Cushing disease : Influence of microsurgery on hormonal balance

in European Neuroendocrine Association - Workshop : Novel insights in the management of Cushing's syndrome (2009)

The Genetics in Cushing syndrome

in European Neuroendocrine Association - Workshop : Novel experimental data on the pituitary and adrenal tumors responsible for Cushing's syndrome (2009)

Elevated GH and normal IGF-1

in Acromegaly Consensus (2009)

Functioning Pituitary Adenomas

in Refetoff, Samuel; Weiss, Roy E (Eds.) Genetic diagnosis of endocrine disorders (2009)

Epidémiologie des adénomes hypophysaires

in Feuillets de Biologie (2009)

Les adénomes hypophysaires représentent une pathologie fréquente parmi les tumeurs cérébrales. Une étude liègoise récente a montré une prévalence des adénomes hypophysaires cliniquement actifs de 1:1064 ... [more ▼]

Les adénomes hypophysaires représentent une pathologie fréquente parmi les tumeurs cérébrales. Une étude liègoise récente a montré une prévalence des adénomes hypophysaires cliniquement actifs de 1:1064 habitants, nettement supérieure aux données antérieures. Tant dans les séries cliniques qu'autopsiques, les microadénomes à prolactine sont les adénomes prédominants. Les adénomes hypophysaires sont le plus souvent de présentation sporadique, mais des adénomes familiaux dans des formes isolées ou syndromiques sont également décrits. Les adénomes hypophysaires familiaux représentent 5 p.100 des adénomes hypophysaires et leur caractérisation clinique et génétique a été récemment enrichie par la description de la nouvelle entité FIPA (Familial Isolated Pituitary Adenomas) et des mutations du gène AIP (Aryl hydrocarbon receptor-Interactircg Protein). Bien que d'évolution habituellement bénigne, les adénomes hypophysaires sont caractérisés par une grande diversité clinique et génétique qui réclame des moyens diagnostiques et thérapeutiques spécifiques. [less ▲]

Caractérisation clinique et génétique des adénomes hypophysaires familiaux isolés (FIPA).

in Revue Médicale de Liège (2009), 64(S1), 15-19

Pituitary adenomas are common brain tumours at autopsy and radiological series of unselected population. Historically, few epidemiologic data regarding the prevalence of clinically apparent pituitary ... [more ▼]

Pituitary adenomas are common brain tumours at autopsy and radiological series of unselected population. Historically, few epidemiologic data regarding the prevalence of clinically apparent pituitary adenomas have been available. Recently, a cross-sectional study conducted in Liege, Belgium, noted that clinically-apparent pituitary adenomas occurred with a prevalence of 1:1064 inhabitants, which is 3.5-5 times the previously reported prevalence. Pituitary adenomas occur predominantly as sporadic tumors, but also in a familial setting or associated to some familial/isolated tumoral syndromes. The recent characterization of the novel clinical entity FIPA (Familial Isolated Pituitary Adenomas) increased the prevalence of familial pituitary adenomas which account now for about 5% of pituitary tumors. Distinct genetic mechanisms are continuously identified and increase our understanding of the complex clinical presentation and sometimes unpredictable evolution of pituitary adenomas. [less ▲]

Adénomes hypophysaires, épidémiologie et génétique

in Chuchotis (2009), 27(printemps), 13

Genetic, molecular and clinical features of familial isolated pituitary adenomas.

in Hormone Research (2009), 71(Suppl 2), 116-122

Pituitary adenomas occur in a familial setting in about 5% of all cases, and over half of these are due to multiple endocrine neoplasia type 1 (MEN1) and Carney's complex (CNC). Non-MEN1/CNC familial ... [more ▼]

Pituitary adenomas occur in a familial setting in about 5% of all cases, and over half of these are due to multiple endocrine neoplasia type 1 (MEN1) and Carney's complex (CNC). Non-MEN1/CNC familial pituitary tumours of all tumour phenotypes, known as familial isolated pituitary adenomas (FIPA), were first described in the late 1990s. Clinical features of FIPA differ from those of sporadic pituitary adenomas, as patients with FIPA have a younger age at diagnosis and larger tumours. About 15% of patients with FIPA have mutations in the aryl hydrocarbon receptor-interacting protein gene (AIP), which indicates that FIPA may have a diverse genetic pathophysiology. This article describes the clinical features of FIPA, the tumour pathologies found in this setting and the genetic/molecular data that have recently been reported in FIPA. [less ▲]

Update on the treatment of pituitary adenomas: familial and genetic considerations.

in Acta Clinica Belgica (2008), 63(6), 418-424

Clinically-relevant pituitary adenomas occur with a prevalence of approximately 1 per 1000 population in Belgium. Pituitary adenomas that occur in families are likely to have an important genetic ... [more ▼]

Clinically-relevant pituitary adenomas occur with a prevalence of approximately 1 per 1000 population in Belgium. Pituitary adenomas that occur in families are likely to have an important genetic pathophysiological basis. Currently about 5% of all pituitary adenoma cases have a family history of pituitary adenomas, classically due to multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). Over the last decade we have described non-MEN1/CNC familial pituitary tumours that include all tumour phenotypes, a condition named 'familial isolated pituitary adenoma' (FIPA). Clinical features of FIPA differ from those of sporadic pituitary adenomas in that patients with FIPA are often younger and have larger tumours at diagnosis. Approximately 15% of FIPA patients have mutations in the aryl hydrocarbon receptor interacting protein gene (AIP), which indicates that FIPA may have a diverse genetic pathophysiology. In this review we examine new findings on the epidemiology of pituitary adenomas and we review familial causes of pituitary adenomas with a particular emphasis on modern clinical testing. In addition, the clinical and genetic features of FIPA are described as FIPA represents a useful framework to study the features of pituitary adenomas that occur in a familial setting. [less ▲]

M Cushing : a life long experience with pituitary surgery

Scientific conference (2008, October 07)