eMedicine Specialties > Dermatology > Internal Medicine
Carney Syndrome: Treatment & Medication
Updated: Oct 7, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- In Carney complex (CNC), medical care is restricted to the treatment of the endocrine overactivity that is commonly present.
- See Acromegaly, Cushing Syndrome, Hyperparathyroidism, and Hyperprolactinemia for the treatment of these conditions.
- No medical treatment for myxomas exists.
Surgical Care
- A primary cardiac tumor requires prompt surgical excision.
- Large or symptomatic skin myxomas can be excised.
- Other tumors (eg, schwannoma, Sertoli-cell tumors, parathyroid tumors, adrenal tumors) should be surgically treated, and adjuvant radiation therapy and/or chemotherapy should be administered if necessary.
Consultations
- Cardiologist
- Geneticist
- Cardiothoracic surgeon
- Endocrinologist
- Oncologist
More on Carney Syndrome |
| Overview: Carney Syndrome |
| Differential Diagnoses & Workup: Carney Syndrome |
Treatment & Medication: Carney Syndrome |
| Follow-up: Carney Syndrome |
| Multimedia: Carney Syndrome |
| References |
| « Previous Page | Next Page » |
References
Atherton DJ, Pitcher DW, Wells RS, MacDonald DM. A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata and atrial myxoma: the NAME syndrome. Br J Dermatol. Oct 1980;103(4):421-9. [Medline].
Rhodes AR, Silverman RA, Harrist TJ, Perez-Atayde AR. Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi: the "LAMB" syndrome. J Am Acad Dermatol. Jan 1984;10(1):72-82. [Medline].
Carney JA. Differences between nonfamilial and familial cardiac myxoma. Am J Surg Pathol. Jan 1985;9(1):53-5. [Medline].
Groussin L, Cazabat L, Rene-Corail F, Jullian E, Bertherat J. Adrenal pathophysiology: lessons from the Carney complex. Horm Res. 2005;64(3):132-9. [Medline].
Wilkes D, Charitakis K, Basson CT. Inherited disposition to cardiac myxoma development. Nat Rev Cancer. Feb 2006;6(2):157-65. [Medline].
Groussin L, Horvath A, Jullian E, et al. A PRKAR1A mutation associated with primary pigmented nodular adrenocortical disease in 12 kindreds. J Clin Endocrinol Metab. May 2006;91(5):1943-9. [Medline].
Urban C, Weinhäusel A, Fritsch P, et al. Primary pigmented nodular adrenocortical disease (PPNAD) and pituitary adenoma in a boy with sporadic Carney complex due to a novel, de novo paternal PRKAR1A mutation (R96X). J Pediatr Endocrinol Metab. Feb 2007;20(2):247-52. [Medline].
Almeida MQ, Brito LP, Domenice S, et al. [Absence of PRKAR1A loss of heterozygosity in laser-captured microdissected pigmented nodular adrenocortical tissue from a patient with Carney complex caused by the novel nonsense mutation p.Y21X.]. Arq Bras Endocrinol Metabol. Nov 2008;52(8):1257-63. [Medline].
Sasaki A, Horikawa Y, Suwa T, Enya M, Kawachi S, Takeda J. Case report of familial Carney complex due to novel frameshift mutation c.597del C (p.Phe200LeufsX6) in PRKAR1A. Mol Genet Metab. Nov 2008;95(3):182-7. [Medline].
Vandersteen A, Turnbull J, Jan W, et al. Cutaneous signs are important in the diagnosis of the rare neoplasia syndrome Carney complex. Eur J Pediatr. Nov 2009;168(11):1401-4. [Medline].
Lee B, Sir JJ, Park SW, et al. Right-sided myxomas with extramedullary hematopoiesis and ossification in Carney complex. Int J Cardiol. Nov 12 2008;130(2):e63-5. [Medline].
Kirschner LS, Carney JA, Pack SD, et al. Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex. Nat Genet. Sep 2000;26(1):89-92. [Medline].
Kirschner LS, Sandrini F, Monbo J, Lin JP, Carney JA, Stratakis CA. Genetic heterogeneity and spectrum of mutations of the PRKAR1A gene in patients with the carney complex. Hum Mol Genet. Dec 12 2000;9(20):3037-46. [Medline].
Abdelmalek NF, Gerber TL, Menter A. Cardiocutaneous syndromes and associations. J Am Acad Dermatol. Feb 2002;46(2):161-83; quiz 183-6. [Medline].
Arce B, Licea M, Hung S, Padron R. Familial Cushing's syndrome. Acta Endocrinol (Copenh). Jan 1978;87(1):139-47. [Medline].
Barlow JF, Abu-Gazeleh S, Tam GE, et al. Myxoid tumor of the uterus and right atrial myxomas. S D J Med. Jul 1983;36(7):9-13. [Medline].
Basson CT, MacRae CA, Korf B, Merliss A. Genetic heterogeneity of familial atrial myxoma syndromes (Carney complex). Am J Cardiol. Apr 1 1997;79(7):994-5. [Medline].
