Spiradenoma 

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 2, 2011
 

Background

The histogenesis of spiradenomas remains in question, but many lesions demonstrate apocrine differentiation. The term eccrine spiradenoma may join the list of other misnomers in dermatology, including mycosis fungoides and granuloma faciale. Lesions usually manifest as solitary, 1-cm-diameter, gray, pink, purple, red, or blue nodules on the upper half of the ventral side of the body. Spiradenomas can be painful, often in paroxysms. Multiple spiradenomas have been reported. Their initial elaboration is attributed to Kersting and Helwig.[1]

Spiradenomas are usually benign. It can occur in infancy but most commonly arises in persons aged 15-35 years. About 15 cases of linear/zosteriform/nevoid/blaschkoid multiple spiradenomas exist in the literature.[2, 3] About 50 case reports of malignant spiradenoma exist in the literature. Dabska[4] first described malignant spiradenoma in 1972.

Spiradenomas can occur in Brooke-Spiegler syndrome, which manifests with cylindromas, spiradenomas, and trichoepitheliomas. In this syndrome, lesions can have combined features of both cylindromas and spiradenomas.[5, 6]

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Pathophysiology

A defective tumor suppressor gene is believed to result in the development of spiradenomas. In Brooke-Spiegler syndrome, of which spiradenomas are a manifestation, the defective gene is the CYLD gene on chromosome 9. Work remains to be performed on the genetic defect causing isolated and sporadic spiradenomas. The CYLD seems to be a hot spot of mutations as novel mutations continue to be reported.[7]

The expression of p53 in malignant spiradenomas seems to be increased.

The cells of origin of spiradenomas appear to have apocrine and trichoepitheliomatous differentiation, ie, they have complex hair follicle (folliculosebaceous apocrine) differentiation rather than eccrine differentiation.

In cases of linear/zosteriform/nevoid/blaschkoid multiple spiradenomas, an abnormal clone arising during embryogenesis is postulated to produce the multiple abnormal cells that result in such spiradenomas.

In a cytogenetic study, Dijkhuizen et al[8] found a spiradenoma and 2 lymph node metastases, with a growth pattern and microscopic appearance typical for benign spiradenoma, a 46,XY-5,del(16)(q22),+mar(t(?;5)(?::5q13----5qter)) karyotype.

These similar genetic defects seem to support a relationship between the chromosomal abnormalities and the clinical malignant action of this benign-appearing neoplasm.

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Epidemiology

Frequency

Spiradenomas are rare worldwide. Malignant spiradenomas are very rare worldwide.

Mortality/Morbidity

Spiradenomas can be painful. The rate of malignant transformation is very low, and, sometimes, malignant transformation has been reported to develop spontaneously. The rate of metastasis is about 50% and can result in death.

Race

No racial link exists for spiradenomas.

Sex

No sexual predilection exists for spiradenomas or malignant spiradenomas.

Age

Spiradenomas have been reported to arise in infancy but this is rare. However, most spiradenomas arise in persons aged 15-35 years. Malignant spiradenomas tend to develop after age 50 years. Malignant spiradenomas presents at an average age of 59 years (range, 21-92 y).

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

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

Disclosure: Optigenex Consulting fee Independent contractor

Specialty Editor Board

Evan R Farmer, MD  Clinical Professor of Pathology and Dermatology, Department of Pathology, Virginia Commonwealth University School of Medicine

Evan R Farmer, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, and International Society of Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside 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: 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, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

References

  1. Kerstein DW, Helwig EB. Eccrine spiradenoma. AMA Arch Derm. Mar 1956;73(3):199-227. [Medline].

  2. Ekmekci TR, Koslu A, Sakiz D. Congenital blaschkoid eccrine spiradenoma on the face. Eur J Dermatol. Mar-Apr 2005;15(2):73-4. [Medline].

  3. Scheinfeld NS, Tarlow MM, Burgin S. Blaschkoid eccrine spiradenomas. Cutis. Jul 2002;70(1):73-5. [Medline].

  4. Dabska M. Malignant transformation of eccrine spiradenoma. Pol Med J. 1972;11(2):388-96. [Medline].

  5. Bumgardner AC, Hsu S, Nunez-Gussman JK, Schwartz MR. Trichoepitheliomas and eccrine spiradenomas with spiradenoma/cylindroma overlap. Int J Dermatol. May 2005;44(5):415-7. [Medline].

