Cystic partially differentiated nephroblastoma in an adult: a case imitating the process of normal nephrogenesis along with corresponding WT1 expression

Int J Clin Exp Pathol. 2015 Jan 1;8(1):989-94. eCollection 2015.

Abstract

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.

Keywords: Cystic partially differentiated nephroblastoma; WT1; adult; maturation; nephrogenesis.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Immunohistochemistry
  • Kidney Neoplasms / metabolism
  • Kidney Neoplasms / pathology*
  • Male
  • Middle Aged
  • WT1 Proteins / biosynthesis*
  • Wilms Tumor / metabolism
  • Wilms Tumor / pathology*

Substances

  • WT1 Proteins
  • WT1 protein, human