{{distinguish|Carney triad}} {{Infobox medical condition (new) | name = Carney complex | synonyms = LAMB syndrome | image = Carney complex with main associated diseases.png | caption = Carney complex with main associated diseases: Lentiginosis, myxoma of skin and heart, and primary pigmented nodular adrenocortical disease (PPNAD) | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} '''Carney complex''' and its subsets '''LAMB syndrome'''<ref name="Schwartz20110502">{{EMedicine|article|1097150|Carney Syndrome}}</ref> and '''NAME syndrome'''<ref name="Schwartz20110502" /> are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity.<ref name="Carney1985">{{Cite journal | last1 = Carney | first1 = J. | last2 = Gordon | first2 = H. | last3 = Carpenter | first3 = P. | last4 = Shenoy | first4 = B. | last5 = Go | first5 = V. | title = The complex of myxomas, spotty pigmentation, and endocrine overactivity | journal = Medicine | volume = 64 | issue = 4 | pages = 270–283 | year = 1985 | pmid = 4010501 | doi=10.1097/00005792-198507000-00007| s2cid = 20522398 | doi-access = free }}</ref><ref name="McCarthy1986">{{Cite journal | last1 = McCarthy | first1 = P. | last2 = Piehler | first2 = J. | last3 = Schaff | first3 = H. | last4 = Pluth | first4 = J. | last5 = Orszulak | first5 = T. | last6 = Vidaillet Jr | first6 = H. | last7 = Carney | first7 = J. | title = The significance of multiple, recurrent, and "complex" cardiac myxomas | journal = The Journal of Thoracic and Cardiovascular Surgery | volume = 91 | issue = 3 | pages = 389–396 | year = 1986 | pmid = 3951243 | doi = 10.1016/s0022-5223(19)36054-4| doi-access = free }}</ref> It is distinct from Carney triad. Approximately 7% of all cardiac myxomas are associated with Carney complex.<ref name="Reynen1995">{{Cite journal | last1 = Reynen | first1 = K. | title = Cardiac Myxomas | journal = New England Journal of Medicine | volume = 333 | issue = 24 | pages = 1610–1617 | year = 1995 | pmid = 7477198 | doi = 10.1056/NEJM199512143332407}}</ref>
==Presentation== The spotty skin pigmentation and lentigines occur most commonly on the face, especially on the lips, eyelids, conjunctiva and oral mucosa.<ref name="McCarthy1986"/> Cardiac myxomas may lead to embolic strokes and heart failure<ref name="Reynen1995"/> and may present with fever, joint pain, shortness of breath, diastolic rumble and tumor plop. Myxomas may also occur outside the heart, usually in the skin and breast. Endocrine tumors may manifest as disorders such as Cushing syndrome. The most common endocrine gland manifestation is an ACTH-independent Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD).<ref>{{cite journal |url= https://www.ncbi.nlm.nih.gov/books/NBK507877/ | title= Carney Complex |website= National Center for Biotechnology Information, U.S. National Library of Medicine | year= 2022 | pmid= 29939654 | access-date= 5 July 2021| last1= Vindhyal | first1= M. R. | last2= Elshimy | first2= G. | last3= Elhomsy | first3= G. }}</ref><ref>{{Cite journal |last=Qian |first=Tai |last2=Li |first2=Chun-Yang |last3=Liu |first3=Qian-Long |last4=Yang |first4=Zhe |last5=Cui |first5=Wei |date=October 19, 2025 |title=Carney Complex During Six-Year Follow-Up and Its Association With Attention-Deficit Hyperactivity Disorder: A Case Report. |url=https://www.cureus.com/articles/410449-carney-complex-during-six-year-follow-up-and-its-association-with-attention-deficit-hyperactivity-disorder-a-case-report#!