Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Microcystic Adnexal Carcinoma Treatment & Management

  • Author: Nektarios I Lountzis, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 27, 2016
 

Medical Care

Surgery is the mainstay of treatment. Radiation therapy has also been used with some success as either primary therapy[59] or adjuvant to surgery in approximately 25 cases to date.[60, 26, 61, 62, 63] However, radiation alone often results in recurrence. One case of a lesion of the upper lip received radiation monotherapy with recurrence in 48 months. Reirradiation revealed remission at 15-month follow-up. Only one case report of a sweat gland carcinoma of the lower lip, suspected of being a microcystic adnexal carcinoma (MAC), described success with radiation monotherapy with only a 6-month follow-up period.[64]

Of note, some tumors are also radioresistant,[59] and, as a caveat, radiation exposure is also implicated as a cause of microcystic adnexal carcinoma.[15, 65, 66] In 2 studies, a prior history of radiation exposure was found in 19.5% (14 of 84) to 50% (5 of 10) of patients with microcystic adnexal carcinoma.[3, 32]

Overall, the use of radiation therapy, as either primary or adjuvant, is inconclusive but may have a role in complicated or nonoperable cases. If considered, doses of 66-70 Gy in standard fractionation is typically recommended, extrapolating from the squamous cell head and neck literature, with target volume of the clinically apparent lesion plus 3 cm margins where possible.[62]

Next

Surgical Care

Mohs micrographic surgery (MMS) is the current treatment of choice. Clinical margins are difficult to define, and MMS offers an excellent way of following the infiltrating nature of the tumor and tracing perineural involvement. The discrepancy between clinical and histologic extent can be marked, in that the size of the defect after complete tumor removal following MMS can be as much as 4[16] to 6[5] times that of the clinically apparent size. Therefore, standardized, predictable, predetermined surgical margins cannot be used in the treatment of microcystic adnexal carcinoma (MAC).

MMS versus simple excision

In the largest series to date (48 patients), Chiller et al[16] found little difference in the overall recurrence rate between tumors treated with MMS (2.4% per person-year) versus standard surgical excision (1.5% per person-year) at a mean follow-up of 3.2 years. However, MMS exhibited a clear benefit over simple excision in that 30% of tumors treated with simple excision required at least another office visit to clear the patient of histological tumor findings, with 1 patient still not tumor free after 4 simple excisions. This rate was compared with 0% if treated by MMS. A study by Abbate et al[3] reported similar findings (MMS with 0 recurrences out of 4 cases vs excision with 1 recurrence out of 6 cases).

In a large prospective study of patients with microcystic adnexal carcinoma, only one case of recurrence was reported out of 20 (5%) at a 5-year follow-up period after MMS,[42] and a study by Friedman et al[15] with a similar follow-up period had no recurrence in 11 patients treated with MMS. Thomas et al[5] found a 12% recurrence rate with a mean follow up of 3.3 years in their study, similar to the 10.3% recurrence rate and 2-year follow up noted by Snow et al.[67] The overall recurrence rates for MMS range from 0-12%. Local recurrence rates of up to 30-47% have been reported with standard surgical excision. Recurrences have also been noted from 5 months to 30 years after excision; thus, longer follow-up is necessary to fully assess both treatment modalities.

Some have suggested the use of an additional layer after complete clearance of margins with MMS for further histologic control.[8, 68] No outcome data exist on this technique.

Staged excision (slow MMS)

Problems can also be encountered in interpreting frozen sections during MMS, when microscopic invasion can be elusive. Barlow et al[69] proposed that delayed-closure MMS using formalin-fixed, paraffin-embedded specimens improves the histologic assessment and decreases the risk of missing a strand of tumor. Some advocate the use of tangential frozen sections with MMS plus formalin-fixed paraffin sections for the final layer, and this is a workable compromise. Moreover, other reports advocate the use of toluidine-blue staining on frozen sections to highlight microcystic adnexal carcinoma extension. Tumor stroma has a pink halo, owing to the presence of mucopolysaccharide and hyaluronic acid, and perineural involvement has a magenta hue, thereby augmenting visualization and clearance with MMS.[47]

Previous
Next

Complications

Local recurrence is a complication if it is not properly excised. Additionally, because of the deep penetration of the tumor, destruction of underlying tissue (eg, bone, cartilage, muscle) can lead to structural changes and increased morbidity.

Previous
Next

Prevention

Sun avoidance minimizes UV exposure, which may be a predisposing factor.

