Panlobar Nephroblastomatosis with Cystic Dysplasia: An Unusual Case with Diffuse Renal Involvement Studied by Immunohistochemistry

Abstract

A unilateral cystic renal process discovered prenatally was removed in a neonate. Dysplastic cysts were associated with diffuse (both intra- and perilobar) nephroblastomatosis. We describe a comprehensive immunohistological study confirming the transition observed on simple histology between the different structures: nephrogenic rests (CD9⁺, CD24^+/-, CD56^+/-), glomeruloid bodies (CD10⁺⁺, CD35⁺⁺), ducts lined by columnar epithelium (CD9⁺, CD24⁺, CD56⁺⁺), cysts lined by cuboidal or thin epithelium (some cells CD10⁺, CD26⁺, others EMA⁺, CD24⁺). Although no typical S-shaped bodies are seen, small cysts and ducts with a columnar epithelium are considered similar. The dysplastic primitive ducts are KL1⁺⁺, vimentin^+/-, CD9⁺, CD24⁺. With a view to assessing dysplastic preneoplastic potential, the value of CD56 and Ki67 as activation antigens with possible prognostic significance is discussed.

Key Words:

• multicystic kidney
• nephroblastomatosis
• nephrogenic rests
• renal dysplasia
• Wilms' tumor