/authors |journal=Cureus |volume=17 |issue=10}}</ref> Primary pigmented nodular adrenocortical disease (PPNAD) is a rare disorder characterized by the overproduction of the hormone cortisol by the adrenal glands. Elevated cortisol levels can lead to the development of Cushing syndrome, independent of adrenocorticotropic hormone (ACTH) regulation. Clinical features of Cushing syndrome associated with PPNAD may include central (upper body) weight gain, growth retardation in children, decreased bone density, skin fragility, fatigue, and other systemic health complications.<ref name="MedlinePlusCarney">{{cite web |title=Carney complex |url=https://medlineplus.gov/download/genetics/condition/carney-complex.pdf |website=MedlinePlus Genetics |publisher=U.S. National Library of Medicine |access-date=April 12, 2026}}</ref> {|class=wikitable align=right !Manifestations of Carney complex<ref name="pmid25298879">{{cite journal| author=Birla S, Aggarwal S, Sharma A, Tandon N| title=Rare association of acromegaly with left atrial myxoma in Carney's complex due to novel PRKAR1A mutation. | journal=Endocrinol Diabetes Metab Case Rep | year= 2014 | volume= 2014 | issue= | pages= 140023 | pmid=25298879 | doi=10.1530/EDM-14-0023 | pmc=4174593 }} </ref> ! Percentage<ref name="pmid25298879"/> |- | Lentiginosis || 60-70% |- | Cardiac myxoma || 30-60% |- | Primary pigmented nodular<br>adrenocortical disease (PPNAD) || 25-60% |- | Ovarian cyst || 20-67% |- | Skin myxoma || 20-63% |- | Testicular tumor || 33-56% |- | Ductal carcinoma of the breast || 25% |- | Thyroid tumor || 10-25% |- | Melanotic schwannoma || 8-18% |- | Acromegaly || 10% |- | Osteochondrotic myxoma || <10% |- | Multiple blue nevi || |} The LAMB acronym refers to lentigines, atrial myxomas, and blue nevi.<ref name="Schwartz20110502"/> NAME refers to nevi, atrial myxoma, myxoid neurofibromas, and ephelides.<ref name="Schwartz20110502"/>
Testicular cancer, particularly Sertoli cell type, is associated with Carney syndrome.<ref>Campbell Walsh urology, 10th edition, page 1693</ref> Thyroid and pancreas cancer may also occur.<ref name="Gaujoux2011">{{Cite journal |vauthors=Gaujoux S, Tissier F, Ragazzon B, Rebours V, Saloustros E, Perlemoine K, Vincent-Dejean C, Meurette G, Cassagnau E, Dousset B, Bertagna X, Horvath A, Terris B, Carney JA, Stratakis CA, Bertherat J | title = Pancreatic ductal and acinar cell neoplasms in Carney complex: a possible new association. | journal = J Clin Endocrinol Metab | volume = 96 | issue = 11 | pages = E1888–95 | year = 2011 | pmid = 21900385 | doi=10.1210/jc.2011-1433 | pmc=3205895}}</ref><ref name="Bano2016">{{Cite book |vauthors=Bano G, Hodgson S | chapter = Diagnosis and Management of Hereditary Thyroid Cancer | title = Rare Hereditary Cancers | journal = Recent Results in Cancer Research. Fortschritte der Krebsforschung. Progres dans les Recherches Sur le Cancer | series = Recent Results in Cancer Research | volume = 205 | pages = 29–44 | year = 2016 | pmid = 27075347 | doi=10.1007/978-3-319-29998-3_3| isbn = 978-3-319-29996-9 }}</ref>
Although J Aidan Carney also described Carney's triad it is entirely different.<ref>{{cite journal |author=Gaissmaier C |title=Carney complex |journal=Circulation |volume=100 |issue=25 |pages=e150 |date=December 1999 |pmid=10604916|doi=10.1161/01.cir.100.25.e150|doi-access=free }}</ref>