Previous
Next

Long-Term Monitoring

The longest latent period for microcystic adnexal carcinoma (MAC) recurrence following simple excision is 30 years. This particular patient underwent a simple excision for adnexal adenoma in 1953. When he presented again 30 years later, the initial slides were reviewed, and a retrospective diagnosis of microcystic adnexal carcinoma was made. This case illustrates the indolent nature of microcystic adnexal carcinoma and supports the view that simple excision may be not adequate and long-term follow-up care is required.

Previous
 
Contributor Information and Disclosures
Author

Nektarios I Lountzis, MD Consulting Staff, Advanced Dermatology Associates, Ltd, Lehigh Valley Health Network

Nektarios I Lountzis, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, American Contact Dermatitis Society, International Society of Dermatopathology

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 Surgery, American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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

Disclosure: Nothing to disclose.

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

Kelly M Cordoro, MD Assistant Professor of Clinical Dermatology and Pediatrics, 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, Medical Society of Virginia, Society for Pediatric Dermatology, Women's Dermatologic Society, Association of Professors of Dermatology, National Psoriasis Foundation, Dermatology Foundation

Disclosure: Nothing to disclose.

Acknowledgements

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

Disclosure: Nothing to disclose.

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

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

Disclosure: Nothing to disclose.

Kevin Hollowood, MD, MBBS, MRCP, FRCPath Consulting Staff, Department of Pathology, John Radcliffe Hospital of Oxford, UK

Disclosure: Nothing to disclose.

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

  2. Cooper PH, Mills SE. Microcystic adnexal carcinoma. J Am Acad Dermatol. 1984 May. 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. 2003 Oct. 29(10):1035-8. [Medline].

  4. Page RN, Hanggi MC, King R, Googe PB. Multiple microcystic adnexal carcinomas. Cutis. 2007 Apr. 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. 2007 Mar. 33(3):333-9. [Medline].

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

  7. Yu JB, Blitzblau RC, Patel SC, Decker RH, Wilson LD. Surveillance, Epidemiology, and End Results (SEER) database analysis of microcystic adnexal carcinoma (sclerosing sweat duct carcinoma) of the skin. Am J Clin Oncol. 2010 Apr. 33(2):125-7. [Medline].

  8. 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. 1997 Jun. 27(3):197-9. [Medline].

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

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

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

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

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

  14. Nelson PS, Bourgeois KM, Nicotri T Jr, Chiu ES, Poole JC. Sclerosing sweat duct carcinoma in a 6-year-old African American child. Pediatr Dermatol. 2008 Jan-Feb. 25(1):38-42. [Medline].

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

  16. 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. 2000 Nov. 136(11):1355-9. [Medline].

  17. Fu T, Clark FL, Lorenz HP, Bruckner AL. Congenital microcystic adnexal carcinoma. Arch Dermatol. 2011 Feb. 147(2):256-7. [Medline].

  18. Smart DR, Taintor AR, Kelly ME, Lyon VB, Segura A, Jensen JN, et al. Microcystic adnexal carcinoma: the first reported congenital case. Pediatr Dermatol. 2011 Jan-Feb. 28(1):35-8. [Medline].

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

  20. Burns MK, Chen SP, Goldberg LH. Microcystic adnexal carcinoma. Ten cases treated by Mohs micrographic surgery. J Dermatol Surg Oncol. 1994 Jul. 20(7):429-34. [Medline].

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

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

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

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

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

  26. 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. 2009 Jun. 44(3):e17-8. [Medline].

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

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

  29. 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].

  30. Wu-Chen WY, Weng CY, Rajan KD, Eberhart C, Miller NR. Unusual presentation of primary orbital microcystic adnexal carcinoma. J Neuroophthalmol. 2011 Jun. 31(2):147-50. [Medline].

  31. 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. 2006 Aug. 28(4):346-51. [Medline].

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

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

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

  35. Weissferdt A, Moran CA. Microcystic squamous cell carcinoma of the lung: a clinicopathologic study of three cases. Am J Clin Pathol. 2011 Sep. 136(3):436-41. [Medline].

  36. Schaller J, Rytina E, Rütten A, Hendricks C, Ha T, Requena L. Sweat duct proliferation associated with aggregates of elastic tissue and atrophodermia vermiculata: a simulator of microcystic adnexal carcinoma. Report of two cases. J Cutan Pathol. 2010 Sep. 37(9):1002-9. [Medline].

