The clinical, pathological, and genetic features of familial isolated pituitary adenomas
نویسندگان
چکیده
منابع مشابه
The clinical, pathological, and genetic features of familial isolated pituitary adenomas.
Pituitary adenomas occur in a familial setting in multiple endocrine neoplasia type 1 (MEN1) and Carney's complex (CNC), which occur due to mutations in the genes MEN1 and PRKAR1A respectively. Isolated familial somatotropinoma (IFS) is also a well-described clinical syndrome related only to patients with acrogigantism. Pituitary adenomas of all types--not limited to IFS--can occur in a familia...
متن کاملClinical and genetic aspects of familial isolated pituitary adenomas
Pituitary adenomas represent a group of functionally diverse neoplasms with relatively high prevalence in the general population. Most occur sporadically, but inherited genetic predisposing factors are increasingly recognized. Familial isolated pituitary adenoma is a recently defined clinical entity, and is characterized by hereditary presentation of pituitary adenomas in the absence of clinica...
متن کاملClinical characterization of familial isolated pituitary adenomas.
CONTEXT Familial pituitary adenomas occur rarely in the absence of multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). OBJECTIVE Our objective was to characterize the clinical and genealogical features of non-MEN1/CNC familial isolated pituitary adenomas (FIPA). DESIGN AND SETTING We conducted a retrospective study of clinical and genealogical characteristics of FIPA cases ...
متن کاملFamilial Isolated Pituitary Adenomas
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 Interac...
متن کاملPituitary Disorders Familial Isolated Pituitary Adenomas
Over the last century several families have been described with familial isolated pituitary adenomas (FIPAs). Most commonly, family members have acromegaly or prolactinoma, but other types of pituitary adenomas can also occur. Recently, mutations in the AIP (aryl hydrocarbon receptor interacting protein) gene have been found to occur in 30–50% of FIPA patients, while for the rest of the patient...
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ژورنال
عنوان ژورنال: European Journal of Endocrinology
سال: 2007
ISSN: 0804-4643,1479-683X
DOI: 10.1530/eje-07-0348