open access

Vol 68, No 1 (2017)
Case report
Published online: 2017-03-01
Submitted: 2016-02-14
Accepted: 2016-06-04
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Bilateral primary pigmented nodular adrenal disease as a component of Carney syndrome — case report

Dorota Sikorska, Lucyna Bednarek-Papierska, Ewa Mojs, Ewa Makowska, Marek Ruchała, Włodzimierz Samborski
DOI: 10.5603/EP.2017.0008
·
Pubmed: 28255981
·
Endokrynologia Polska 2017;68(1):70-72.

open access

Vol 68, No 1 (2017)
Case report
Published online: 2017-03-01
Submitted: 2016-02-14
Accepted: 2016-06-04

Abstract

We report a case of a 20-year-old patient with Cushing’s syndrome as a component of Carney syndrome. Carney syndrome is an autosomal dominant disease with co-existing bilateral pigmented nodular adrenal disease, heart and skin myxoma, skin pigmentation, breast fibroadenoma, testicular and ovarian tumours, thyroid tumours, and pituitary adenomas. (Endokrynol Pol 2017; 68 (1): 70–72)

Abstract

We report a case of a 20-year-old patient with Cushing’s syndrome as a component of Carney syndrome. Carney syndrome is an autosomal dominant disease with co-existing bilateral pigmented nodular adrenal disease, heart and skin myxoma, skin pigmentation, breast fibroadenoma, testicular and ovarian tumours, thyroid tumours, and pituitary adenomas. (Endokrynol Pol 2017; 68 (1): 70–72)

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Keywords

Carney syndrome; Cushing' s syndrome; primary pigmented nodular adrenal disease; adrenalectomy

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About this article
Title

Bilateral primary pigmented nodular adrenal disease as a component of Carney syndrome — case report

Journal

Endokrynologia Polska

Issue

Vol 68, No 1 (2017)

Pages

70-72

Published online

2017-03-01

DOI

10.5603/EP.2017.0008

Pubmed

28255981

Bibliographic record

Endokrynologia Polska 2017;68(1):70-72.

Keywords

Carney syndrome
Cushing's syndrome
primary pigmented nodular adrenal disease
adrenalectomy

Authors

Dorota Sikorska
Lucyna Bednarek-Papierska
Ewa Mojs
Ewa Makowska
Marek Ruchała
Włodzimierz Samborski

References (10)
  1. Louiset E, Gobet F, Libé R, et al. ACTH-independent Cushing's syndrome with bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma. J Clin Endocrinol Metab. 2010; 95(1): 18–24.
  2. Bohdanowicz-Pawlak A, Szymczak J, Waszczuk E, Bolanowski M, Bednarek-Tupikowska G. Subclinical Cushing's syndrome in adrenal incidentalomas--possible metabolic consequences. Endokrynol Pol. 2013;64(3):186-91. PubMed PMID: 23873421.
  3. Ngow HA, Khairina WM. Primary pigmented nodular adrenocortical disease. Endokrynol Pol. 2011; 62(3): 268–270.
  4. Groussin L, Cazabat L, René-Corail F, et al. Adrenal pathophysiology: lessons from the Carney complex. Horm Res. 2005; 64(3): 132–139.
  5. Stratakis CA, Sarlis N, Kirschner LS, et al. Paradoxical response to dexamethasone in the diagnosis of primary pigmented nodular adrenocortical disease. Ann Intern Med. 1999; 131(8): 585–591.
  6. Bossis I, Stratakis CA. Minireview: PRKAR1A: normal and abnormal functions. Endocrinology. 2004; 145(12): 5452–5458.
  7. Groussin L, Kirschner LS, Vincent-Dejean C, et al. Molecular analysis of the cyclic AMP-dependent protein kinase A (PKA) regulatory subunit 1A (PRKAR1A) gene in patients with Carney complex and primary pigmented nodular adrenocortical disease (PPNAD) reveals novel mutations and clues for pathophysiology: augmented PKA signaling is associated with adrenal tumorigenesis in PPNAD. Am J Hum Genet. 2002; 71(6): 1433–1442.
  8. Gonçalves FT, Feibelmann TC, Mendes CM, et al. Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review. Sao Paulo Med J. 2006; 124(6): 336–339.
  9. J. Larry Jameson, Leslie J. De Groot, A. Grossman, Endocrinology: Adult and Pediatric (Seventh Edition), Elsevier 2016.
  10. Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab. 2001; 86(9): 4041–4046.

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