  37. Tawfik AM, Kreft A, Wagner W, Vogl TJ. MRI of a microcystic adnexal carcinoma of the skin mimicking a fibrous tumour: case report and literature review. Br J Radiol. 2011 Jun. 84(1002):e114-7. [Medline].

  38. Inskip M, Magee J. Microcystic adnexal carcinoma of the cheek-a case report with dermatoscopy and dermatopathology. Dermatol Pract Concept. 2015 Jan. 5 (1):43-6. [Medline].

  39. Alawi SA, Batz S, Röwert-Huber J, Fluhr JW, Lademann J, Ulrich M. Correlation of optical coherence tomography and histology in microcystic adnexal carcinoma: a case report. Skin Res Technol. 2014 Feb 17. [Medline].

  40. Giambrone D, Salvaggio C, Victor FC, Rao BK. Microcystic Adnexal Carcinoma Detected by Reflectance Confocal Microscopy. Dermatol Surg. 2016 Jan. 42 (1):126-7. [Medline].

  41. Sirikanjanapong S, Seymour AW, Amin B. Cytologic features of microcystic adnexal carcinoma. Cytojournal. 2011 Mar 3. 8:5. [Medline]. [Full Text].

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

  43. Cooper PH, Mills SE, Leonard DD, Santa Cruz DJ, Headington JT, Barr RJ, et al. Sclerosing sweat duct (syringomatous) carcinoma. Am J Surg Pathol. 1985 Jun. 9(6):422-33. [Medline].

  44. McCalmont TH, Ye J. Eosinophils as a clue to the diagnosis of microcystic adnexal carcinoma. J Cutan Pathol. 2011 Nov. 38(11):849, 850-2. [Medline].

  45. 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. 1993 Oct. 29(4):609-18. [Medline].

  46. Kazakov DV, Kacerovska D, Michal M. Microcystic adnexal carcinoma with multiple areas of follicular differentiation toward germinative cells and specific follicular stroma (trichoblastomatous areas). Am J Dermatopathol. 2011 Jun. 33(4):e47-9. [Medline].

  47. 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. 2007 Jun. 56(6):1067-9. [Medline].

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

  49. Krahl D, Sellheyer K. p75 Neurotrophin receptor differentiates between morphoeic basal cell carcinoma and desmoplastic trichoepithelioma: insights into the histogenesis of adnexal tumours based on embryology and hair follicle biology. Br J Dermatol. 2010 Jul. 163(1):138-45. [Medline].

  50. Jedrych J, McNiff JM. Expression of p75 neurotrophin receptor in desmoplastic trichoepithelioma, infiltrative basal cell carcinoma, and microcystic adnexal carcinoma. Am J Dermatopathol. 2013 May. 35(3):308-15. [Medline].

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

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

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

  54. Jedrych J, Leffell D, McNiff JM. Desmoplastic trichoepithelioma with perineural involvement: a series of seven cases. J Cutan Pathol. 2012 Mar. 39(3):317-23. [Medline].

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

  56. 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. 2010 Apr. 37(4):403-10. [Medline].

  57. Rollins-Raval M, Chivukula M, Tseng GC, Jukic D, Dabbs DJ. An immunohistochemical panel to differentiate metastatic breast carcinoma to skin from primary sweat gland carcinomas with a review of the literature. Arch Pathol Lab Med. 2011 Aug. 135(8):975-83. [Medline].

  58. 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. 2009 Dec. 31(8):849-52. [Medline].

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

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

  61. Baxi S, Deb S, Weedon D, Baumann K, Poulsen M. Microcystic adnexal carcinoma of the skin: the role of adjuvant radiotherapy. J Med Imaging Radiat Oncol. 2010 Oct. 54(5):477-82. [Medline].

  62. Pugh TJ, Lee NY, Pacheco T, Raben D. Microcystic adnexal carcinoma of the face treated with radiation therapy: A case report and review of the literature. Head Neck. 2012 Jul. 34(7):1045-50. [Medline].

  63. Kim LH, Teston L, Sasani S, Henderson C. Microcystic adnexal carcinoma: successful management of a large scalp lesion. J Plast Surg Hand Surg. 2014 Apr. 48(2):158-60. [Medline].

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

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

  66. Borenstein A, Seidman DS, Trau H, Tsur H. Microcystic adnexal carcinoma following radiotherapy in childhood. Am J Med Sci. 1991 Apr. 301(4):259-61. [Medline].

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

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

  69. 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. 1996 Dec. 135(6):911-7. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.