Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Cutaneous adnexal tumors

Jeffrey P North, MD
Timothy H McCalmont, MD
Beth S Ruben, MD
Section Editor
June K Robinson, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Cutaneous adnexal tumors are a large group of benign and malignant neoplasms that exhibit morphologic differentiation towards one of the four primary adnexal structures present in normal skin: hair follicles, sebaceous glands, apocrine glands, and eccrine glands [1,2]. They may occur sporadically or may be markers of rare genetic syndromes, including Birt-Hogg-Dubé syndrome, Brooke-Spiegler syndrome, Cowden syndrome, and Muir-Torre syndrome [3].

This topic will review the clinical features, diagnosis, and treatment of cutaneous adnexal tumors and associated syndromes. Microcystic adnexal carcinoma, sebaceous carcinoma, Muir-Torre syndrome, and Cowden syndrome are discussed separately.

(See "Microcystic adnexal carcinoma".)

(See "Sebaceous carcinoma".)

(See "Muir-Torre syndrome".)


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Aug 23, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Alsaad KO, Obaidat NA, Ghazarian D. Skin adnexal neoplasms--part 1: an approach to tumours of the pilosebaceous unit. J Clin Pathol 2007; 60:129.
  2. Obaidat NA, Alsaad KO, Ghazarian D. Skin adnexal neoplasms--part 2: an approach to tumours of cutaneous sweat glands. J Clin Pathol 2007; 60:145.
  3. Lee DA, Grossman ME, Schneiderman P, Celebi JT. Genetics of skin appendage neoplasms and related syndromes. J Med Genet 2005; 42:811.
  4. Winkelmann RK, Wolff K. Solid-cystic hidradenoma of the skin. Clinical and histopathologic study. Arch Dermatol 1968; 97:651.
  5. Kazakov DV, Kacerovska D, Hantschke M, et al. Cutaneous mixed tumor, eccrine variant: a clinicopathologic and immunohistochemical study of 50 cases, with emphasis on unusual histopathologic features. Am J Dermatopathol 2011; 33:557.
  6. Kazakov DV, McKee PH, Michal M, Kacerovska D. Cutaneous Adnexal Tumors, 1st ed, Lippincott Williams & Wilkins Health, Philadelphia 2012.
  7. Mayer I. Zur Histologie der Hidradenoma. Frankf Z Path 1941; 55:548.
  8. Martinez SR, Barr KL, Canter RJ. Rare tumors through the looking glass: an examination of malignant cutaneous adnexal tumors. Arch Dermatol 2011; 147:1058.
  9. Al-Zaid T, Ditelberg JS, Prieto VG, et al. Trichilemmomas show loss of PTEN in Cowden syndrome but only rarely in sporadic tumors. J Cutan Pathol 2012; 39:493.
  10. Sariya D, Ruth K, Adams-McDonnell R, et al. Clinicopathologic correlation of cutaneous metastases: experience from a cancer center. Arch Dermatol 2007; 143:613.
  11. Mahalingam M, Nguyen LP, Richards JE, et al. The diagnostic utility of immunohistochemistry in distinguishing primary skin adnexal carcinomas from metastatic adenocarcinoma to skin: an immunohistochemical reappraisal using cytokeratin 15, nestin, p63, D2-40, and calretinin. Mod Pathol 2010; 23:713.
  12. Williams K, Shinkai K. Evaluation and management of the patient with multiple syringomas: A systematic review of the literature. J Am Acad Dermatol 2016; 74:1234.
  13. Ciarloni L, Frouin E, Bodin F, Cribier B. Syringoma: A clinicopathological study of 244 cases. Ann Dermatol Venereol 2016; 143:521.
  14. Urban CD, Cannon JR, Cole RD. Eruptive syringomas in Down's syndrome. Arch Dermatol 1981; 117:374.
  15. Oztürk F, Ermertcan AT, Bilaç C, Temiz P. A case report of postpubertal eruptive syringoma triggered with antiepileptic drugs. J Drugs Dermatol 2010; 9:707.
