eMedicine Specialties > Dermatology > Malignant Neoplasms

Eccrine Carcinoma

Author: Anthony Wong, MD, FAAD, Consulting Staff, Department of Dermatology, SUNY Health Science Center at Brooklyn, St Catherine's of Sienna, and Long Island Skin Cancer and Dermatologic Surgery, PC
Coauthor(s): Darren Keith Mollick, MD, Clinical Assistant Professor, Department of Dermatology, State University of New York Downstate Medical Center; Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Contributor Information and Disclosures

Updated: Mar 26, 2010

Introduction

Background

Carcinomas of the eccrine sweat gland represent a rare group of tumors with potential for local destruction and metastasis. High recurrence rates have been reported following conventional surgical excision of eccrine carcinomas. The specific classification of eccrine carcinomas is both complex and nebulous, in large part because of the paucity of reported cases but also because many of these tumors show little histologic resemblance to mature eccrine glands; the histogenetic association is based primarily on histochemical, immunochemical, or ultrastructural features. Nevertheless, eccrine carcinomas may be taxonomically segregated into 2 main groups, as follows: those that are histologically similar to certain benign appendage tumors (eg, sclerosing sweat duct carcinoma, porocarcinoma, malignant chondroid syringoma, malignant nodular hidradenoma, malignant eccrine spiradenoma) and those that show a diverse array of histologic features, not recapitulating to any degree aspects of a benign counterpart.

A slightly different method of eccrine carcinoma classification is suggested by Galadari et al,1 who divide these tumors into those that arise de novo in normal skin and those that originate within preexisting benign sweat gland tumors. Precise identification based on histology is of significant importance because therapy and prognosis vary according to microscopic appearance (see Histologic Findings).

Pathophysiology

Eccrine carcinoma may be derived de novo from any portion of the normal eccrine apparatus or result from the transformation of an existing benign eccrine tumor.

A 2000 study by Takata et al2 examining the incidence of cytogenetic abnormalities in malignant eccrine tumors showed low incidences of loss of heterozygosity (LOH) or TP53 alterations in a mixed group of these neoplasms, in contrast to the frequent and multiple genetic abnormalities seen in tumors arising from epidermal keratinocytes. The authors speculate that this difference may be partly explained by the fact that the bulk of a sweat gland lies deep in the dermis where it is relatively protected from the sun and environmental mutagens. The precise role of ultraviolet radiation (UVR) remains to be elucidated, as another study analyzing TP53 mutations in 16 sweat gland carcinomas identified 3 G:C ® A:T transitions at dipyrimidine sequences on the antisense strand.3

Abbate et al4 suggest that genetics may play a role in the development of microcystic adnexal carcinoma (MAC).

Frequency

United States

Primary eccrine carcinomas are exceedingly rare, accounting for roughly 1 of 13,000 specimens submitted to a dermatopathology laboratory. The more common subtypes include microcystic adnexal carcinoma, eccrine porocarcinoma, and hidradenocarcinoma. The less common subtypes include eccrine mucinous carcinoma, malignant eccrine spiradenoma, malignant mixed tumor, malignant cylindroma, and papillary eccrine adenoma.

International

Only several hundred cases of eccrine carcinoma have been reported in the literature worldwide. No specific data are available regarding United States versus international incidence of eccrine carcinoma.

Mortality/Morbidity

Data regarding precise figures for eccrine carcinoma are insufficient; however, many of these tumors metastasize (up to 60%), with a fatal outcome.

Race

MAC was previously only described in white patients; however, Peterson et al5 and Gardner et al6 report the first and second cases of MAC affecting African Americans, respectively.

Sex

Sex incidence would appear to be equal for eccrine carcinoma, although this has not been definitively stated. Exceptions to this are the malignant chondroid syringoma and primary cutaneous adenoid cystic eccrine carcinoma, both of which occur more commonly in females than in males.

Age

Eccrine carcinomas most commonly are diagnosed in patients in their fifth through eighth decades of life.

Clinical

History

  • Malignant eccrine tumors generally present as a single, asymptomatic, nondescript cutaneous lesion.
  • Eccrine carcinomas grow either slowly over years or rapidly, reaching a size of several centimeters over a few months.
  • With disseminated disease, patients may report symptoms related to organ specific metastases.

