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What is Most common tumor in tuberous sclerosis in females: and explain in detail?
Ans: (b) AngiomyolipomaRobbins Pathology, 9th editionTumours in tuberous sclerosisRenal angiomyolipomas develop in as many as 80% of people with tuberous sclerosis by the age of 10 years.Lymphangioleiomyomatosis is present in as many as 40% of adult women with tuberous sclerosis. Though the incidence is more common in women than in men, since the question also has angiomyolipoma I personally prefer to go for renal angiomyolipomas.If the question comes in future without angiomyolipomas - go for Lymphangioleiomyomatosis - since the overall incidence of it is 1-4% (WHO) and in women it is close to 40% (WHO).Diagnostic criteria for tuberous sclerosisMajor features*. >3 hypomelanotic macules > 5 mm in diameter*. >3 angiofibromas or fibrous cephalic plaque*. >2 ungual fibromas*. Shagreen patch*. Multiple retinal hamartomas*. Cortical dysplasias (including tubers and cerebral white matter radial migration lines)*. Subependymal nodules*. Subependymal giant cell astrocytoma*. Cardiac rhabdomyoma*. Lymphangioleiomyomatosis*. > 2 angiomyolipomasMinor featuresConfetti skin lesions*. >4 dental enamel pits*. >2 intraora! fibromas, Retinal achromic patch, Multiple renal cysts, Non-renal hamartomasDefinitive diagnosis; 2 major features or 1 major feature with >2 minor featuresPossible diagnosis: 1 major feature or 2 minor features