Bauer AJ, Stratakis CA. The lentiginoses: cutaneous markers of systemic disease and a window to new aspects of tumourigenesis. J Med Genet. Nov 2005;42(11):801-10. [Medline].
Carney JA, Ferreiro JA. The epithelioid blue nevus. A multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol. Mar 1996;20(3):259-72. [Medline].
Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore). Jul 1985;64(4):270-83. [Medline].
Carney JA, Headington JT, Su WP. Cutaneous myxomas. A major component of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Arch Dermatol. Jul 1986;122(7):790-8. [Medline].
Carney JA, Hruska LS, Beauchamp GD, Gordon H. Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc. Mar 1986;61(3):165-72. [Medline].
Carney JA, Stratakis CA. Epithelioid blue nevus and psammomatous melanotic schwannoma: the unusual pigmented skin tumors of the Carney complex. Semin Diagn Pathol. Aug 1998;15(3):216-24. [Medline].
Casey M, Mah C, Merliss AD, et al. Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation. Dec 8 1998;98(23):2560-6. [Medline].
Dao LN, Scheithauer BW, Erlandson RA, Young WF Jr, Aidan Carney J. Divergent myoid, neuroendocrine, and perineural differentiation in a nasal tumor of a patient with Carney complex. Am J Surg Pathol. Jan 2008;32(1):167-71. [Medline].
Goldstein MM, Casey M, Carney JA, Basson CT. Molecular genetic diagnosis of the familial myxoma syndrome (Carney complex). Am J Med Genet. Sep 3 1999;86(1):62-5. [Medline].
Groben PA, Harvell JD, White WL. Epithelioid blue nevus: neoplasm Sui generis or variation on a theme?. Am J Dermatopathol. Dec 2000;22(6):473-88. [Medline].
Handley J, Carson D, Sloan J, et al. Multiple lentigines, myxoid tumours and endocrine overactivity; four cases of Carney's complex. Br J Dermatol. Apr 1992;126(4):367-71. [Medline].
Henley DJ, van Heerden JA, Grant CS, Carney JA, Carpenter PC. Adrenal cortical carcinoma--a continuing challenge. Surgery. Dec 1983;94(6):926-31. [Medline].
Iacobellis G, Di Gioia CR, Tamburrano G. Images in Cardiology: Asymptomatic right atrial myxoma in acromegalic man: a case of Carney complex. Heart. Jan 2001;85(1):86. [Medline].
Jayasena SN, Ariyasinghe JT, Gunawardena DM, Gunawardena SA, de Silva MV. Large-cell calcifying sertoli cell tumour of the testis detected at screening of a family with Carney syndrome. Urol Int. 2005;75(4):365-7. [Medline].
Kennedy RH, Waller RR, Carney JA. Ocular pigmented spots and eyelid myxomas. Am J Ophthalmol. Nov 15 1987;104(5):533-8. [Medline].
Kirschner LS, Carney JA, Pack SD, et al. Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex. Nat Genet. Sep 2000;26(1):89-92. [Medline].
Kirschner LS, Taymans SE, Stratakis CA. Characterization of the adrenal gland pathology of Carney complex, and molecular genetics of the disease. Endocr Res. Aug-Nov 1998;24(3-4):863-4. [Medline].
Koopman RJ, Happle R. Autosomal dominant transmission of the NAME syndrome (nevi, atrial myxoma, mucinosis of the skin and endocrine overactivity). Hum Genet. Jan 1991;86(3):300-4. [Medline].
Legius E, Daenen W, Vandenbergh V, Verbeeck G, Bex M, Fryns JP. Syndrome of myxomas, spotty skin pigmentation, and endocrine overactivity (Carney complex). Genet Couns. 1998;9(4):287-90. [Medline].
Mai PL, Korde L, Kramer J, et al. A possible new syndrome with growth-hormone secreting pituitary adenoma, colonic polyposis, lipomatosis, lentigines and renal carcinoma in association with familial testicular germ cell malignancy: A case report. J Med Case Reports. Mar 28 2007;1:9. [Medline].
McCarthy PM, Piehler JM, Schaff HV, et al. The significance of multiple, recurrent, and "complex" cardiac myxomas. J Thorac Cardiovasc Surg. Mar 1986;91(3):389-96. [Medline].
Moreno C, Requena L, Kutzner H, de la Cruz A, Jaqueti G, Yus ES. Epithelioid blue nevus: a rare variant of blue nevus not always associated with the Carney complex. J Cutan Pathol. May 2000;27(5):218-23. [Medline].
Nwokoro NA, Korytkowski MT, Rose S, et al. Spectrum of malignancy and premalignancy in Carney syndrome. Am J Med Genet. Dec 31 1997;73(4):369-77. [Medline].
O'Grady TC, Barr RJ, Billman G, Cunningham BB. Epithelioid blue nevus occurring in children with no evidence of Carney complex. Am J Dermatopathol. Oct 1999;21(5):483-6. [Medline].
Pack SD, Kirschner LS, Pak E, Zhuang Z, Carney JA, Stratakis CA. Genetic and histologic studies of somatomammotropic pituitary tumors in patients with the "complex of spotty skin pigmentation, myxomas, endocrine overactivity and schwannomas" (Carney complex). J Clin Endocrinol Metab. Oct 2000;85(10):3860-5. [Medline].