  16. Polat M, Pelitli A, Oztaş P, et al. Eruptive syringoma associated with hyperthyroidism. Skinmed 2010; 8:124.
  17. Guitart J, Rosenbaum MM, Requena L. 'Eruptive syringoma': a misnomer for a reactive eccrine gland ductal proliferation? J Cutan Pathol 2003; 30:202.
  18. Garrido-Ruiz MC, Enguita AB, Navas R, et al. Eruptive syringoma developed over a waxing skin area. Am J Dermatopathol 2008; 30:377.
  19. Yoshimi N, Kurokawa I, Kakuno A, et al. Case of generalized eruptive clear cell syringoma with diabetes mellitus. J Dermatol 2012; 39:744.
  20. Battistella M, Langbein L, Peltre B, Cribier B. From hidroacanthoma simplex to poroid hidradenoma: clinicopathologic and immunohistochemic study of poroid neoplasms and reappraisal of their histogenesis. Am J Dermatopathol 2010; 32:459.
  21. Betti R, Bombonato C, Cerri A, et al. Unusual sites for poromas are not very unusual: a survey of 101 cases. Clin Exp Dermatol 2014; 39:119.
  22. Wilkinson RD, Schopflocher P, Rozenfeld M. Hidrotic ectodermal dysplasia with diffuse eccrine poromatosis. Arch Dermatol 1977; 113:472.
  23. Ullah K, Pichler E, Fritsch P. Multiple eccrine poromas arising in chronic radiation dermatitis. Acta Derm Venereol 1989; 69:70.
  24. Nguyen BT, Lortscher DN, Lee RA. Multiple poromas in a bone marrow transplant recipient: A case report. Dermatol Online J 2012; 18:9.
  25. North JP, Lo J, Landers M. Poromatosis in pregnancy: a case of 8 eruptive poromas in the third trimester. Cutis 2012; 89:81.
  26. Fujii K, Aochi S, Takeshima C, et al. Eccrine poromatosis associated with polychemotherapy. Acta Derm Venereol 2012; 92:687.
  27. Guerin M, Droney T, Keohane C, Fenton S. Eccrine syringofibroadenoma of the eyelid in association with eye prosthesis. Eye (Lond) 2011; 25:1102.
  28. Noguchi M, Akiyama M, Kawakami M, et al. Eccrine syringofibroadenoma developing in a sebaceous naevus. Br J Dermatol 2000; 142:1050.
  29. Poonawalla T, Xia L, Patten S, Stratman EJ. Clouston syndrome and eccrine syringofibroadenomas. Am J Dermatopathol 2009; 31:157.
  30. Heng YK, Pan JY, Tan SH, Tey HL. Eccrine syringofibroadenomatosis of the reactive subtype occurring in chronic poorly-controlled psoriasis. Indian J Dermatol Venereol Leprol 2014; 80:534.
  31. HELWIG EB, HACKNEY VC. Syringadenoma papilliferum; lesions with and without naevus sebaceous and basal cell carcinoma. AMA Arch Derm 1955; 71:361.
  32. Baker GM, Selim MA, Hoang MP. Vulvar adnexal lesions: a 32-year, single-institution review from Massachusetts General Hospital. Arch Pathol Lab Med 2013; 137:1237.
  33. Konstantinova AM, Michal M, Kacerovska D, et al. Hidradenoma Papilliferum: A Clinicopathologic Study of 264 Tumors From 261 Patients, With Emphasis on Mammary-Type Alterations. Am J Dermatopathol 2016; 38:598.
  34. El-Khoury J, Renald MH, Plantier F, et al. Vulvar hidradenoma papilliferum (HP) is located on the sites of mammary-like anogenital glands (MLAGs): Analysis of the photographs of 52 tumors. J Am Acad Dermatol 2016; 75:380.
