eMedicine Specialties > Dermatology > Internal Medicine

Carney Syndrome

Author: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey; Ali Haider, Department of Dermatology, Albert Einstein College of Medicine of Yeshiva University; Manuel A Cruz, MA, Adjunct Assistant Professor, Department of Pathology, UMDNJ-New Jersey Medical School; Walter HC Burgdorf, MD, Clinical Lecturer, Department of Dermatology, Ludwig Maximilian University, Munich, Germany
Contributor Information and Disclosures

Updated: May 16, 2008

Introduction

Background

Carney complex (CNC) is a familial multiple neoplasia and lentiginosis syndrome. Historically, the complex involved the association of the following conditions: (1) primary pigmented nodular adrenocortical disease (PPNAD), a pituitary-independent, primary adrenal form of hypercortisolism; (2) lentigines, ephelides, and blue nevi of the skin and mucosae; and (3) a variety of nonendocrine and endocrine tumors. The latter include myxomas of the skin, heart, breast, and other sites; psammomatous melanotic schwannoma; growth hormone–producing pituitary adenoma; testicular Sertoli-cell tumor; and, possibly, other benign and malignant neoplasms and conditions, including tumors of the thyroid gland and ductal adenoma of the breast and acromegaly due to somatomammotroph hyperplasia and adenoma not dependent on growth hormone–releasing hormone.

Although the existence of the complex as an unrecognized inherited syndrome was first suggested in 1985, combinations of several components of the syndrome and their familial occurrence were reported earlier.

The pathologic findings of multiple, small, pigmented adrenocortical nodules and internodular cortical atrophy were described as early as 1949 in the adrenal glands of children and young adults with Cushing syndrome. In 1980, Atherton and colleagues1 described a patient with a vast number of macular pigmented lesions on the skin, which involved the lips; myxoid neurofibroma; and domed blue nevi. Echocardiograms revealed dense echoes arising from the space between the mitral leaflets; these findings were consistent with the histologic features of a myxoma. Atherton suggested the term nevi, atrial myxoma, myxoid neurofibromas, and ephelides (NAME) syndrome.

Rhodes and colleagues2 then described a similar condition for which the term lentigines, atrial myxomas, and blue nevi (LAMB) syndrome was suggested.

Carney3 conducted a review of cases in 40 patients with cardiac myxomas. He found that many of these patients also had multiple pigmented lesions (lentigines and several types of nevi), which affected the lips, as well as PPNAD, which presented as Cushing syndrome. Some of these patients also presented with testicular tumors, fibroadenomas, and pituitary adenomas. He concluded that these rare conditions were unlikely to occur together by chance and that they represented a unique syndrome. Endocrine overactivity is also part of this syndrome.4 In fact, corticotropin hormone–independent Cushing syndrome due to primary pigmented nodular adrenocortical disease is an important syndrome characteristic.

The eMedicine article Carney Complex also may be of interest.

Pathophysiology

The Carney myxoma-endocrine complex is believed to exist in at least 2 genetically distinct forms: One form can be mapped to chromosome 17, and the other, to chromosome 2. The chromosome 17 form, designated CNC type I, is due to mutations in the PRKAR1A gene. Further research is needed to delineate the exact genetic mutations in CNC type II.

CNC genes are associated with genomic instability as cell lines established from CNC tumors accumulate chromosomal changes, including telomeric associations (tas) and dicentric chromosomes. Myxomas in CNC show a high rate of apoptosis, in concordance with the cytogenetic abnormalities in these tumors. Both PPNAD (a primary bilateral adrenal disorder leading to Cushing syndrome) and myxomatous tumors from patients with CNC stain positive for synaptophysin, a neuroendocrine (NE) marker, and the lesions have NE properties on electron microscopy.

An inherited disposition to cardiac myxoma development is seen in these CNC patients.5 They have a mutation in the PRKAR1A gene, which encodes the regulatory R1alpha subunit of protein kinase A—a significant component of the cyclic adenosine monophosphate (cAMP) signaling pathway. Genetically engineered mutant Prkar1a mouse models also show a tendency to develop tumors. PPNAD, a rare cause of corticotropin-independent Cushing syndrome, may be part of CNC. A small intronic deletion of the PRKAR1A gene is a low-penetrance cause of mainly PPNAD; it is the first PRKAR1A genetic defect to have an association with a specific phenotype.6 PPNAD and pituitary adenoma in a boy with sporadic CNC was found to be due to a novel, de novo paternal PRKAR1A mutation (R96X).7

Frequency

United States

Cardiac myxomas are the most common primary cardiac tumor in the general population and occur with a frequency of 7 cases per 10,000 individuals. Myxomas occurring as part of CNC account for 7% of all cardiac myxomas.