Physical

  • Cutaneous/primary lesion of eccrine carcinoma 
    • A solitary nodule or plaque on the head or extremities and, less commonly, the trunk is present.
    • Occasionally, the lesion may ulcerate.
    • A few tumor subtypes do exhibit salient clinical features. 
      • MAC occurs as an indurated plaque especially in the nasolabial area.
      • Mucinous eccrine carcinoma predominantly involves the eyelids and has also been described on the scalp.7,8
      • Aggressive digital papillary adenoma/adenocarcinoma typically is seen in males on the digits and adjacent skin.
      • Most hidradenocarcinomas occur on the head and neck of older patients.
      • Most eccrine porocarcinomas are found on the lower extremities of older adults.
  • Disseminated eccrine carcinoma disease 
    • Nodal and distant metastases may be detected.
    • In particular, eccrine porocarcinoma (the most common eccrine cancer subtype) shows a characteristic propensity to produce multiple cutaneous metastatic deposits concomitant with visceral spread.

Causes

An etiologic role for UVR has been suggested for eccrine carcinoma. A case of MAC was reported in the neck of a man who received radiation therapy to the site years earlier. In addition, Abbate et al4 report that 5 out of 10 patients with MAC gave a prior history of radiation therapy. Chiller et al9 report a potential etiologic role for UVR as they describe MAC predominantly affecting the left side of the face, corresponding to higher UVR exposures while driving.

Immunosuppression has been known to increase the risk of nonmelanoma skin cancers, particularly squamous cell carcinomas. In a 2003 retrospective review of appendageal tumors in organ transplant recipients, Harwood et al10 suggest that patients who are immunosuppressed have a propensity to also develop cutaneous appendageal tumors over their immunocompetent counterparts, with increased rates of both benign eccrine tumors and malignant eccrine tumors.

More on Eccrine Carcinoma

Overview: Eccrine Carcinoma
Differential Diagnoses & Workup: Eccrine Carcinoma
Treatment & Medication: Eccrine Carcinoma
Follow-up: Eccrine Carcinoma
References
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References

  1. Galadari E, Mehregan AH, Lee KC. Malignant transformation of eccrine tumors. J Cutan Pathol. Feb 1987;14(1):15-22. [Medline].

  2. Takata M, Hashimoto K, Mehregan P, et al. Genetic changes in sweat gland carcinomas. J Cutan Pathol. Jan 2000;27(1):30-5. [Medline].

  3. Biernat W, Peraud A, Wozniak L, Ohgaki H. p53 mutations in sweat gland carcinomas. Int J Cancer. May 4 1998;76(3):317-20. [Medline].

  4. Abbate M, Zeitouni NC, Seyler M, Hicks W, Loree T, Cheney RT. Clinical course, risk factors, and treatment of microcystic adnexal carcinoma: a short series report. Dermatol Surg. Oct 2003;29(10):1035-8. [Medline].

  5. Peterson CM, Ratz JL, Sangueza OP. Microcystic adnexal carcinoma: First reported case in an African American man. J Am Acad Dermatol. Aug 2001;45(2):283-5. [Medline].

  6. Gardner ES, Goldberg LH. Neglected microcystic adnexal carcinoma: the second reported case in a black patient. Dermatol Surg. Jul 2001;27(7):678-80. [Medline].

  7. Chauhan A, Ganguly M, Takkar P, Dutta V. Primary mucinous carcinoma of eyelid: a rare clinical entity. Indian J Ophthalmol. Mar-Apr 2009;57(2):150-2. [Medline].

  8. Bannur HB, Mastiholimath RD, Malur PR. Primary mucinous eccrine adenocarcinoma of the scalp: a case report. Acta Cytol. Nov-Dec 2009;53(6):698-700. [Medline].

  9. Chiller K, Passaro D, Scheuller M, Singer M, McCalmont T, Grekin RC. Microcystic adnexal carcinoma: forty-eight cases, their treatment, and their outcome. Arch Dermatol. Nov 2000;136(11):1355-9. [Medline].

  10. Harwood CA, McGregor JM, Swale VJ, et al. High frequency and diversity of cutaneous appendageal tumors in organ transplant recipients. J Am Acad Dermatol. Mar 2003;48(3):401-8. [Medline].

  11. Scholz IM, Hartschuh W. Primary mucinous eccrine carcinoma of the skin - a rare clinical tumor with many differential diagnoses. J Dtsch Dermatol Ges. Oct 13 2009;[Medline].

  12. Wildemore JK, Lee JB, Humphreys TR. Mohs surgery for malignant eccrine neoplasms. Dermatol Surg. Dec 2004;30(12 Pt 2):1574-9. [Medline].

