Birt-Hogg-Dube syndrome associated with:
Correct Answer: Renal cell carcinoma
Description: Ans: (a) Renal cell carcinomaRef: Brenner and Rector's The Kidney, 10th edition, Page 1372Birt-Hogg-Dube syndromeCharacterized by prominent cutaneous manifestations, the development of spontaneous pneumothorax in association with lung cystsPredisposition to kidney neoplasms (chromophobe renal cancers, oncocytomas, or mixed oncocytic)Affected gene is folliculin*Characteristic skin lesions, fibrofolliculomas (hamartomas of the hair follicle), develop on the skin of the head and neck of patients with the syndrome after age 30Genetic syndromes associated with renal cell carcinomaVHL syndrome-clear cell histologyMultiple cutaneous and uterine leiomyomas (MCUL)Hereditary leiomyomatosis and renal cell cancer (HLRCC) syndromeBirt-Hogg-Dube syndromeTuberous sclerosis
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