International

More than 150 patients have been identified as having Carney syndrome since its recognition as a complex in 1985. Cases in persons with only limited involvement may not be reported. The syndrome is distributed worldwide.

Mortality/Morbidity

  • Cardiac myxomas account for a mortality rate of 25% in patients with this syndrome.
  • Cardiac myxomas are a silent killer, causing major disability with its embolic capacity and even sudden death.
  • The psammomatous melanotic schwannomas are typically benign; however, as many as 10% of cases can metastasize.

Race

Most patients who are affected with CNC are white, although the disease has been described in blacks.

Sex

Males and females are affected equally.

Age

The mean patient age at diagnosis is 10-20 years.

Clinical

History

Cutaneous manifestations are a major clue in the diagnosis of this condition. These manifestations include Cushing syndrome and skin changes associated with pigmentation.

  • Symptoms of Cushing syndrome may develop in young patients.
    • The skin is fragile and thin.
    • Patients bruise easily and heal poorly.
    • The bones are weakened, and routine activities such as bending, lifting, or rising from a chair may lead to backaches and rib and spinal column fractures.
    • Severe fatigue, muscle weakness, high blood pressure, and high blood sugar levels may be present.
    • Irritability, anxiety, and depression are also common.
    • Women may have menstrual periods that become irregular or stop.
    • Men have decreased fertility with a diminished or absent desire for sex.
  • Symptoms of a prolactin-secreting tumor include hypogonadism (eg, amenorrhea, impotence) associated with symptoms of increased prolactin levels (eg, galactorrhea) in female patients.
  • Symptoms of acromegaly include headaches and visual abnormalities.
  • Symptoms of hyperparathyroidism include fatigue, anemia, muscle weakness, joint pain, constipation with nausea, frequent urination (sometimes bloody), mood change with confusion, abdominal pain due to ulcers, flank or back pain due to stones, bone pain due to bone erosions or fractures, and hypertension.
  • Atrial myxomas may create ball-valve obstructions that cause unexpected syncopal attacks, cardiac insufficiency, and sudden death in apparently healthy young children and adults.
  • Right-sided myxomas with extramedullary hematopoiesis and ossification in CNC has been described in a patient who also had a tiny eyelid cutaneous myxoma, multiple hypoechoic thyroid follicular adenomas, and multiple small testicular tumors.8

Physical

Myxomas recur in approximately 12-22% of familial cases and in about 1-2% of sporadic cases.

  • Atrial myxomas
    • On physical examination, an accentuated first heart sound can be appreciated in patients with cardiac myxomas.
    • In adults, myxomas are the most common primary tumor of the heart. They arise in any of the 4 chambers or on the heart valves; however, about 90% are located in the atria. Myxomas in the atria have a left-to-right ratio of approximately 4:1.
    • Myxomas are mostly single and rarely multiple in several chambers.
    • Tumors can be 1-10 cm or larger in diameter.
    • The tumors can be sessile or pedunculated. In addition, the pedunculated form of atrial myxomas is often sufficiently mobile to move into or sometimes through the atrioventricular valves during diastole. Sometimes, such mobility exerts a wrecking-ball effect on the valve leaflets.
  • Multiple nevi, diffuse facial lentigines, and mucosal labial pigmentation
    • The classic presentation is a spotty pigmentation on the face, the chest and shoulders, and the vermilion border of the lips and conjunctiva.
    • The pigmentation can be tan or dark brown to black.
    • The lesions can be irregularly shaped or sharply delineated, and they can be small or several millimeters in diameter.
  • Subcutaneous myxoid neurofibromas and mammary fibroadenosis
    • Cutaneous myxomas have a predilection for the eyelids and external ear canals, although they may affect any part of the skin.
    • The mammary myxoid fibroadenomas in the complex are often multiple and bilateral; these fibroadenomas are an unusual finding in an otherwise normal breast.
  • Signs of acromegaly
    • Macroglossia may be present.
    • Prominent jaw and frontal bossing may be observed.
    • The patient may have large spadelike hands.
    • Skin tags may be observed.
    • Hyperhidrosis may occur.
  • Signs of Cushing syndrome
    • The skin is fragile and thin.
    • Patients bruise easily and heal poorly.
    • Purplish pink stretch marks are noted on the abdomen, thighs, buttocks, arms, and breasts.
    • Upper-body obesity, a rounded face, increased fat around the neck, and thin arms and legs are observed.
    • Children tend to be obese, with slowed growth rates.
    • Women usually have excessive hair growth on their face, neck, chest, abdomen, and thighs.
  • Endocrine involvement in CNC also includes 3 types of testicular tumors: large-cell calcifying Sertoli-cell tumor (one of the rarest testicular neoplasms), adrenocortical rests, and Leydig-cell tumor.
    • These tumors appear with testicular masses, and about one third of affected male patients have them.
    • Large-cell calcifying Sertoli-cell tumors may also appear as bilateral masses. They can also secrete estrogens, resulting in precocious puberty, gynecomastia, or both.
  • As many as 75% of patients with CNC may have multiple thyroid nodules.
    • The nodules can appear as a goiter or thyroid mass when palpated on physical examination.
    • Patients can also present with symptoms of hyperthyroidism or hypothyroidism.
  • Psammomatous melanotic schwannoma most frequently occurs in the gastrointestinal tract (esophagus and stomach) and paraspinal sympathetic chain. Masses can be palpated on abdominal or paraspinal examination.

