Microcystic Adnexal Carcinoma 

  • Author: Nektarios I Lountzis, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 28, 2010
 

Background

Microcystic adnexal carcinoma (MAC) is a rare, malignant appendage tumor commonly classified as a low-grade sweat gland carcinoma that typically occurs on the head and neck, particularly the central face. Microcystic adnexal carcinoma shows aggressive local invasion but has little metastatic potential. Goldstein et al[1] described the first series in 1982, emphasizing the importance of its distinction from the benign histologic mimics termed syringoma, desmoplastic trichoepithelioma, and trichoadenoma. Note the image below.

Clinical photo of a microcystic adnexal carcinoma Clinical photo of a microcystic adnexal carcinoma on the left upper lip of an elderly woman. Note the close clinical resemblance to basal cell carcinoma. Courtesy of Dirk M. Elston, MD.

Despite subsequent widespread recognition of microcystic adnexal carcinoma as a discrete clinicopathologic entity, its precise relationship to and histologic discrimination from other putative locally aggressive sweat gland carcinomas (reported under a variety of names, including sclerosing sweat duct carcinoma, syringoid eccrine carcinoma, syringomatous carcinoma, and eccrine epithelioma) remains unresolved and has provoked considerable nosologic and diagnostic confusion. The authors regard microcystic adnexal carcinoma as synonymous with group 1 sclerosing sweat duct carcinomas as described by Cooper et al.[2] In addition, the tumor described as syringomatous adenoma of the nipple is regarded by some authorities as microcystic adnexal carcinoma.

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Pathophysiology

The differentiation pathway of microcystic adnexal carcinoma (MAC) has provoked considerable debate. Goldstein et al[1] initially suggested that it showed dual pilar (the superficially located keratocysts resembling follicular infundibula) and eccrine differentiation. Others have supported this notion, noting, in particular, that the keratocysts often expressed pilar-type keratins and that occasionally, trichohyalin granules are present. However, some authors have suggested that microcystic adnexal carcinoma shows only eccrine differentiation.

More recently, based on the ontogenetic relationship between hair follicles and apocrine glands, microcystic adnexal carcinomas have been theorized to display folliculo-apocrine or sometimes folliculo-sebaceous-apocrine differentiation rather than folliculo-eccrine differentiation. Support for this premise is provided by occasional cases showing focal apocrine decapitation secretion and the demonstration of sebaceous foci in some tumors.

Little information is available on the molecular pathophysiology of microcystic adnexal carcinomas, although 1 report documented a deletion on arm 6q in 1 case. A case of microcystic adnexal carcinoma occurring in siblings also suggests a genetic role.[3]

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Epidemiology

Frequency

United States

Microcystic adnexal carcinoma is a rare tumor, with only slightly more than 300 cases reported worldwide.[4] Thomas et al[5] reported a mean number of 1.63 cases per year in a rural northeastern area of the United States.

Mortality/Morbidity

Morbidity is high because of the deeply infiltrating nature of the tumor, which can invade into bone, muscle, blood vessels, cartilage, and nerves. In one study, the mean clinical lesion size was 3 cm2, but the final defect size was 18 cm2, highlighting its occult extension.[5]

Mortality from metastatic disease is rare, with only 6 local[6, 7, 8, 9, 10, 11] and 3 distant metastases reported.[12, 13, 14] Local metastatic disease may, in most cases, be occult contiguous extension along neurovasculature bundles.[9, 10, 11] Considering that more than 300 cases have been reported in the medical literature worldwide,[4] only 2 deaths have been attributed to microcystic adnexal carcinoma,[15, 11] both due to metastatic disease.

Of possible relation, Fernandez-Figueras et al reported a case of a high-grade carcinosarcoma with an architectural pattern similar to microcystic adnexal carcinoma but also exhibiting nuclear pleomorphism, hyperchromasia, and large nucleoli histologically. The patient developed metastatic lung disease from the tumor and died within 6 months.[16] The authors further noted histologic high-grade features in their case that were similar to other cases reported with metastatic disease,[10, 13, 15] and they suggested this may be an indicator for more aggressive disease. Whether this particular tumor represents an undefined adnexal carcinoma with an architectural pattern similar to microcystic adnexal carcinoma or a morphological variant of microcystic adnexal carcinoma with high-grade histologic features remains unknown.