  35. Kazakov DV, Spagnolo DV, Kacerovska D, Michal M. Lesions of anogenital mammary-like glands: an update. Adv Anat Pathol 2011; 18:1.
  36. Morimura S, Kadono T, Sugaya M, Sato S. Ectopic hidradenoma papilliferum on the abdomen. Eur J Dermatol 2011; 21:278.
  37. Jain D, Siraj F, Grover AK, Garg KK. Hidradenoma papilliferum presenting as an eyelid mass. Ophthal Plast Reconstr Surg 2012; 28:e152.
  38. Rosmaninho AD, de Almeida MT, Costa V, et al. Ectopic hidradenoma papilliferum. Dermatol Res Pract 2010; 2010:709371.
  39. Ramakrishnan R, Chaudhry IH, Ramdial P, et al. Primary cutaneous adenoid cystic carcinoma: a clinicopathologic and immunohistochemical study of 27 cases. Am J Surg Pathol 2013; 37:1603.
  40. Kamalpour L, Brindise RT, Nodzenski M, et al. Primary cutaneous mucinous carcinoma: a systematic review and meta-analysis of outcomes after surgery. JAMA Dermatol 2014; 150:380.
  41. Hansen T, Kingsley M, Mallatt BD, Krishnan R. Extrafacial microcystic adnexal carcinoma: case report and review of the literature. Dermatol Surg 2009; 35:1835.
  42. Chiller K, Passaro D, Scheuller M, et al. Microcystic adnexal carcinoma: forty-eight cases, their treatment, and their outcome. Arch Dermatol 2000; 136:1355.
  43. Duke WH, Sherrod TT, Lupton GP. Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited). Am J Surg Pathol 2000; 24:775.
  44. Suchak R, Wang WL, Prieto VG, et al. Cutaneous digital papillary adenocarcinoma: a clinicopathologic study of 31 cases of a rare neoplasm with new observations. Am J Surg Pathol 2012; 36:1883.
  45. Regauer S. Extramammary Paget's disease--a proliferation of adnexal origin? Histopathology 2006; 48:723.
  46. Chen YH, Wong TW, Lee JY. Depigmented genital extramammary Paget's disease: a possible histogenetic link to Toker's clear cells and clear cell papulosis. J Cutan Pathol 2001; 28:105.
  47. Plewig G. Sebaceous trichofolliculoma. J Cutan Pathol 1980; 7:394.
  48. Rohwedder A, Keminer O, Hendricks C, Schaller J. Detection of HPV DNA in trichilemmomas by polymerase chain reaction. J Med Virol 1997; 51:119.
  49. Stierman S, Chen S, Nuovo G, Thomas J. Detection of human papillomavirus infection in trichilemmomas and verrucae using in situ hybridization. J Cutan Pathol 2010; 37:75.
  50. Leonardi CL, Zhu WY, Kinsey WH, Penneys NS. Trichilemmomas are not associated with human papillomavirus DNA. J Cutan Pathol 1991; 18:193.
  51. Kambiz KH, Kaveh D, Maede D, et al. Human Papillomavirus Deoxyribonucleic Acid may not be Detected in Non-genital Benign Papillomatous Skin Lesions by Polymerase Chain Reaction. Indian J Dermatol 2014; 59:334.
  52. Tsai JH, Huang WC, Jhuang JY, et al. Frequent activating HRAS mutations in trichilemmoma. Br J Dermatol 2014; 171:1073.
  53. Ackerman AB, Reddy VB, Soyer HP. Neoplasms with Follicular Differentiation, 2nd ed, Ardor Scribendi Publishers, New York 2000.
  54. Alomari A, Subtil A, Owen CE, McNiff JM. Solitary and multiple tumors of follicular infundibulum: a review of 168 cases with emphasis on staining patterns and clinical variants. J Cutan Pathol 2013; 40:532.