  6. Clarke J, Ioffreda M, Helm KF. Multiple familial trichoepitheliomas: a folliculosebaceous-apocrine genodermatosis. Am J Dermatopathol. Oct 2002;24(5):402-5. [Medline].

  7. 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. Aug 2010;37(8):886-90. [Medline].

  8. Dijkhuizen T, van den Berg E, Nikkels PG, Hoekstra HJ, de Jong B. Cytogenetics of a case of eccrine spiradenoma. Hum Pathol. Sep 1992;23(9):1085-7. [Medline].

  9. Mambo NC. Eccrine spiradenoma: clinical and pathologic study of 49 tumors. J Cutan Pathol. Oct 1983;10(5):312-20. [Medline].

  10. Yildirim S, Akoz T, Akan M, Ege GA. De novo malignant eccrine spiradenoma with an interesting and unusual location. Dermatol Surg. Apr 2001;27(4):417-20. [Medline].

  11. Granter SR, Seeger K, Calonje E, Busam K, McKee PH. Malignant eccrine spiradenoma (spiradenocarcinoma): a clinicopathologic study of 12 cases. Am J Dermatopathol. Apr 2000;22(2):97-103. [Medline].

  12. Chou SC, Lin SL, Tseng HH. Malignant eccrine spiradenoma: a case report with pulmonary metastasis. Pathol Int. Mar 2004;54(3):208-12. [Medline].

  13. Kao GF, Laskin WB, Weiss SW. Eccrine spiradenoma occurring in infancy mimicking mesenchymal tumor. J Cutan Pathol. Aug 1990;17(4):214-9. [Medline].

  14. Kazakov DV, Soukup R, Mukensnabl P, Boudova L, Michal M. Brooke-Spiegler syndrome: report of a case with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation. Am J Dermatopathol. Feb 2005;27(1):27-33. [Medline].

  15. Amoroso C, Grandi E, Carinci F. Eccrine spiradenoma of the ear: case report. Int J Oral Maxillofac Surg. Dec 2003;32(6):662-3. [Medline].

  16. Nadig SK, Alderdice JM, Adair RA, Rao TJ. Eccrine spiradenoma: an unusual presentation with otalgia. Otolaryngol Head Neck Surg. Feb 2004;130(2):277-8. [Medline].

  17. 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. Jun 2003;25(6):505-10. [Medline].

  18. Leach BC, Graham BS. Papular lesion of the proximal nail fold. Eccrine spiradenoma. Arch Dermatol. Aug 2004;140(8):1003-8. [Medline].

  19. Chen G, Cheuk W, Cheung JS, Chan JK. Carcinosarcoma ex eccrine spiradenoma of the vulva: report of the first case. Int J Gynecol Pathol. May 2011;30(3):301-5. [Medline].

  20. Amann J, Spraul CW, Mattfeld T, Lang GK. [Eccrine spiradenoma of the eyelid]. Klin Monbl Augenheilkd. Jan 1999;214(1):53-4. [Medline].

  21. Gupta S, Radotra BD, Kaur I, Handa S, Kumar B. Multiple linear eccrine spiradenomas with eyelid involvement. J Eur Acad Dermatol Venereol. Mar 2001;15(2):163-6. [Medline].

  22. Beekley AC, Brown TA, Porter C. Malignant eccrine spiradenoma: a previously unreported presentation and review of the literature. Am Surg. Mar 1999;65(3):236-40. [Medline].

  23. Jamshidi M, Nowak MA, Chiu YT, Perry EA, Fatteh SM. Giant malignant eccrine spiradenoma of the scalp. Dermatol Surg. Jan 1999;25(1):45-8. [Medline].

  24. Arslan E, Unal S, Cinel L, Demirkan F, Cin I. Malignant eccrine spiradenoma occurring on a traumatized area. Plast Reconstr Surg. Jul 2002;110(1):365-7. [Medline].

  25. 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. May-Jun 2006;16(3):1465-9. [Medline].

  26. 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. Jun 2010;16(2):e32-3. [Medline].

  27. 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). Aug 2008;37(8):E141-3. [Medline].