Race

Most cases of microcystic adnexal carcinoma occur in whites, but it may occur in persons of other races such as Latinos and Asians.[17] Furthermore, 8 cases have been reported in 7 African American patients[4, 15, 17, 18, 19, 20] and a large series of 51 cases has been reported in a Japan.[21, 22]

It is uncertain whether sun exposure plays a role in persons of African American origin, with one lesion occurring on the vulva of an African American woman[19] and multiple lesions occurring on the trunk and extremities (with sparing of the face) in an African American man.[4] However, other reported cases in African Americans have occurred on the scalp[18, 20] and upper cutaneous lip,[15] where sun exposure is prominent.

In the large series from Japan, distribution was found to be similar to that of whites.[22] The overall clinical behavior of microcystic adnexal carcinoma appears to be similar in all races.

Sex

No significant sexual predilection is reported, although some studies suggest a slight female predominance.[3, 23, 24]

Age

The age range is broad; microcystic adnexal carcinoma has been reported in a 10-year-old girl,[25] an 11-year-old boy,[26] and a 90-year-old person.[27] Microcystic adnexal carcinoma may manifest at any time from the second to the eighth decade of life, with most patients in their 40s, 50s, or 60s.[3, 27] In one series of cases, the mean age was found to be slightly higher (70 y).[5]

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Contributor Information and Disclosures
Author

Nektarios I Lountzis, MD  Consulting Staff, Departments of Dermatology and Pathology, Geisinger Wyoming Valley Medical Center

Nektarios I Lountzis, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Mary Grace Petrick, MD  Consulting Staff, Department of Dermatology, Geisinger Medical Center

Mary Grace Petrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, and American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Bruce C Gee, MB, BCh, MRCP  Specialist Registrar, Department of Dermatology, Queen's Medical Centre, Nottingham, UK

Bruce C Gee, MB, BCh, MRCP is a member of the following medical societies: Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Kelly M Cordoro, MD  Assistant Professor of Pediatric and Adult Dermatology, Department of Dermatology, University of California, San Francisco School of Medicine

Kelly M Cordoro, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Association of Professors of Dermatology, Dermatology Foundation, Medical Society of Virginia, National Psoriasis Foundation, Society for Pediatric Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Mary Farley, MD  Dermatologic Surgeon/Mohs Surgeon, Anne Arundel Surgery Center

Disclosure: Nothing to disclose.

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.

References

  1. Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma: a distinct clinicopathologic entity. Cancer. Aug 1 1982;50(3):566-72. [Medline].

  2. Cooper PH, Mills SE. Microcystic adnexal carcinoma. J Am Acad Dermatol. May 1984;10(5 Pt 2):908-14. [Medline].

  3. 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].

  4. Page RN, Hanggi MC, King R, Googe PB. Multiple microcystic adnexal carcinomas. Cutis. Apr 2007;79(4):299-303. [Medline].

  5. Thomas CJ, Wood GC, Marks VJ. Mohs micrographic surgery in the treatment of rare aggressive cutaneous tumors: the Geisinger experience. Dermatol Surg. Mar 2007;33(3):333-9. [Medline].

  6. Bier-Laning CM, Hom DB, Gapany M, Manivel JC, Duvall AJ 3rd. Microcystic adnexal carcinoma: management options based on long-term follow-up. Laryngoscope. Nov 1995;105(11):1197-201. [Medline].

  7. Kirkland PM, Solomons NB, Ratcliffe NA. Microcystic adnexal carcinoma. J Laryngol Otol. Jul 1997;111(7):674-5. [Medline].

  8. Carroll P, Goldstein GD, Brown CW Jr. Metastatic microcystic adnexal carcinoma in an immunocompromised patient. Dermatol Surg. Jun 2000;26(6):531-4. [Medline].

  9. Rotter N, Wagner H, Fuchshuber S, Issing WJ. Cervical metastases of microcystic adnexal carcinoma in an otherwise healthy woman. Eur Arch Otorhinolaryngol. May 2003;260(5):254-7. [Medline].

  10. Ban M, Sugie S, Kamiya H, Kitajima Y. Microcystic adnexal carcinoma with lymph node metastasis. Dermatology. 2003;207(4):395-7. [Medline].

  11. Gomez-Maestra MJ, Espana-Gregori E, Avino-Martinez JA, Mancheno-Franch N, Pena S. Brainstem and cavernous sinus metastases arising from a microcystic adnexal carcinoma of the eyebrow by perineural spreading. Can J Ophthalmol. Jun 2009;44(3):e17-8. [Medline].