  55. Kubba A, Batrani M, Taneja A, Jain V. Tumor of follicular infundibulum: an unsuspected cause of macular hypopigmentation. Indian J Dermatol Venereol Leprol 2014; 80:141.
  56. Cheng AC, Chang YL, Wu YY, et al. Multiple tumors of the follicular infundibulum. Dermatol Surg 2004; 30:1246.
  57. Shan SJ, Guo Y. Panfolliculoma and histopathologic variants: a study of 19 cases. Am J Dermatopathol 2014; 36:965.
  58. Chan EF, Gat U, McNiff JM, Fuchs E. A common human skin tumour is caused by activating mutations in beta-catenin. Nat Genet 1999; 21:410.
  59. Lazar AJ, Calonje E, Grayson W, et al. Pilomatrix carcinomas contain mutations in CTNNB1, the gene encoding beta-catenin. J Cutan Pathol 2005; 32:148.
  60. Hernández-Núñez A, Nájera Botello L, Romero Maté A, et al. Retrospective study of pilomatricoma: 261 tumors in 239 patients. Actas Dermosifiliogr 2014; 105:699.
  61. Rothman D, Kendall AB, Baldi A. Giant pilomatrixoma (Malherbe calcifying epithelioma). Arch Surg 1976; 111:86.
  62. Chiaramonti A, Gilgor RS. Pilomatricomas associated with myotonic dystrophy. Arch Dermatol 1978; 114:1363.
  63. Pujol RM, Casanova JM, Egido R, et al. Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome? Pediatr Dermatol 1995; 12:331.
  64. Maeda D, Kubo T, Miwa H, et al. Multiple pilomatricomas in a patient with Turner syndrome. J Dermatol 2014; 41:563.
  65. Blaya B, Gonzalez-Hermosa R, Gardeazabal J, Diaz-Perez JL. Multiple pilomatricomas in association with trisomy 9. Pediatr Dermatol 2009; 26:482.
  66. Hamahata A, Kamei W, Ishikawa M, et al. Multiple pilomatricomas in Kabuki syndrome. Pediatr Dermatol 2013; 30:253.
  67. Cambiaghi S, Ermacora E, Brusasco A, et al. Multiple pilomatricomas in Rubinstein-Taybi syndrome: a case report. Pediatr Dermatol 1994; 11:21.
  68. Hollmig ST, Tollefson MM, Kim J, Khuu P. Multiple eruptive pilomatricomas in a 9-year-old boy with glioblastoma. Pediatr Dermatol 2013; 30:756.
  69. Park BS, Yang SG, Cho KH. Malignant proliferating trichilemmal tumor showing distant metastases. Am J Dermatopathol 1997; 19:536.
  70. Sellheyer K, Krahl D. Basal cell (trichoblastic) carcinoma common expression pattern for epithelial cell adhesion molecule links basal cell carcinoma to early follicular embryogenesis, secondary hair germ, and outer root sheath of the vellus hair follicle: A clue to the adnexal nature of basal cell carcinoma? J Am Acad Dermatol 2008; 58:158.
  71. Hamman MS, Brian Jiang SI. Management of trichilemmal carcinoma: an update and comprehensive review of the literature. Dermatol Surg 2014; 40:711.
  72. Dalton SR, LeBoit PE. Squamous cell carcinoma with clear cells: how often is there evidence of tricholemmal differentiation? Am J Dermatopathol 2008; 30:333.
  73. Misago N, Toda S, Narisawa Y. Tricholemmoma and clear cell squamous cell carcinoma (associated with Bowen's disease): immunohistochemical profile in comparison to normal hair follicles. Am J Dermatopathol 2012; 34:394.
  74. Tolkachjov SN, Hocker TL, Camilleri MJ, Baum CL. Mohs micrographic surgery in the treatment of trichilemmal carcinoma: the Mayo Clinic experience. J Am Acad Dermatol 2015; 72:195.
