Approach Considerations

Treatment of spiradenomas is surgical.

Malignant spiradenomas may be treated with surgery, radiation, hyperthermic limb perfusion chemotherapy, and chemotherapy. They may also be sensitive to tamoxifen therapy.^{[47, 48]}

Surgical Care

The mainstay of treatment of spiradenomas and malignant spiradenomas is surgical removal. The lesions do not tend to recur after surgery. Mohs micrographic surgery has been used on the anterior neck to surgically remove infiltrative persistent spiradenoma.^[49]

Multiple spiradenomas, such as those found in Brooke-Spiegler syndrome, have been treated with a high-energy continuous wave carbon dioxide laser after debulking with bipolar scissors in one patient.^{[50, 51]}

Consultations

Patients with malignant spiradenoma should see a surgeon, an oncologist, and possibly a radiation oncologist.

Activity

Sometimes, pain can bother patients, but this pain should not affect their activities.

Prevention

Complete excision of lesions should be performed to prevent recurrence.

Long-Term Monitoring

Surgery is usually curative; therefore, further care is not needed. Patients with malignant spiradenomas need to follow up with oncologists and radiation oncologists.

Medication

Sellheyer K. Spiradenoma and cylindroma originate from the hair follicle bulge and not from the eccrine sweat gland: an immunohistochemical study with CD200 and other stem cell markers. J Cutan Pathol. 2014 Oct 29. [QxMD MEDLINE Link].
Dabska M. Malignant transformation of eccrine spiradenoma. Pol Med J. 1972. 11(2):388-96. [QxMD MEDLINE Link].
Bumgardner AC, Hsu S, Nunez-Gussman JK, Schwartz MR. Trichoepitheliomas and eccrine spiradenomas with spiradenoma/cylindroma overlap. Int J Dermatol. 2005 May. 44(5):415-7. [QxMD MEDLINE Link].
Clarke J, Ioffreda M, Helm KF. Multiple familial trichoepitheliomas: a folliculosebaceous-apocrine genodermatosis. Am J Dermatopathol. 2002 Oct. 24(5):402-5. [QxMD MEDLINE Link].
Sun SC. CYLD: a tumor suppressor deubiquitinase regulating NF-kappaB activation and diverse biological processes. Cell Death Differ. 2010 Jan. 17 (1):25-34. [QxMD MEDLINE Link].
Grossmann P, Vanecek T, Steiner P, et al. Novel and Recurrent Germline and Somatic Mutations in a Cohort of 67 Patients From 48 Families With Brooke-Spiegler Syndrome Including the Phenotypic Variant of Multiple Familial Trichoepitheliomas and Correlation With the Histopathologic Findings in 379 Biopsy Specimens. Am J Dermatopathol. 2013 Feb. 35(1):34-44. [QxMD MEDLINE Link].
Im M, Kim DH, Park JS, Chung H, Lee Y, Kim CD, et al. Alteration of the β-catenin pathway in spiradenoma. J Cutan Pathol. 2011 Aug. 38 (8):657-62. [QxMD MEDLINE Link].
Rashid M, van der Horst M, Mentzel T, et al. ALPK1 hotspot mutation as a driver of human spiradenoma and spiradenocarcinoma. Nat Commun. 2019 May 17. 10 (1):2213. [QxMD MEDLINE Link].
Englander L, Emer JJ, McClain D, Amin B, Turner RB. A rare case of multiple segmental eccrine spiradenomas. J Clin Aesthet Dermatol. 2011 Apr. 4(4):38-44. [QxMD MEDLINE Link]. [Full Text].
Kazakov DV, Schaller J, Vanecek T, Kacerovska D, Michal M. Brooke-Spiegler syndrome: report of a case with a novel mutation in the CYLD gene and different types of somatic mutations in benign and malignant tumors. J Cutan Pathol. 2010 Aug. 37(8):886-90. [QxMD MEDLINE Link].
Bowen S, Gill M, Lee DA, Fisher G, Geronemus RG, Vazquez ME, 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 May. 124 (5):919-20. [QxMD MEDLINE Link].
Staiger RD, Helmchen B, Papet C, Mattiello D, Zingg U. Spiradenocarcinoma: A Comprehensive Data Review. Am J Dermatopathol. 2017 Oct. 39 (10):715-725. [QxMD MEDLINE Link].