  12. Yugueros P, Kane WJ, Goellner JR. Sweat gland carcinoma: a clinicopathologic analysis of an expanded series in a single institution. Plast Reconstr Surg. Sep 1998;102(3):705-10. [Medline].

  13. Ohta M, Hiramoto M, Ohtsuka H. Metastatic microcystic adnexal carcinoma: an autopsy case. Dermatol Surg. Jun 2004;30(6):957-60. [Medline].

  14. Gabillot-Carré M, Weill F, Mamelle G, et al. Microcystic adnexal carcinoma: report of seven cases including one with lung metastasis. Dermatology. 2006;212(3):221-8. [Medline].

  15. 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].

  16. Fernández-Figueras MT, Montero MA, Admella J, de la Torre N, Quer A, Ariza A. High (nuclear) grade adnexal carcinoma with microcystic adnexal carcinoma-like structural features. Am J Dermatopathol. Aug 2006;28(4):346-51. [Medline].

  17. Nadiminti H, Nadiminti U, Washington C. Microcystic adnexal carcinoma in African-Americans. Dermatol Surg. Nov 2007;33(11):1384-7. [Medline].

  18. Park JY, Parry EL. Microcystic adnexal carcinoma. First reported case in a black patient. Dermatol Surg. Aug 1998;24(8):905-7. [Medline].

  19. Buhl A, Landow S, Lee YC, Holcomb K, Heilman E, Abulafia O. Microcystic adnexal carcinoma of the vulva. Gynecol Oncol. Sep 2001;82(3):571-4. [Medline].

  20. 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].

  21. Murata S, Fujita S, Sugihara K, Akasu T, Moriya Y, Nakanishi Y. Sclerosing sweat duct carcinoma in the peri-anal skin: a case report. Jpn J Clin Oncol. Jun 1997;27(3):197-9. [Medline].

  22. Ohtsuka H, Nagamatsu S. Microcystic adnexal carcinoma: review of 51 Japanese patients. Dermatology. 2002;204(3):190-3. [Medline].

  23. Friedman PM, Friedman RH, Jiang SB, Nouri K, Amonette R, Robins P. Microcystic adnexal carcinoma: collaborative series review and update. J Am Acad Dermatol. Aug 1999;41(2 Pt 1):225-31. [Medline].

  24. 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].

  25. Leibovitch I, Huilgol SC, Selva D, Lun K, Richards S, Paver R. Microcystic adnexal carcinoma: treatment with Mohs micrographic surgery. J Am Acad Dermatol. Feb 2005;52(2):295-300. [Medline].

  26. McAlvany JP, Stonecipher MR, Leshin B, Prichard E, White W. Sclerosing sweat duct carcinoma in an 11-year-old boy. J Dermatol Surg Oncol. Nov 1994;20(11):767-8. [Medline].

  27. Cooper PH. Carcinomas of sweat glands. Pathol Annu. 1987;22 Pt 1:83-124. [Medline].

  28. Eisen DB, Zloty D. Microcystic adnexal carcinoma involving a large portion of the face: when is surgery not reasonable?. Dermatol Surg. Nov 2005;31(11 Pt 1):1472-7; discussion 1478. [Medline].

  29. Antley CA, Carney M, Smoller BR. Microcystic adnexal carcinoma arising in the setting of previous radiation therapy. J Cutan Pathol. Jan 1999;26(1):48-50. [Medline].

  30. Schwarze HP, Loche F, Lamant L, Kuchta J, Bazex J. Microcystic adnexal carcinoma induced by multiple radiation therapy. Int J Dermatol. May 2000;39(5):369-72. [Medline].

  31. Wang SQ, Goldberg LH, Nemeth A. The merits of adding toluidine blue-stained slides in Mohs surgery in the treatment of a microcystic adnexal carcinoma. J Am Acad Dermatol. Jun 2007;56(6):1067-9. [Medline].

  32. Vidal CI, Goldberg M, Burstein DE, Emanuel HJ, Emanuel PO. p63 Immunohistochemisty Is a Useful Adjunct in Distinguishing Sclerosing Cutaneous Tumors. Am J Dermatopathol. Jan 21 2010;[Medline].

  33. Hoang MP, Dresser KA, Kapur P, High WA, Mahalingam M. Microcystic adnexal carcinoma: an immunohistochemical reappraisal. Mod Pathol. Feb 2008;21(2):178-85. [Medline].

