Microcystic Adnexal Carcinoma Treatment & Management

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

Medical Care

Surgery is the mainstay of treatment. Radiation therapy has also been used as either primary therapy[39] or adjuvant to surgery in approximately 7 cases to date.[40, 11] However, radiation alone often results in recurrence. 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.[41] Some tumors are also radioresistant,[39] and, as a caveat, radiation exposure is also implicated as a cause of microcystic adnexal carcinoma. 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 in 2 studies.[3, 28] Overall, the paucity of cases that have used radiation as either primary or adjuvant therapy makes it difficult to have definitive conclusions on its efficacy.

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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[24] 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[24] 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,[25] and a study by Friedman et al[23] 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.[42] 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.[21, 43] 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[44] 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.[31]

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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.

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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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