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Autosomal dominant transmission of the NAME syndrome (nevi, atrial myxoma, mucinosis of the skin and endocrine overactivity)

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Summary

A family affected in two generations with the NAME syndrome is described, and the literature on this phenotype reviewed. The term “NAME” was originally proposed as an acronym for “nevi, atrial myxoma, myxoid neurofibromata and ephelides”. However, in order to give a more comprehensive description of this syndrome, we recommend the following alternative interpretation of “NAME”: nevi, atrial myxoma, mucinosis of the skin, endocrine overactivity. Previous family observations suggested a dominant, and probably autosomal, inheritance but an X-linked dominant transmission could not be excluded. We describe male-to-male transmission, strongly supporting the concept of the autosomal inheritance of this trait.

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References

  • André P, Janin-Mercier A, Souteyrand P, Avril MF, Payne CR, Citron B, Ortonne JP (1988) Lentiginose cutanéo-muqueuse, éphélides et myxome cardiaque. Ann Dermatol Venereol 115:151–157

    Google Scholar 

  • Atherton DJ, Pitcher DW, Wells RS, MacDonald DM (1980) A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata and atrial myxoma: the NAME syndrome. Br J Dermatol 103:421–429

    CAS  PubMed  Google Scholar 

  • Attum AA, Johnson GS, Masri Z, Girardet R, Lansing AM (1987) Malignant clinical behavior of cardiac myxomas and “myxoid imitators”. Ann Thorac Surg 44:217–222

    Google Scholar 

  • Balk AH, Wagenam SS, Bruschke AVG (1979) Bilateral cardiac myxomas and peripheral myxomas in a patient with recent myocardial infarction. Am J Cardiol 44:767–770

    Google Scholar 

  • Barlow JF, Abu-Gazeleh S, Tarn GE, Wirtz PS, Ofstein LC, O'Brien CP, Woods GL, Drymalski WG (1983) Myxoid tumor of the uterus and right atrial myxomas. S Dakota J Med 36:9–13

    Google Scholar 

  • Beohr PC, Malhotra V, Khalilullah M, Khanna SK, Narayanan PS, Gupta MP, Rani S (1983) Cardiac myxomas — a clinicopathological study. Indian Heart J 35:341–344

    Google Scholar 

  • Bulkley BH, Hutchins GM (1979) Atrial myxomas: a fifty year review. Am Heart J 97:639–643

    Google Scholar 

  • Carney JA, Gordon H, Carpenter PC, Lenoy BV, Go VLW (1985) The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 64:270–283

    Google Scholar 

  • Carney JA, Hruska LS, Beauchamp GD, Gordon H (1986a) Dominant inheritance of the complex of myxomas, spotty pigmentation and endocrine overactivity. Mayo Clin Proc 61:165–172

    Google Scholar 

  • Carney JA, Headington JT, Su WPD (1986b) Cutaneous myxomas. A major component of the complex of myxomas, spotty pigmentation and endocrine overactivity. Arch Dermatol 122:790–798

    Google Scholar 

  • Chandraratna PAN, San Pedro S, Elkins RC, Grantham N (1977) Echocardiographic, angiocardiographic, and surgical correlations in right ventricular myxoma simulating valvar pulmonic stenosis. Circulation 55:619–622

    Google Scholar 

  • Chopra P (1984) Intracardiac myxoma. Jpn Heart J 26:457–462

    Google Scholar 

  • Crawford FA Jr, Selby JH, Watson D, Joransen J (1978) Unusual aspects of atrial myxoma. Ann Surg 188:240–244

    Google Scholar 

  • Danoff A, Jormak S, Lorber D, Fleischer N (1987) Adrenocortical micronodular dysplasia, cardiac myxomas, lentigines and spindle cell tumors. Report of a kindred. Arch Intern Med 147:443–448

    Google Scholar 

  • Dashkoff N, Boersma RB, Nanda NC, Gramiak R, Andersen MN, Subramanian S (1978) Bilateral atrial myxomas. Echocardiographic considerations. Am J Med 68:361–366

    Google Scholar 

  • De Moor P, Roels H, Delaere K, Crabbe J (1965) Unusual case of adrenocortical hyperfunction. J Clin Endocrinol Metab 25:612–620

    Google Scholar 

  • Fligiel Z, Kaneko M, Leiter E (1976) Bilateral Sertoli cell tumor of testes with feminizing and masculinizing activity occurring in a child. Cancer 38:1853–1858

    Google Scholar 

  • Frankenfeld RH, Waters CH, Steiner RC (1960) Bilateral myxomas of the heart. Ann Intern Med 53:827–838

    Google Scholar 

  • Honey M, Axelrad MA (1962) Intracardiac endodermal heterotopia. Br Heart J 24:667–670

    Google Scholar 

  • Kracht J, Tamm J (1960) Bilaterale kleinknotige Adenomatose der Nebennierenrinde bei Cushing-Syndrom. Virchows Arch Pathol Anat 333:1–9

    Google Scholar 

  • Laboux L, Mussini-Montpellier J, Michaud JL, Gaillard F, Pelleray C, Cornet E (1978) Myxome du ventricule droit chez des jumeaux monozygotes. Ablation chirurgicale. Arch Mal Coeur 71:953–959

