Presence of a Gsα mutation in an adrenal tumor expressing LH/hCG receptors and clinically associated with Cushing's syndrome

被引：31

作者：

Bugalho, MJM ^{[1
]}

Li, X ^{[1
]}

Rao, CV ^{[1
]}

Soares, J ^{[1
]}

Sobrinho, LG ^{[1
]}

机构：

[1] Inst Portugues Oncol, Dept Endocrinol, Mol Biol Lab, P-1093 Lisbon, Portugal

来源：

GYNECOLOGICAL ENDOCRINOLOGY | 2000年 / 14卷 / 01期

关键词：

Cushing's syndrome; IL/HCGg receptors; gsp mutations;

D O I：

10.3109/09513590009167660

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We describe the case of a patient with Cushing's syndrome due to a functioning adrenal adenoma. There was a pronounced increase in serum and urinary cortisol after administration of human chorionic gonadotropin. Immunocytochemistry revealed positive immunostaining for LH/hCG receptors. Molecular analysis documented the presence of a gsp mutation at codon 201 (CGT to TGT). The existence of this type of hCG-responsive adrenal tumor may help explain the higher prevalence of cortisol-secreting adrenal tumors versus pituitary-dependent disease in pregnant women with Cushing's syndrome as well as some reported cares of remission following delivery.

引用

页码：50 / 54

页数：5

共 20 条

[1]

BUGALHO MJ, 1992, ARQUI MED, V6, P250

[2] Clinical case seminar - G(s)alpha mutation at codon 201 in pituitary adenoma causing gigantism in a 6-year-old boy with McCune-Albright syndrome [J].

Dotsch, J ;

Kiess, W ;

Hanze, J ;

Repp, R ;

Ludecke, D ;

Blum, WF ;

Rascher, W .

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1996, 81 (11) :3839-3842

[3] GONADOTROPIN-RESPONSIVE ADRENOCORTICAL ADENOMA [J].

GIVENS, JR ;

ANDERSEN, RN ;

WISER, WL ;

COLEMAN, SA ;

FISH, SA .

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1974, 38 (01) :126-133

[4] GTPASE INHIBITING MUTATIONS ACTIVATE THE ALPHA-CHAIN OF GS AND STIMULATE ADENYLYL CYCLASE IN HUMAN PITUITARY-TUMORS [J].

LANDIS, CA ;

MASTERS, SB ;

SPADA, A ;

PACE, AM ;

BOURNE, HR ;

VALLAR, L .

NATURE, 1989, 340 (6236) :692-696

[5] LEVELS OF CHORIONIC GONADOTROPHIN IN NEWBORN INFANT AND THEIR RELATIONSHIP TO ADRENAL DEHYDROEPIANDROSTERONE [J].

LAURITZEN, C ;

LEHMANN, WD .

JOURNAL OF ENDOCRINOLOGY, 1967, 39 (02) :173-+

[6] NOVEL EXPRESSION OF HUMAN CHORIONIC-GONADOTROPIN LUTEINIZING-HORMONE RECEPTOR GENE IN BRAIN [J].

LEI, ZM ;

RAO, CV ;

KORNYEI, JL ;

LICHT, P ;

HIATT, ES .

ENDOCRINOLOGY, 1993, 132 (05) :2262-2270

[7] EXACERBATION OF CUSHINGS-DISEASE DURING PREGNANCY [J].

LIU, L ;

JAFFE, R ;

BOROWSKI, GD ;

ROSE, LI .

AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY, 1983, 145 (01) :110-111

[8] 2 G-PROTEIN ONCOGENES IN HUMAN ENDOCRINE TUMORS [J].

LYONS, J ;

LANDIS, CA ;

HARSH, G ;

VALLAR, L ;

GRUNEWALD, K ;

FEICHTINGER, H ;

DUH, QY ;

CLARK, OH ;

KAWASAKI, E ;

BOURNE, HR ;

MCCORMICK, F .

SCIENCE, 1990, 249 (4969) :655-659

[9] ACTIVATING POINT MUTATIONS OF THE GSP ONCOGENE IN HUMAN THYROID ADENOMAS [J].

OSULLIVAN, C ;

BARTON, CM ;

STADDON, SL ;

BROWN, CL ;

LEMOINE, NR .

MOLECULAR CARCINOGENESIS, 1991, 4 (05) :345-349

[10] Novel presence of luteinizing hormone chorionic gonadotropin receptors in human adrenal glands [J].

Pabon, JE ;

Li, X ;

Lei, ZM ;

Sanfilippo, JS ;

Yussman, MA ;

Rao, CV .

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1996, 81 (06) :2397-2400

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