==Pathophysiology== Carney complex is most commonly caused by mutations in the PRKAR1A gene on chromosome 17 (17q23-q24)<ref name="OMIM160980">{{OMIM|160980|Carney Complex, type 1; CNC1}}</ref> which may function as a tumor-suppressor gene. The encoded protein is a type 1A regulatory subunit of protein kinase A. Inactivating germline mutations of this gene are found in 70% of people with Carney complex.<ref>{{cite web |url= https://www.lecturio.com/concepts/cardiac-myxoma/ | title= Cardiac Myxoma | website= The Lecturio Medical Concept Library |access-date= 6 July 2021}}</ref>
Less commonly, the molecular pathogenesis of Carney complex is a variety of genetic changes at chromosome 2 (2p16).<ref name="Stratakis2001">{{Cite journal | last1 = Stratakis | first1 = C. A. | last2 = Kirschner | first2 = L. S. | last3 = Carney | first3 = J. A. | title = Clinical and Molecular Features of the Carney Complex: Diagnostic Criteria and Recommendations for Patient Evaluation | journal = Journal of Clinical Endocrinology & Metabolism | volume = 86 | issue = 9 | pages = 4041–4046 | year = 2001 | doi = 10.1210/jc.86.9.4041| pmid = 11549623 | doi-access = free }}</ref><ref>{{OMIM|605244|Carney Complex, type 2; CNC2}}</ref>
Both types of Carney complex are autosomal dominant. Despite dissimilar genetics, there appears to be no phenotypic difference between PRKAR1A and chromosome 2p16 mutations.<ref name="Stratakis2001" />
==Treatment== Cardiac myxomas can be difficult to manage surgically because of recurrence within the heart, often far away from the site of the initial tumor.<ref name="McCarthy1986" /><ref name="Reynen1995" />
==History==
In 1914 an American neurosurgeon, Harvey Cushing, reported on a patient with a pituitary tumour on whom he had operated. The post mortem findings as reported were consistent with Carney complex, though at the time this condition had yet to be described. In 2017 archived tissue from the operation in Cushing's report was subjected to DNA sequencing, revealing an Arg74His (arginine to histidine: guanine (G)-> adenosine (A) transition in the second codon position of the 74th codon in the protein) mutation in the PRKAR1A gene, confirming a diagnosis of Carney complex. Therefore, Cushing's paper appears to be the first report of this complex.<ref name="c216">{{cite journal | last1=Tsay | first1=Cynthia J | last2=Stratakis | first2=Constantine A | last3=Faucz | first3=Fabio Rueda | last4=London | first4=Edra | last5=Stathopoulou | first5=Chaido | last6=Allgauer | first6=Michael | last7=Quezado | first7=Martha | last8=Dagradi | first8=Terry | last9=Spencer | first9=Dennis D | last10=Lodish | first10=Maya | title=Harvey Cushing Treated the First Known Patient With Carney Complex | journal=Journal of the Endocrine Society | publisher=The Endocrine Society | volume=1 | issue=10 | date=2017-09-28 | issn=2472-1972 | doi=10.1210/js.2017-00283 | pages=1312–1321 | pmid=29264456 | doi-access=free }}</ref>
==See also== * Epithelioid blue nevus * List of cutaneous neoplasms associated with systemic syndromes
==References== {{reflist}}
== External links == {{Medical resources | DiseasesDB = | ICD10 = | ICD9 = | ICDO = | OMIM = 160980 | OMIM_mult = {{OMIM|605244||none}} | MedlinePlus = | eMedicineSubj = med | eMedicineTopic = 2941 | MeshID = | Orphanet = 1359 }} * [https://www.ncbi.nlm.nih.gov/books/NBK1286/ GeneReview/UW/NIH entry on Carney complex]
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{{DEFAULTSORT:Carney Complex}} Category:Hereditary cancers Category:Cardiology Category:Melanocytic nevi and neoplasms Category:Syndromes affecting the skin Category:Syndromes affecting the heart Category:Syndromes with tumors