximately one-third occurs in the female population, with a peak in the fifth and sixth decades of life.…”

Cystic renal cell carcinoma arising from multilocular cystic nephroma of the same kidney

“…Under the age of 5 years, MCN occurs most frequently in males, whereas the adult group has a female predominance between the ages of 40 and 60. The most common presenting symptoms are a painless abdominal mass, abdominal or flank pain, and occasionally hematuria (2).…”

Actual Approach to Cystic Nephroma

“…Mostly atypical features were noted: flank dullness, colic pain, weight loss, hematuria, hypertension or urinary infection. In 6 been used: renal multilocular cyst, benign multilocular cystic nephroma, renal cystadenoma, partial polycystic kidney, and polycystic nephroblastoma were the most commonly used, but quite confusing [2][3][4][5][6], Cystic nephroma has a special age and sex distribu tion. Children not older than 4 years are males in 75% of the cases.…”

“…Nevertheless some of these criteria will have to be adated because recently 3 bilateral cystic nephromas have been reported [2,13,14] and reports of cystic ne phroma with a Wilms' tumor in the remaining kidney have been published [2,3,15], The reports of renal ade nocarcinoma found in cystic nephroma [2,16,17] are probably adenocarcinoma with cystic degeneration due to tumor necrosis and/or hemorrhage. Cystic nephroma, certainly in the adult, is a benign neoplastic lesion of the kidney originating in the metanephrogenic tissue, out of which the functional renal parenchyma normally develops.…”

“…It is a neoplastic lesion because it is as a well-delineated and encapsulated mass, growing slowly, but sometimes explosively, not seldom in previously perfectly normal kidneys [2,13,19]. Recurrences after resection are noted (case 3 of our series) [2], In the septa cystic nephroma contains imma ture renal tubuli, abortive glomeruli, and foci of undif ferentiated metanephrogenic blastema as an expression of the primitive character and growing potential [4,20]. A small number of foci does not affect the benign behav ior of the lesion.…”