Article
Malignant pancreatic endocrine tumor in a child with tuberous sclerosis.
The American journal of surgical pathology - 1 Oct 2003
Francalanci Paola, Diomedi-Camassei Francesca, Purificato Cristina, Santorelli Filippo Maria, Giannotti Aldo, Dominici Carlo, Inserra Alessandro, Boldrini Renata
Abstract excerpt
Tuberous sclerosis complex (TSC) is an autosomal dominant condition whose signs and symptoms may vary from a few hypopigmented skin spots to epilepsy, severe mental retardation, and renal failure. The disease is caused by mutations in either TSC1 or TSC2 gene, at chromosome 9q34 and 16p13.3. Inactivation of both alleles at TSC1 or TSC2 loci is associated with the development of hamartomas in different organs, and...
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