Tumor associated with tuberous sclerosis is/ are-
Correct Answer: All the above
Description: Ans. is 'd' i.e.. All the above Tuberous SclerosisAutosomal Dominant Neurocutaneous DisorderCutaneous FeaturesNeurological FeaturesOther Featureso Hypopigmented macules/Ash-leaf spots# Seen in >90% of cases.# Are small, ovoid, scattered and variable in number.# Larger lesions are known as 'Ash-leaf spots as these may have jagged edges resembling a 'leaf'.o Shagreen Spots# Roughened, raised, leathery lesions with an orange peel consistency.o Adenoma Seacium# Facial skin hamartoma that develops in early childhood (~ 4-6 years of age).# Appears as tiny red nodules over nose and cheeks resembling acne.o Facial Angiofibromas/Ungual fibromas# Facial angiofibromas may seen ungula fibromas are more common in toes.o Cafe-an-lait spots# These may be seen occasionallyo Seizures# Seizrues are the most common presenting symptoms.# Infantile spasms may be the presenting feature during infancy.o Mental Retardation# Mental Retardation occurs inupto 50% of patients refered to tertiary care.# Patients with seizures are more prone to mental retardation,o Subependymal giant cell astrocytoma.o Renal Lesions# Renal cysts or angiomyolipomas may occur (Hematuria /obstructions)o Cardiac Lesions# Rhabdomyomas of the heart may occur.o Pulmonary Lesions# Lymphagiomyomatosis is the classical pulmonary lesion. Rarely cystic lung disease may occur.o Eye lesions# Retinal Hamartomas may occur.o Skeletal Lesions# Cystic rarefaction of the bones or the fingers of toes.
Category:
Pathology
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