Tiradogonzalez M, Beierle E, Hammers Y, Andea A, Mroczek E. Neonatal Spiradenoma. Pediatr Dermatol. 2012 Jul 2. [QxMD MEDLINE Link].
Miedema JR, Burgon E, Burkhart C, Stitzenberg K, Hipps J, Zedek D. Metastatic spiradenocarcinoma occurring in an 8-year-old boy. Pediatr Dermatol. 2015 Jan-Feb. 32 (1):122-7. [QxMD MEDLINE Link].
Kersting DW, Helwig EB. Eccrine spiradenoma. AMA Arch Derm. 1956 Mar. 73(3):199-227. [QxMD MEDLINE Link].
Mambo NC. Eccrine spiradenoma: clinical and pathologic study of 49 tumors. J Cutan Pathol. 1983 Oct. 10(5):312-20. [QxMD MEDLINE Link].
Yildirim S, Akoz T, Akan M, Ege GA. De novo malignant eccrine spiradenoma with an interesting and unusual location. Dermatol Surg. 2001 Apr. 27(4):417-20. [QxMD MEDLINE Link].
Amoroso C, Grandi E, Carinci F. Eccrine spiradenoma of the ear: case report. Int J Oral Maxillofac Surg. 2003 Dec. 32(6):662-3. [QxMD MEDLINE Link].
Nadig SK, Alderdice JM, Adair RA, Rao TJ. Eccrine spiradenoma: an unusual presentation with otalgia. Otolaryngol Head Neck Surg. 2004 Feb. 130(2):277-8. [QxMD MEDLINE Link].
Meyer TK, Rhee JS, Smith MM, Cruz MJ, Osipov VO, Wackym PA. External auditory canal eccrine spiradenocarcinoma: a case report and review of literature. Head Neck. 2003 Jun. 25(6):505-10. [QxMD MEDLINE Link].
Amann J, Spraul CW, Mattfeld T, Lang GK. [Eccrine spiradenoma of the eyelid]. Klin Monbl Augenheilkd. 1999 Jan. 214(1):53-4. [QxMD MEDLINE Link].
Gupta S, Radotra BD, Kaur I, Handa S, Kumar B. Multiple linear eccrine spiradenomas with eyelid involvement. J Eur Acad Dermatol Venereol. 2001 Mar. 15(2):163-6. [QxMD MEDLINE Link].
Leach BC, Graham BS. Papular lesion of the proximal nail fold. Eccrine spiradenoma. Arch Dermatol. 2004 Aug. 140(8):1003-8. [QxMD MEDLINE Link].
Chen G, Cheuk W, Cheung JS, Chan JK. Carcinosarcoma ex eccrine spiradenoma of the vulva: report of the first case. Int J Gynecol Pathol. 2011 May. 30(3):301-5. [QxMD MEDLINE Link].
Siegel HJ, Said-Al-Naief N, Long J, Lopez-Ben RR, Klein M. Giant eccrine spiradenoma of the hand. Am J Orthop (Belle Mead NJ). 2008 Aug. 37(8):E141-3. [QxMD MEDLINE Link].
Chow W, Griffiths M. A malignant eccrine spiradenoma of the scalp. BMJ Case Rep. 2014 May 19. 2014.:pii. [QxMD MEDLINE Link].
Beekley AC, Brown TA, Porter C. Malignant eccrine spiradenoma: a previously unreported presentation and review of the literature. Am Surg. 1999 Mar. 65(3):236-40. [QxMD MEDLINE Link].
Jamshidi M, Nowak MA, Chiu YT, Perry EA, Fatteh SM. Giant malignant eccrine spiradenoma of the scalp. Dermatol Surg. 1999 Jan. 25(1):45-8. [QxMD MEDLINE Link].
Emam EE, Sawan AS, Al-Tamimi SR, Molah RM. Malignant spiradenoma/cylindroma of the vulva. Saudi Med J. 2012 Nov. 33(11):1229-33. [QxMD MEDLINE Link].
Chase DM, Basu T, Saffari B, Ries S, Berman ML. Malignant eccrine spiradenoma of the vulva: a case report and review of the literature. Int J Gynecol Cancer. 2006 May-Jun. 16(3):1465-9. [QxMD MEDLINE Link].
Jariwala A, Evans A, McLeod G. Not all stubbed toes are innocuous--A case report of rare malignant eccrine spiradenoma (spiradenocarcinoma) of the toe. Foot Ankle Surg. 2010 Jun. 16(2):e32-3. [QxMD MEDLINE Link].
Arslan E, Unal S, Cinel L, Demirkan F, Cin I. Malignant eccrine spiradenoma occurring on a traumatized area. Plast Reconstr Surg. 2002 Jul. 110(1):365-7. [QxMD MEDLINE Link].
Jin W, Kim GY, Lew BL, Yang DM, Kim HC, Ryu JK, et al. Sonographic findings of an eccrine spiradenoma: case report and literature review. J Ultrasound Med. 2008 May. 27 (5):813-8. [QxMD MEDLINE Link].
Hwang CM, Kang BS, Hong HJ, Lee JY, Suh JH, Han EM, et al. Ultrasonographic Features of Eccrine Spiradenoma. J Ultrasound Med. 2018 May. 37 (5):1267-1272. [QxMD MEDLINE Link].