Perry CW, Rothkopf DM. Carney syndrome and the plastic surgeon: presentation, recognition, and evaluation. Br J Plast Surg. Apr 2005;58(3):413-5. [Medline].
Proppe KH, Scully RE. Large-cell calcifying Sertoli cell tumor of the testis. Am J Clin Pathol. Nov 1980;74(5):607-19. [Medline].
Rees JR, Ross FG, Keen G. Lentiginosis and left atrial myxoma. Br Heart J. Aug 1973;35(8):874-6. [Medline].
Rhodes AR. The risk of malignant melanoma arising in congenital melanocytic nevi. An argument against the assignment of risk based on size alone. Am J Dermatopathol. Summer 1984;6 Suppl:184-8. [Medline].
Schweizer-Cagianut M, Froesch ER, Hedinger C. Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia). Acta Endocrinol (Copenh). Aug 1980;94(4):529-35. [Medline].
Schweizer-Cagianut M, Salomon F, Hedinger CE. Primary adrenocortical nodular dysplasia with Cushing's syndrome and cardiac myxomas. A peculiar familial disease. Virchows Arch A Pathol Anat Histol. 1982;397(2):183-92. [Medline].
Shenoy BV, Carpenter PC, Carney JA. Bilateral primary pigmented nodular adrenocortical disease. Rare cause of the Cushing syndrome. Am J Surg Pathol. May 1984;8(5):335-44. [Medline].
Sigg C, Schweizer-Cagianut M, Hedinger C. [Morphology of the adrenal gland in primary hyperaldosteronism]. Schweiz Med Wochenschr. Mar 12 1983;113(10):357-67. [Medline].
Stratakis CA, Carney JA, Lin JP, et al. Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2. J Clin Invest. Feb 1 1996;97(3):699-705. [Medline].
Stratakis CA, Kirschner LS, Carney JA. Carney complex: diagnosis and management of the complex of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas. Am J Med Genet. Nov 2 1998;80(2):183-5. [Medline].
Stratakis CA, Papageorgiou T, Premkumar A, et al. Ovarian lesions in Carney complex: clinical genetics and possible predisposition to malignancy. J Clin Endocrinol Metab. Nov 2000;85(11):4359-66. [Medline].
Stratakis CA, Papageorgiou T, Premkumar A, et al. Ovarian lesions in Carney complex: clinical genetics and possible predisposition to malignancy. J Clin Endocrinol Metab. Nov 2000;85(11):4359-66. [Medline].
Valverde K, Henderson M, Smith CR, Tallett S, Chan HS. Typical and atypical Carney's triad presenting with malignant hypertension and papilledema. J Pediatr Hematol Oncol. Nov 2001;23(8):519-24. [Medline].
Vatterott PJ, Seward JB, Vidaillet HJ, Su WP, Oftedahl GL. Syndrome cardiac myxoma: more than just a sporadic event. Am Heart J. Oct 1987;114(4 Pt 1):886-9. [Medline].
Vidaillet HJ Jr, Seward JB, Fyke FE 3rd, Su WP, Tajik AJ. "Syndrome myxoma": a subset of patients with cardiac myxoma associated with pigmented skin lesions and peripheral and endocrine neoplasms. Br Heart J. Mar 1987;57(3):247-55. [Medline].
Wahid ST, Jones R, Chawla SL, Connolly VM, Kelly WF, Bilous RW. A new variant of Carney's triad: phaeochromocytoma and chondrosarcoma. Postgrad Med J. Aug 2001;77(910):527-8. [Medline].
Wilsher ML, Roche AH, Neutze JM, Synek BJ, Holdaway IM, Nicholson GI. A familial syndrome of cardiac myxomas, myxoid neurofibromata, cutaneous pigmented lesions, and endocrine abnormalities. Aust N Z J Med. Jun 1986;16(3):393-6. [Medline].
Winkelmann RK, Carney JA. Cutaneous neuropathology in multiple endocrine neoplasia, type 2b. J Invest Dermatol. Nov 1982;79(5):307-12. [Medline].
Young WF Jr, Carney JA, Musa BU, Wulffraat NM, Lens JW, Drexhage HA. Familial Cushing's syndrome due to primary pigmented nodular adrenocortical disease. Reinvestigation 50 years later. N Engl J Med. Dec 14 1989;321(24):1659-64. [Medline].
Further Reading
Keywords
Carney CNC, Carney complex, Carney's complex, Carney's syndrome, CNC type I, CNC type II, Carney myxoma-endocrine complex, lentigines, blue nevi syndrome, LAMB syndrome, nevi, atrial myxoma, myxoid neurofibromata, ephelides, NAME syndrome, complex of myxomas, spotty pigmentation, endocrine overactivity, multiple neoplasia and lentiginosis syndrome, primary pigmented nodular adrenocortical disease, PPNAD, hypercortisolism, nonendocrine tumors, endocrine tumors,
Treatment & Medication: Carney Syndrome