  13. Penneys NS, Zlatkiss I. Immunohistochemical demonstration of ferritin in sweat gland and sweat gland neoplasms. J Cutan Pathol. Feb 1990;17(1):32-6. [Medline].

  14. Yeung KY, Stinson JC. Mucinous (adenocystic) carcinoma of sweat glands with widespread metastasis. Case report with ultrastructural study. Cancer. Jun 1977;39(6):2556-62. [Medline].

  15. Snow S, Madjar DD, Hardy S, et al. Microcystic adnexal carcinoma: report of 13 cases and review of the literature. Dermatol Surg. Apr 2001;27(4):401-8. [Medline].

  16. Lozano Orella JA, Valcayo Penalba A, San Juan CC, et al. Eccrine porocarcinoma. Report of nine cases. Dermatol Surg. Oct 1997;23(10):925-8. [Medline].

  17. Mehregan AH, Hashimoto K, Rahbari H. Eccrine adenocarcinoma. A clinicopathologic study of 35 cases. Arch Dermatol. Feb 1983;119(2):104-14. [Medline].

  18. Lober CW, Larbig GG. Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma). South Med J. Feb 1994;87(2):259-62. [Medline].

  19. Moy RL, Rivkin JE, Lee H, Brooks WS, Zitelli JA. Syringoid eccrine carcinoma. J Am Acad Dermatol. May 1991;24(5 Pt 2):857-60. [Medline].

  20. Duke WH, Sherrod TT, Lupton GP. Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited). Am J Surg Pathol. Jun 2000;24(6):775-84. [Medline].

  21. Cabell CE, Helm KF, Sakol PJ, Billingsley EM. Primary mucinous carcinoma in a 54-year-old man. J Am Acad Dermatol. Nov 2003;49(5):941-3. [Medline].

  22. Cooper PH, Adelson GL, Holthaus WH. Primary cutaneous adenoid cystic carcinoma. Arch Dermatol. Jun 1984;120(6):774-7. [Medline].

  23. Goel R, Contos MJ, Wallace ML. Widespread metastatic eccrine porocarcinoma. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S252-4. [Medline].

  24. Harrist TJ, Aretz TH, Mihm MC Jr, Evans GW, Rodriquez FL. Cutaneous malignant mixed tumor. Arch Dermatol. Nov 1981;117(11):719-24. [Medline].

  25. Hashimoto K, Mehregan AH, Kumakiri M. Tumors of Skin Appendages. Boston, Mass: Butterworth; 1987.

  26. Mitsui H, Watanabe T, Jinnin M, Kadono T, Idezuki T, Tamaki K. Mucinous carcinoma of the skin could have either an eccrine or an apocrine origin. Br J Dermatol. Dec 2004;151(6):1285-6. [Medline].

  27. Murphy GF, Elder DE. Non melanocytic tumors of the skin. In: Atlas of Tumor Pathology. 3rd Series. Fascicle 1. Washington DC: Armed Forces Institute of Pathology; 1991.

  28. Wick MR, Swanson PE, Kaye VN, Pittelkow MR. Sweat gland carcinoma ex eccrine spiradenoma. Am J Dermatopathol. Apr 1987;9(2):90-8. [Medline].

  29. Yildirim S, Aköz 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].

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Further Reading

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Keywords

eccrine carcinoma, eccrine adenocarcinoma, sweat gland tumors, malignant tumors with eccrine differentiation, microcystic adnexal carcinoma, eccrine porocarcinoma, malignant eccrine spiradenoma, digital papillary adenocarcinoma, papillary adenocarcinoma, adenoid cystic carcinoma

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Contributor Information and Disclosures

Author

Anthony Wong, MD, FAAD, Consulting Staff, Department of Dermatology, SUNY Health Science Center at Brooklyn, St Catherine's of Sienna, and Long Island Skin Cancer and Dermatologic Surgery, PC
Anthony Wong, MD, FAAD is a member of the following medical societies: American Academy of Dermatology and American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

Coauthor(s)

Darren Keith Mollick, MD, Clinical Assistant Professor, Department of Dermatology, State University of New York Downstate Medical Center
Disclosure: Nothing to disclose.

Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Medical Editor

R Stan Taylor, MD, Professor of Dermatology, University of Texas Southwestern Medical School; Director of Skin Surgery and Oncology Clinic, Department of Dermatology, University of Texas Southwestern Medical Center
R Stan Taylor, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, Christian Medical & Dental Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Mary Farley, MD, Dermatologic Surgeon/Mohs Surgeon, Anne Arundel Surgery Center
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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