Nephropathy Associated with Wilms’ Tumor

Abstract

The association of nephropathy with Wilms’ tumor and male pseudohermaphroditism is known as Drash’s syndrome. The absence of either pseudohermaphroditism or Wilms’ tumor has also been reported. The nephropathy, characterized by proteinuria or nephrotic syndrome, leads rapidly and inevitably to renal failure. The renal findings are those of a chronic glomerulopathy with mesangial hypercellularity, glomerular sclerosis, interstitial infiltration and marked tubular dilation. This infrequent syndrome usually appears in early life, between 1 and 3 years of age. The case of nephropathy associated with Wilms’ tumor we present here is characterized by age and onset: an abrupt onset of renal insufficiency without previous signs of nephropathy in a 13-year-old girl.

Glomemlonephritis Associated with Robertsonian Translocation t(13;14)

“…The presence of a Robertsonian translocation in this patient may be coincidental. However, similar renal pathology has been described in a patient with Wilm's tumor [12]. In that 13-year-old girl, histology was mesangial hypercellularity, glomerulosclerosis, interstitial infiltration, and deposits of IgG and C3 were found in mesangial areas.…”

“…A number of genetic syndromes has been reported to be associated with nephropathy including Alport, Drash's, Wiskott Al drich and Down's syndrome [14][15][16][17]. In Wilm's tumor with nephropathy (Drash's syndrome), the hypothesis of a com mon embryo genetic defect of the urogenital ridge was suggested [12,18]. Apart from the genetic aspects, there is no evidence of any immunological abnormalities in this pa tient to suggest any secondary cause of glomerulonephritis such as systemic lupus erythematosus, hepatitis B virus infection or poststreptococcal infection.…”

Nephrotic syndrome and Hodgkin disease in children: a report of five cases

“…Paraneoplastic glomerulopathy has also been reported in other malignancies that are uncommon in children (myeloma, carcinoma and Kaposi sarcoma) [2,12], as well as in patients with Burkitt lymphoma [9], and Wilm tumour [8]. Minimal-change lesions are the most frequent in patients with HD, while membranous nephropathy is more suggestive of carcinoma, although there are many exceptions to this general rule.…”