Han YD, Huan Y, Deng JL, Zhang YG, Zhang CH. MRI appearance of multiple eccrine spiradenoma. Br J Radiol. 2007 Jan. 80(949):e27-9. [QxMD MEDLINE Link].
Bosch MM, Boon ME. Fine-needle cytology of an eccrine spiradenoma of the breast: diagnosis made by a holistic approach. Diagn Cytopathol. 1992. 8(4):366-8. [QxMD MEDLINE Link].
Patterson JW. Tumors. Weedon's Skin Pathology E-Book. 4th ed. Churchill Livingstone; 2014. 938.
Meybehm M, Fischer HP. Spiradenoma and dermal cylindroma: comparative immunohistochemical analysis and histogenetic considerations. Am J Dermatopathol. 1997 Apr. 19(2):154-61. [QxMD MEDLINE Link].
Kao GF, Laskin WB, Weiss SW. Eccrine spiradenoma occurring in infancy mimicking mesenchymal tumor. J Cutan Pathol. 1990 Aug. 17(4):214-9. [QxMD MEDLINE Link].
Park HR, Im SB, Kim HK, Shin DS, Park YL. Painful eccrine spiradenoma containing nerve fibers: a case report. Dermatology. 2012. 224(4):301-6. [QxMD MEDLINE Link].
Granter SR, Seeger K, Calonje E, Busam K, McKee PH. Malignant eccrine spiradenoma (spiradenocarcinoma): a clinicopathologic study of 12 cases. Am J Dermatopathol. 2000 Apr. 22(2):97-103. [QxMD MEDLINE Link].
Saboorian MH, Kenny M, Ashfaq R, Albores-Saavedra J. Carcinosarcoma arising in eccrine spiradenoma of the breast. Report of a case and review of the literature. Arch Pathol Lab Med. 1996 May. 120(5):501-4. [QxMD MEDLINE Link].
Jitsukawa K, Sueki H, Sato S, Anzai T. Eccrine spiradenoma. An electron microscopic study. Am J Dermatopathol. 1987 Apr. 9(2):99-108. [QxMD MEDLINE Link].
Ishihara M, Mehregan DR, Hashimoto K, et al. Staining of eccrine and apocrine neoplasms and metastatic adenocarcinoma with IKH-4, a monoclonal antibody specific for the eccrine gland. J Cutan Pathol. 1998 Feb. 25(2):100-5. [QxMD MEDLINE Link].
Yasui S, Ando I, Kukita A, Hino H, Ohara K. DF3 (CA15-3) antibody as a marker of cutaneous adnexal tumors. Acta Derm Venereol. 1994 Mar. 74(2):98-100. [QxMD MEDLINE Link].
Kurokawa I, Nishimura K, Tarumi C, et al. Eccrine spiradenoma: co-expression of cytokeratin and smooth muscle actin suggesting differentiation toward myoepithelial cells. J Eur Acad Dermatol Venereol. 2007 Jan. 21(1):121-3. [QxMD MEDLINE Link].
Sridhar KS, Benedetto P, Otrakji CL, Charyulu KK. Response of eccrine adenocarcinoma to tamoxifen. Cancer. 1989 Jul 15. 64(2):366-70. [QxMD MEDLINE Link].
Mirza I, Kloss R, Sieber SC. Malignant eccrine spiradenoma. Arch Pathol Lab Med. 2002 May. 126(5):591-4. [QxMD MEDLINE Link].
Rahim RR, Rajan N, Langtry JA. Infiltrative Recurrent Eccrine Spiradenoma of the Anterior Neck Treated Using Mohs Micrographic Surgery. Dermatol Surg. 2013 Oct 1. [QxMD MEDLINE Link].
Martins C, Bartolo E. Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser. Dermatol Surg. 2000 Sep. 26(9):877-80; discussion 881. [QxMD MEDLINE Link].
Richard A, Chevalier JM, Verneuil L, Sergent B, Tesnière A, Dolfus C, et al. CO2 laser treatment of skin cylindromas in Brooke-Spiegler syndrome. Ann Dermatol Venereol. 2014 May. 141(5):346-53. [QxMD MEDLINE Link].

Media Gallery

Spiradenoma. Vascular channels within a basaloid tumor. Courtesy of Sarahkayb (own work), via Wikimedia Commons.

of 1

Author

Nikki A Levin, MD, PhD Associate Professor of Medicine, Division of Dermatology, University of Massachusetts Medical School

Nikki A Levin, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Holly D Neale, BS MD Candidate, University of Massachusetts Medical School

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Noah S Scheinfeld, JD, MD, FAAD † Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.