Cystic nephroma pathophysiology

Cystic nephroma Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Overview

On gross pathology, large, unilateral, multiloculated, and well-encapsulated cystic tumor is the characteristic finding of cystic nephroma. On microscopic histopathological analysis, cysts lined by a simple epithelium with a hobnail morphology and ovarian-like stroma are characteristic findings of cystic nephroma.

Gross Pathology

Characteristic findings of cystic nephroma on gross pathology include:

• Large, unilateral, and multiloculated cystic tumor
• Well-encapsulated
• Nodular surface

Microscopic Pathology

The characteristics of cystic nephromas include:

• Cysts lined by a simple epithelium with a hobnail morphology（i.e. the nuclei of the cyst lining epithelium bulges into the lumen of the cysts）
• Ovarian-like stroma

• Spindle cell morphology
• Basophilic cytoplasm

• 

  Cystic nephroma

Genetics

• DICER1 mutations are involved in the pathogenesis of cystic nephroma.

• DICER1 is a human gene. This gene encodes a protein possessing an RNA helicase motif containing a DEXH box in its amino terminus and an RNA motif in the carboxy terminus. The encoded protein functions as a ribonuclease and is required by the RNA interference and small temporal RNA (stRNA) pathways to produce the active small RNA component that represses gene expression. Two transcript variants encoding the same protein have been identified for this gene.