Pituitary pathology in patients with Carney Complex: growth-hormone producing hyperplasia or tumors and their association with other abnormalities.

Pituitary 2006 ;9(3):203-9

Section on Endocrinology & Genetics, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892-1103, USA.

First described in the mid 80's, Carney Complex (CNC) is a rare, dominantly heritable disorder with features overlapping those of McCune-Albright syndrome (MAS) and other multiple endocrine neoplasia (MEN) syndromes like MEN type 1 (MEN 1). Pituitary tumors have been described in a number of patients with CNC; they present with elevated growth hormone (GH) levels and mild hyperprolactinemia. However, most patients with CNC have mild hypersomatomammotropinemia starting in adolescence; this is similar to the situation in MAS patients: in both disorders, pituitary hyperplasia appears to precede tumor development. Familial pituitary tumor syndromes such as CNC provide an important insight into the genetics and molecular pathology of pituitary and other endocrine tumors. Our understanding of these conditions is expanding rapidly due to the identification of the causative genes and the availability of murine disease models. The present report reviews the clinical findings related to pituitary tumor development among patients with CNC and provides an update on murine models of the complex.

Download full-text PDF

Source
http://link.springer.com/10.1007/s11102-006-0265-2
Publisher Site
http://dx.doi.org/10.1007/s11102-006-0265-2DOI Listing
June 2009
4 Reads

Publication Analysis

Top Keywords

patients cnc
12
pituitary tumor
8
tumor development
8
carney complex
8
pituitary
6
cnc
5
patients
5
disorders pituitary
4
pituitary hyperplasia
4
patients disorders
4
mas patients
4
hyperplasia appears
4
tumor syndromes
4
familial pituitary
4
situation mas
4
precede tumor
4
development familial
4
appears precede
4
hypersomatomammotropinemia starting
4
elevated growth
4

References

(Supplied by CrossRef)

JA Carney et al.
Endocrinologist 1992

CA Stratakis et al.
Horm Metabol Research 1998

JA Carney et al.
Semin Dermatol 1995

CA Stratakis et al.
Am J Med Genet 1998

JA Carney et al.
Mayo Clin Proc 1986

LS Kirschner et al.
Nat Genet 2000

CA Stratakis et al.
J Clin Invest 1996

A Gessl et al.
J Clin Endocrinol Metab 1994

MB Garcia et al.
J Endocrinol Invest 1994

L Cuttler et al.
J Clin Endocrinol Metab 1989

Similar Publications