Chromophobe type renal cell carcinoma
Chromophobe type renal cell carcinoma
Updated: 10/07/2020
© Jun Wang, MD, PhD
General features
- Usually > 45, no gender preference
- Originated from intercalated cell of cortical collecting duct
- May be associated with Birt-Hogg-Dubé syndrome
- 8% multifocal, 3% bilateral
- Less aggressive comparing with clear cell type
Birt-Hogg-Dubé syndrome
- Autosomal dominant with incomplete penetrance
- Germline mutations in BHD
- Skin lesions: fibrofolliculomas, trichodiscomas, acrochordons
- Lung: cysts, bullous emphysema, spontaneous pneumothorax
- Renal tumors: Oncocytoma, chromophobe type renal cell carcinoma, and hybrid oncocytic-chromophobe tumors with areas of clear cells
Pathological features
- Well-circumscribed, tan brown
- Geographic necrosis
- Abundant slightly granular, pale eosinophiliccytoplasm
- Raisinoid nuclei and perinuclear halos
Markers
- Diffusely positive for CK7 (Scattered to negative reactivity in oncocytoma)
- Positive for Hale's colloidal iron stain (Negative in oncocytoma)
Genetic abnormality
- Multiple losses of various chromosome
- P53 and PTEN
- If associated with Birt-Hogg-Dubé syndrome: BHD, encoding folliculin
Differential diagnosis
- Oncocytoma: No perinuclear halo, scattered weak CK7 reactivity, negative colloidal iron
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