Tuberous sclerosis-associated renal cell carcinoma

Tuberous sclerosis-associated renal cell carcinoma, abbreviated TSARCC, is a kidney tumour that is associated with the syndrome tuberous sclerosis.

General

• Not in the WHO classification of renal neoplasia or the Vancouver modification of the WHO classification.
• Afflicts individuals at a younger age than sporadic renal cell carcinoma.
• 2-4% of individual with tuberous sclerosis develop renal cell carcinoma.

Microscopic

Features as per Guo et al. describes three different morphologies:

• 1. Renal angiomyoadenomatous tumour-like.
  2. Chromophobe renal cell carcinoma-like.
  3. Macrocystic architecture composed of cells with a granular eosinophilic cytoplasm.

Notes:

• There is also a paper by Yang et al.; it describes three different patterns:
  1. TSC-associated papillary RCC.
  2. Renal cell carcinoma, unclassified.
  3. Hybrid oncocytic/chromophobe tumour (HOCT).

DDx:

• Renal angiomyoadenomatous tumour.
• Chromophobe renal cell carcinoma.
• Renal oncocytoma.
• Papillary renal cell carcinoma.
• Hereditary leiomyomatosis renal cell carcinoma syndrome-associated renal cell carcinoma.
• Unclassified renal cell carcinoma.

See also

• Renal tumours.
• Tuberous sclerosis.