Causes

CNC is inherited as an autosomal dominant trait, and the genes responsible have been mapped to bands 2p16 and 17q22-24.

  • Because the features of CNC, such as the paradoxical responses to endocrine signals, are similar to those of McCune-Albright syndrome and other conditions, the genes implicated in cyclic nucleotide-dependent signaling have been implicated.
  • Kirschner and colleagues9 examined families with CNC.
    • They first detected a loss of heterozygosity (LOH) in the vicinity of the PRKAR1A gene that encoded the protein kinase A regulatory subunit 1-alpha (R1alpha), which was mapped to chromosome 17q.
    • They subsequently identified 3 unrelated kindreds with an identical mutation in the coding region of PRKAR1A.
    • An analysis of PRKAR1A activity demonstrated that basal activity is decreased and cAMP-stimulated activity is increased in CNC tumors compared with non-CNC tumors.
    • Kirschner et al concluded from these findings that germline mutations in PRKAR1A, an apparent tumor suppressor gene, are responsible for the CNC phenotype in a subset of patients with this disease.
  • Kirschner et al10 also screened the mutations present in 54 CNC kindreds.
    • In 14 of the mutations that were mapped to the PRKAR1A locus, they found a premature stop codon; one altered the initiator ATG codon.
    • The messenger RNAs (mRNAs) resulting from this mutation were unstable, and they rapidly decayed.
    • The PRKAR1A products were absent in the affected cells.
    • These studies were the first to show a disease with mutations in the protein kinase A holoenzymes, critical components of the signaling pathway.

More on Carney Syndrome

Overview: Carney Syndrome
Differential Diagnoses & Workup: Carney Syndrome
Treatment & Medication: Carney Syndrome
Follow-up: Carney Syndrome
Multimedia: Carney Syndrome
References
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References

  1. 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].

  2. 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].

  3. Carney JA. Differences between nonfamilial and familial cardiac myxoma. Am J Surg Pathol. Jan 1985;9(1):53-5. [Medline].

  4. 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].

  5. Wilkes D, Charitakis K, Basson CT. Inherited disposition to cardiac myxoma development. Nat Rev Cancer. Feb 2006;6(2):157-65. [Medline].

  6. Groussin L, Horvath A, Jullian E, Boikos S, Rene-Corail F, Lefebvre H, 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].

  7. Urban C, Weinhausel A, Fritsch P, Sovinz P, Weinhandl G, Lackner H, 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].

  8. Lee B, Sir JJ, Park SW, Kim SB, Nah JC, Kang YK, et al. Right-sided myxomas with extramedullary hematopoiesis and ossification in Carney complex. Int J Cardiol. Jan 28 2008;[Medline].

  9. Kirschner LS, Carney JA, Pack SD, Taymans SE, Giatzakis C, Cho YS, 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].

  10. 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].

  11. Abdelmalek NF, Gerber TL, Menter A. Cardiocutaneous syndromes and associations. J Am Acad Dermatol. Feb 2002;46(2):161-83; quiz 183-6. [Medline].

  12. Arce B, Licea M, Hung S, Padron R. Familial Cushing's syndrome. Acta Endocrinol (Copenh). Jan 1978;87(1):139-47. [Medline].

