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- W2485460018 abstract "Pituitary adenomas are frequent benign tumors that cause a high morbidity due to their complications. Most cases are sporadic but 5% arise in a familial setting, associated with other tumors (as is for MEN1 and MEN4, Carney Complex, McCune-Albright Syndrome) or without other associated disease as is for FIPA (Familial Isolated Pituitary Adenomas) families. AIP (Aryl-hydrocarbon Receptor Interacting Protein), a tumor suppressor gene, is mutated in approximately 30% of FIPA patients and in a much lesser percent in sporadic pituitary adenomas. Patients harboring an AIP mutation have certain characteristics: are younger and with larger pituitary adenomas, usually secreting growth hormone or prolactin and more resistant to current therapies as compared to AIP negative patients. Recently a new genotype was identified in FIPA patients with early onset gigantism - microduplications on chromosome Xq26.3 and the syndrome was called X-LAG (X-linked acrogigantism). In more than a half of FIPA patients the genetic background of the disease is still unknown and more work is needed in this direction." @default.
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- W2485460018 date "2016-03-30" @default.
- W2485460018 modified "2023-09-22" @default.
- W2485460018 title "Familial Isolated Pituitary Adenomas" @default.
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