  75. Herrmann JL, Allan A, Trapp KM, Morgan MB. Pilomatrix carcinoma: 13 new cases and review of the literature with emphasis on predictors of metastasis. J Am Acad Dermatol 2014; 71:38.
  76. Melancon JM, Tom WL, Lee RA, et al. Management of pilomatrix carcinoma: a case report of successful treatment with Mohs micrographic surgery and review of the literature. Dermatol Surg 2011; 37:1798.
  77. Tselis N, Heyd R, Vogt HG, Zamboglou N. Pilomatrix carcinoma with lymph node and pulmonary metastases. Strahlenther Onkol 2006; 182:727.
  78. Arslan D, Gündüz S, Avci F, et al. Pilomatrix carcinoma of the scalp with pulmonary metastasis: A case report of a complete response to oral endoxan and etoposide. Oncol Lett 2014; 7:1959.
  79. Takeda H, Lyle S, Lazar AJ, et al. Human sebaceous tumors harbor inactivating mutations in LEF1. Nat Med 2006; 12:395.
  80. Boennelycke M, Thomsen BM, Holck S. Sebaceous neoplasms and the immunoprofile of mismatch-repair proteins as a screening target for syndromic cases. Pathol Res Pract 2015; 211:78.
  81. Everett JN, Raymond VM, Dandapani M, et al. Screening for germline mismatch repair mutations following diagnosis of sebaceous neoplasm. JAMA Dermatol 2014; 150:1315.
  82. Sato T, Tanaka M. Linear sebaceous hyperplasia on the chest. Dermatol Pract Concept 2014; 4:93.
  83. Vergara G, Belinchón I, Silvestre JF, et al. Linear sebaceous gland hyperplasia of the penis: a case report. J Am Acad Dermatol 2003; 48:149.
  84. Franco G, Donati P, Muscardin L, et al. Juxta-clavicular beaded lines. Australas J Dermatol 2006; 47:204.
  85. Woldow AB, Houk LD, Samie FH. Juxtaclavicular beaded lines: a presentation of sebaceous gland hyperplasia. Dermatol Online J 2009; 15:14.
  86. de Berker DA, Taylor AE, Quinn AG, Simpson NB. Sebaceous hyperplasia in organ transplant recipients: shared aspects of hyperplastic and dysplastic processes? J Am Acad Dermatol 1996; 35:696.
  87. Salim A, Reece SM, Smith AG, et al. Sebaceous hyperplasia and skin cancer in patients undergoing renal transplant. J Am Acad Dermatol 2006; 55:878.
  88. Wang Q, Liu JM, Zhang YZ. Premature sebaceous hyperplasia in an adolescent boy. Pediatr Dermatol 2011; 28:198.
  89. Turan H, Erdem H, Turan A, et al. Premature sebaceous hyperplasia with pre-pubertal onset. Int J Dermatol 2014; 53:e224.
  90. Sener S, Sasmaz S, Dogan DG, Ciralik H. Is "premature sebaceous hyperplasia" really a sebaceous hamartoma? Report of a case with neonatal onset. Pediatr Dermatol 2011; 28:732.
  91. McDonald SK, Goh MS, Chong AH. Successful treatment of cyclosporine-induced sebaceous hyperplasia with oral isotretinoin in two renal transplant recipients. Australas J Dermatol 2011; 52:227.
  92. Noh S, Shin JU, Jung JY, Lee JH. A case of sebaceous hyperplasia maintained on low-dose isotretinoin after carbon dioxide laser treatment. Int J Dermatol 2014; 53:e151.
  93. Richey DF. Aminolevulinic acid photodynamic therapy for sebaceous gland hyperplasia. Dermatol Clin 2007; 25:59.
  94. Wheeland RG, Wiley MD. Q-tip cryosurgery for the treatment of senile sebaceous hyperplasia. J Dermatol Surg Oncol 1987; 13:729.
