Malignant pancreatic endocrine tumor in a child with tuberous sclerosis.

  title={Malignant pancreatic endocrine tumor in a child with tuberous sclerosis.},
  author={Paola Francalanci and Francesca Diomedi-Camassei and Cristina Purificato and Filippo Maria Santorelli and Aldo Giannotti and Carlo Dominici and Alessandro Inserra and Renata Boldrini},
  journal={The American journal of surgical pathology},
  volume={27 10},
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 only rarely with malignant neoplasms. In this study we present a 6-year-old boy… CONTINUE READING


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