  34. Krahl D, Sellheyer K. Monoclonal antibody Ber-EP4 reliably discriminates between microcystic adnexal carcinoma and basal cell carcinoma. J Cutan Pathol. Oct 2007;34(10):782-7. [Medline].

  35. Bogner PN, Su LD, Fullen DR. Cluster designation 5 staining of normal and non-lymphoid neoplastic skin. J Cutan Pathol. Jan 2005;32(1):50-4. [Medline].

  36. Wick MR, Cooper PH, Swanson PE, Kaye VN, Sun TT. Microcystic adnexal carcinoma. An immunohistochemical comparison with other cutaneous appendage tumors. Arch Dermatol. Feb 1990;126(2):189-94. [Medline].

  37. Plaza JA, Ortega PF, Stockman DL, Suster S. Value of p63 and podoplanin (D2-40) immunoreactivity in the distinction between primary cutaneous tumors and adenocarcinomas metastatic to the skin: a clinicopathologic and immunohistochemical study of 79 cases. J Cutan Pathol. Apr 2010;37(4):403-10. [Medline].

  38. Kavand S, Cassarino DS. "Squamoid eccrine ductal carcinoma": an unusual low-grade case with follicular differentiation. Are these tumors squamoid variants of microcystic adnexal carcinoma?. Am J Dermatopathol. Dec 2009;31(8):849-52. [Medline].

  39. Stein JM, Ormsby A, Esclamado R, Bailin P. The effect of radiation therapy on microcystic adnexal carcinoma: a case report. Head Neck. Mar 2003;25(3):251-4. [Medline].

  40. Wetter R, Goldstein GD. Microcystic adnexal carcinoma: a diagnostic and therapeutic challenge. Dermatol Ther. Nov-Dec 2008;21(6):452-8. [Medline].

  41. Gulmen S, Pullon PA. Sweat gland carcinoma of the lips. Oral Surg Oral Med Oral Pathol. May 1976;41(5):643-9. [Medline].

  42. 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].

  43. Khachemoune A, Olbricht SM, Johnson DS. Microcystic adnexal carcinoma: report of four cases treated with Mohs' micrographic surgical technique. Int J Dermatol. Jun 2005;44(6):507-12. [Medline].

  44. Barlow RJ, Ramnarain N, Smith N, Mayou B, Markey AC, Walker NP. Excision of selected skin tumours using Mohs' micrographic surgery with horizontal paraffin-embedded sections. Br J Dermatol. Dec 1996;135(6):911-7. [Medline].

  45. Kumar K, McGregor JC, Watson JD. Microcystic adnexal carcinoma: a report of three cases. J R Coll Surg Edinb. Dec 1998;43(6):412-4. [Medline].

  46. LeBoit PE, Sexton M. Microcystic adnexal carcinoma of the skin. A reappraisal of the differentiation and differential diagnosis of an underrecognized neoplasm. J Am Acad Dermatol. Oct 1993;29(4):609-18. [Medline].

  47. Nagatsuka H, Rivera RS, Gunduz M, et al. Microcystic adnexal carcinoma with mandibular bone marrow involvement: a case report with immunohistochemistry. Am J Dermatopathol. Dec 2006;28(6):518-22. [Medline].

  48. Nickoloff BJ, Fleischmann HE, Carmel J, Wood CC, Roth RJ. Microcystic adnexal carcinoma. Immunohistologic observations suggesting dual (pilar and eccrine) differentiation. Arch Dermatol. Mar 1986;122(3):290-4. [Medline].

  49. Reqena L, Kiryu H, Ackerman AB. Neoplasms With Apocrine Differentiation. Philadelphia: Lippincott-Raven; 1998.

 
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Clinical photo of a microcystic adnexal carcinoma on the left upper lip of an elderly woman. Note the close clinical resemblance to basal cell carcinoma. Courtesy of Dirk M. Elston, MD.
A low-power view of microcystic adnexal carcinoma demonstrates superficially located keratocysts and variably sized tumor nests and ducts. Note the diminution in size of the nests and cysts with the depth of dermal invasion. Courtesy of Dirk M. Elston, MD
Small ductular structures lined by 2-3 cell layers of small eosinophilic cells showing little pleomorphism set in a dense fibrous stroma. Courtesy of Dirk M. Elston, MD.
A high-power view of small, irregularly shaped nests and strands of small tumor cells without obvious ductal formation. Courtesy of Dirk M. Elston, MD.
 
 
 
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