  95. Bader RS, Scarborough DA. Surgical pearl: intralesional electrodesiccation of sebaceous hyperplasia. J Am Acad Dermatol 2000; 42:127.
  96. No D, McClaren M, Chotzen V, Kilmer SL. Sebaceous hyperplasia treated with a 1450-nm diode laser. Dermatol Surg 2004; 30:382.
  97. Troy JL, Ackerman AB. Sebaceoma. A distinctive benign neoplasm of adnexal epithelium differentiating toward sebaceous cells. Am J Dermatopathol 1984; 6:7.
  98. Bhaijee F, Brown AS. Muir-Torre syndrome. Arch Pathol Lab Med 2014; 138:1685.
  99. Singh RS, Grayson W, Redston M, et al. Site and tumor type predicts DNA mismatch repair status in cutaneous sebaceous neoplasia. Am J Surg Pathol 2008; 32:936.
  100. Dasgupta T, Wilson LD, Yu JB. A retrospective review of 1349 cases of sebaceous carcinoma. Cancer 2009; 115:158.
  101. Menko FH, van Steensel MA, Giraud S, et al. Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol 2009; 10:1199.
  102. Farasat S, Yu SS, Neel VA, et al. A new American Joint Committee on Cancer staging system for cutaneous squamous cell carcinoma: creation and rationale for inclusion of tumor (T) characteristics. J Am Acad Dermatol 2011; 64:1051.
  103. Ad Hoc Task Force, Connolly SM, Baker DR, et al. AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery. J Am Acad Dermatol 2012; 67:531.
  104. Conill C, Toscas I, Morilla I, Mascaró JM Jr. Radiation therapy as a curative treatment in extraocular sebaceous carcinoma. Br J Dermatol 2003; 149:441.
  105. Pardo FS, Wang CC, Albert D, Stracher MA. Sebaceous carcinoma of the ocular adnexa: radiotherapeutic management. Int J Radiat Oncol Biol Phys 1989; 17:643.
  106. Avraham JB, Villines D, Maker VK, et al. Survival after resection of cutaneous adnexal carcinomas with eccrine differentiation: risk factors and trends in outcomes. J Surg Oncol 2013; 108:57.
  107. Barnes M, Hestley A, Murray DR, et al. The risk of lymph node involvement in malignant cutaneous adnexal tumors. Am Surg 2014; 80:270.
  108. De Iuliis F, Amoroso L, Taglieri L, et al. Chemotherapy of rare skin adnexal tumors: a review of literature. Anticancer Res 2014; 34:5263.
  109. Snow SN, Larson PO, Lucarelli MJ, et al. Sebaceous carcinoma of the eyelids treated by mohs micrographic surgery: report of nine cases with review of the literature. Dermatol Surg 2002; 28:623.
  110. Blake PW, Toro JR. Update of cylindromatosis gene (CYLD) mutations in Brooke-Spiegler syndrome: novel insights into the role of deubiquitination in cell signaling. Hum Mutat 2009; 30:1025.
  111. Bowen S, Gill M, Lee DA, et al. Mutations in the CYLD gene in Brooke-Spiegler syndrome, familial cylindromatosis, and multiple familial trichoepithelioma: lack of genotype-phenotype correlation. J Invest Dermatol 2005; 124:919.
  112. Young AL, Kellermayer R, Szigeti R, et al. CYLD mutations underlie Brooke-Spiegler, familial cylindromatosis, and multiple familial trichoepithelioma syndromes. Clin Genet 2006; 70:246.
  113. Nelen MR, van Staveren WC, Peeters EA, et al. Germline mutations in the PTEN/MMAC1 gene in patients with Cowden disease. Hum Mol Genet 1997; 6:1383.
  114. Ponti G, Ponz de Leon M. Muir-Torre syndrome. Lancet Oncol 2005; 6:980.
Topic Outline