    Google Scholar 

  • Leedman PJ, Cohen AK, Matz LR (1986) The complex of myxomas, spotty pigmentation and endocrine overactivity. Clin Endocrinol 25:527–534

    Google Scholar 

  • Levin ME (1966) The development of bilateral adenomatous adrenal hyperplasia in a case of Cushing's syndrome of eighteen years' duration. Am J Med 40:318–324

    Google Scholar 

  • Lund HZ (1957) Tumors of the skin. Atlas of tumor pathology, sect 1, fasc 2. (Publication no 276) Armed Forces Institute of Pathology, Washington, DC, pp 274–280

    Google Scholar 

  • Morgan DL, Palazola J, Reed W, Bell HH, Kindred LH, Beauchamp GD (1977) Left heart myxomas. Am J Cardiol 40:611–614

    Google Scholar 

  • Müller GA, Klyscz T, Markovic-Lipkovski J, Seipel L, Waller HD (1987) Phänotypische Heterogenität kardialer Myxome. Klin Wochenschr 65:912–919

    Google Scholar 

  • Pavie A, Escande G, Cham B, Bachrel B, Barra J, Villemot JP, Gandjbakhch I, Cabrol C (1981) Les myxomes de l'oreille droite. A propos de 3 observations. Revue de la littérature. Arch Mal Coeur 74:265–272

    Google Scholar 

  • Peterson LL, Serril WS (1984) Lentiginosis associated with a left atrial myxoma. J Am Acad Dermatol 10:337–340

    Google Scholar 

  • Proppe KG, Sculley RE (1980) Large-cell calcifying Sertoli cell tumor of the testis. Am J Clin Pathol 74:607–619

    Google Scholar 

  • Reed OM, Captain MC, Ramsey J, Fitzpatrick JE (1986) Cutaneous lentiginosis with atrial myxomas. J Am Acad Dermatol 15:398–402

    Google Scholar 

  • Rees JR, Ross FGM, Keen G (1973) Lentiginosis and left atrial myxoma. Br Heart J 35:874–876

    Google Scholar 

  • Rhodes AR, Silverman RA, Harrist TJ, Perez-Atayde AR (1984) Mucocutaneous lentigines, cardiomucocutaneous myxomas and multiple blue nevi: the LAMB syndrome. J Am Acad Dermatol 10:72–82

    CAS  PubMed  Google Scholar 

  • Roper CL, Camp FA, Kempson RL (1965) Atrial myxoma associated with fibroadenomas of the breast. Missouri Med 62:113–116

    Google Scholar 

  • Rosenzweig JL, Lawrence DA, Vogel DL, Costa J, Gorden P (1982) Adrenocorticotropin-independent hypercortisolemia and testicular tumors in a patient with pituitary tumor and gigantism. J Clin Endocrinol Metab 55:421–427

    Google Scholar 

  • Rowe MH, Mullany CJ, Leitl G (1988) Spotty pigmentation and atrial myxoma: a case report of Carney's complex. Med J Aust 149:40–42

    Google Scholar 

  • Ruder HJ, Loriaux DL, Lipsett MB (1974) Severe osteopenia in young adults associated with Cushing's syndrome due to micronodular adrenal disease. J Clin Endocrinol Metab 39:1138–1147

    Google Scholar 

  • Schweizer-Cagianut M, Salomon F, Hedinger CE (1982) Primary adrenocortical nodular dysplasia with Cushing's syndrome and cardiac myxomas. A peculiar familial disease. Virchows Arch Pathol Anat 397:183–192

    Google Scholar 

  • Shenoy BV, Carpenter PC, Carney JA (1984) Bilateral primary pigmented nodular adrenocortical disease. Rare cause of the Cushing syndrome. Am J Surg Pathol 8:335–344

    Google Scholar 

  • Truhan PA, Roenigk HH (1986) The cutaneous mucinoses. J Am Acad Dermatol 14:1–18

    Google Scholar 

  • Vatterott PJ, Seward JB, Vidaillet HJ, Su WPD, Oftedahl GL (1987) Syndrome cardiac myxoma: more than just a sporadic event. Am Heart J 114:886–889

    Google Scholar 

  • Wilsher ML, Roche AHG, Neutze JM, Synek BJL, Holdaway IM, Nicholson GI (1986) A familial syndrome of cardiac myxomas, myxoid neurofibromata, cutaneous pigmented lesions, and endocrine abnormalities. Aust NZ J Med 16:393–396

    Google Scholar 

  • Zajtchuk R, Fitterer JD, Strevey TE, Nelson WP (1975) Bilateral atrial myxomas: preoperative diagnosis and successful removal. J Thorac Cardiovasc Surg 69:291–294

    Google Scholar 

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  1. Department of Dermatology, University of Nijmegen, Javastraat 104, NL-6524, Nijmegen, MJ, The Netherlands

    R. J. J. Koopman & R. Happle

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  1. R. J. J. Koopman
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  2. R. Happle
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Koopman, R.J.J., Happle, R. Autosomal dominant transmission of the NAME syndrome (nevi, atrial myxoma, mucinosis of the skin and endocrine overactivity). Hum Genet 86, 300–304 (1991). https://doi.org/10.1007/BF00202415

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  • DOI: https://doi.org/10.1007/BF00202415

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