  13. Barlow JF, Abu-Gazeleh S, Tam GE, Wirtz PS, Ofstein LC, O'Brien CP, et al. Myxoid tumor of the uterus and right atrial myxomas. S D J Med. Jul 1983;36(7):9-13. [Medline].

  14. 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].

  15. 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].

  16. 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].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

  21. Casey M, Mah C, Merliss AD, Kirschner LS, Taymans SE, Denio AE, et al. Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation. Dec 8 1998;98(23):2560-6. [Medline].

  22. 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].

  23. 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].

  24. 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].

  25. Handley J, Carson D, Sloan J, Walsh M, Thornton C, Hadden D, 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].

  26. 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].

  27. 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].

  28. 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].

  29. Kennedy RH, Waller RR, Carney JA. Ocular pigmented spots and eyelid myxomas. Am J Ophthalmol. Nov 15 1987;104(5):533-8. [Medline].

  30. Kirschner LS, Carney JA, Pack SD, Taymans SE, Giatzakis C, Cho YS, 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].

  31. 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].

  32. 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].

  33. 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].

  34. Mai PL, Korde L, Kramer J, Peters J, Mueller CM, Pfeiffer S, 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. 2007;1:9. [Medline].

  35. McCarthy PM, Piehler JM, Schaff HV, Pluth JR, Orszulak TA, Vidaillet HJ Jr, et al. The significance of multiple, recurrent, and "complex" cardiac myxomas. J Thorac Cardiovasc Surg. Mar 1986;91(3):389-96. [Medline].

  36. 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].

  37. Nwokoro NA, Korytkowski MT, Rose S, Gorin MB, Penles Stadler M, Witchel SF, et al. Spectrum of malignancy and premalignancy in Carney syndrome. Am J Med Genet. Dec 31 1997;73(4):369-77. [Medline].

  38. 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].

  39. 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].

  40. 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].

  41. Proppe KH, Scully RE. Large-cell calcifying Sertoli cell tumor of the testis. Am J Clin Pathol. Nov 1980;74(5):607-19. [Medline].

  42. Rees JR, Ross FG, Keen G. Lentiginosis and left atrial myxoma. Br Heart J. Aug 1973;35(8):874-6. [Medline].

  43. 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. 1984;6 Suppl:184-8. [Medline].

  44. 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].

  45. 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].

  46. 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].

  47. 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].

  48. Stratakis CA, Carney JA, Lin JP, Papanicolaou DA, Karl M, Kastner DL, 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].

  49. 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].

  50. Stratakis CA, Papageorgiou T, Premkumar A, Pack S, Kirschner LS, Taymans SE, 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].

  51. Stratakis CA, Papageorgiou T, Premkumar A, Pack S, Kirschner LS, Taymans SE, 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].

  52. 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].

  53. 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].

  54. 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].

  55. 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].

  56. 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].

  57. Winkelmann RK, Carney JA. Cutaneous neuropathology in multiple endocrine neoplasia, type 2b. J Invest Dermatol. Nov 1982;79(5):307-12. [Medline].

  58. 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].

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Further Reading

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Keywords

CNC, CNC type I, CNC type II, Carney complex, Carney's complex, Carney's syndrome, 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, PRKAR1A

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey
Santiago A Centurion, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Sigma Xi
Disclosure: Nothing to disclose.

Ali Haider, Department of Dermatology, Albert Einstein College of Medicine of Yeshiva University
Disclosure: Nothing to disclose.

Manuel A Cruz, MA, Adjunct Assistant Professor, Department of Pathology, UMDNJ-New Jersey Medical School
Manuel A Cruz, MA is a member of the following medical societies: Sigma Xi
Disclosure: Nothing to disclose.

Walter HC Burgdorf, MD, Clinical Lecturer, Department of Dermatology, Ludwig Maximilian University, Munich, Germany
Walter HC Burgdorf, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Society of Dermatopathology, International Society of Dermatopathology, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Medical Editor

R Stan Taylor, MD, Professor of Dermatology, University of Texas Southwestern Medical School; Director of Skin Surgery and Oncology Clinic, Department of Dermatology, University of Texas Southwestern Medical Center
R Stan Taylor, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Medical Association, American Society for Dermatologic Surgery, Christian Medical & Dental Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Christen M Mowad, MD, Associate Professor, Department of Dermatology, Geisinger Medical Center
Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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