  28. Turhan-Haktanir N, Demir Y, Tokyol C. A case of eccrine spiradenoma arising in nevus sebaceous in an adolescent girl. Am J Dermatopathol. Apr 2008;30(2):196-7. [Medline].

  29. Rodriguez-Martin M, Sanchez Gonzalez R, Saez-Rodríguez M, Garcia-Bustínduy M, Martín-Herrera A, Noda-Cabrera A. An unusual case of congenital linear eccrine spiradenoma. Pediatr Dermatol. Mar-Apr 2009;26(2):180-3. [Medline].

  30. Englander L, Emer JJ, McClain D, Amin B, Turner RB. A rare case of multiple segmental eccrine spiradenomas. J Clin Aesthet Dermatol. Apr 2011;4(4):38-44. [Medline]. [Full Text].

  31. Han YD, Huan Y, Deng JL, Zhang YG, Zhang CH. MRI appearance of multiple eccrine spiradenoma. Br J Radiol. Jan 2007;80(949):e27-9. [Medline].

  32. 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. [Medline].

  33. Jitsukawa K, Sueki H, Sato S, Anzai T. Eccrine spiradenoma. An electron microscopic study. Am J Dermatopathol. Apr 1987;9(2):99-108. [Medline].

  34. Meybehm M, Fischer HP. Spiradenoma and dermal cylindroma: comparative immunohistochemical analysis and histogenetic considerations. Am J Dermatopathol. Apr 1997;19(2):154-61. [Medline].

  35. 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. Feb 1998;25(2):100-5. [Medline].

  36. Yasui S, Ando I, Kukita A, Hino H, Ohara K. DF3 (CA15-3) antibody as a marker of cutaneous adnexal tumors. Acta Derm Venereol. Mar 1994;74(2):98-100. [Medline].

  37. 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. May 1996;120(5):501-4. [Medline].

  38. 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. Jan 2007;21(1):121-3. [Medline].

  39. Ko JY, Lee CW, Moon SH, Song KW, Park CK. Giant vascular eccrine spiradenoma: report of a case with immunohistochemical study. J Korean Med Sci. Feb 2006;21(1):172-6. [Medline].

  40. Ohtsuki Y, Ohtsuka H, Kurabayashi A, et al. Immunohistochemical and electron microscopic studies of Langerhans cells in a case of multiple eccrine spiradenomas. Med Mol Morphol. Dec 2007;40(4):221-5. [Medline].

  41. Sridhar KS, Benedetto P, Otrakji CL, Charyulu KK. Response of eccrine adenocarcinoma to tamoxifen. Cancer. Jul 15 1989;64(2):366-70. [Medline].

  42. Mirza I, Kloss R, Sieber SC. Malignant eccrine spiradenoma. Arch Pathol Lab Med. May 2002;126(5):591-4. [Medline].

  43. Martins C, Bartolo E. Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser. Dermatol Surg. Sep 2000;26(9):877-80; discussion 881. [Medline].

  44. Hantash BM, Chan JL, Egbert BM, Gladstone HB. De novo malignant eccrine spiradenoma: a case report and review of the literature. Dermatol Surg. Sep 2006;32(9):1189-98. [Medline].

  45. Lara JF, Nadeem MA, Asaadi M. Malignant mixed tumor ex eccrine spiradenoma: an unusual pattern of malignant dedifferentiation. Ann Diagn Pathol. Jun 2001;5(3):155-61. [Medline].

  46. Liapakis IE, Korkolis DP, Koutsoumbi A, Fida A, Kokkalis G, Vassilopoulos PP. Malignant hidradenoma: a report of two cases and review of the literature. Anticancer Res. May-Jun 2006;26(3B):2217-20. [Medline].

  47. Michal M, Lamovec J, Mukensnabl P, Pizinger K. Spiradenocylindromas of the skin: tumors with morphological features of spiradenoma and cylindroma in the same lesion: report of 12 cases. Pathol Int. May 1999;49(5):419-25. [Medline].

  48. Rajan N, Burn J, Langtry J, Sieber-Blum M, Lord CJ, Ashworth A. Transition from cylindroma to spiradenoma in CYLD-defective tumours is associatedwith reduced DKK2 expression. J Pathol. Jul 2011;3:309-21. [Medline].

 
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