Sections

Presentation

History

Microcystic adnexal carcinoma (MAC) has an indolent onset, manifesting as an indurated plaque or nodule, often present for many years. MAC is usually asymptomatic, but it may be painful. Numbness, pruritus, paresthesia, or recalcitrant facial nerve palsy may be present in patients with perineural infiltration.^[43]

Physical Examination

Note the possible physical findings for microcystic adnexal carcinoma (MAC) and the images below.

Appearance

MACs are skin colored, yellow, or pink. The most common forms are indurated plaques or nodules. Cystlike tumors have also been reported. The skin surface is usually smooth, but it may show some hyperkeratosis. It can also be atrophic and ill-defined.

Location

It is most commonly found in the head and neck region (85%), with a predilection for the nasolabial area (especially the upper lip) and the periorbital skin.^[22]A left-sided facial preponderance was noted in a large US study, considered to be the result of uneven UV exposure while driving (US steering wheels are placed on the left). MAC has been found to occur in other locations, such as the upper extremity (8%), trunk (4%), and lower extremity (4%).^[22]Involvement of the axillae, buttocks, scalp, nipple,^[44]vulva,^{[14, 45]}or genitalia^{[13, 46]}is rare but has been reported. Lesions in non–sun-exposed sites may occur more often in persons of nonwhite ethic origin. One case of multiple primary MACs (21 total) of the trunk and extremities in sun- and non–sun-exposed areas, with sparing of the face, has been reported.^[15]

Size

The average size range is 1-3 cm in diameter,^[22]and median size is about 1.5-2 cm.^{[1, 21]}One case series reported a pretreatment lesion size range of 0.03-16 cm.^{[1, 10]}Actual clinical size determination can be difficult because of the tendency for extensive subclinical involvement.

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.

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Note the subtle pink plaque on the left mid temple.

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Differential Diagnoses

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Media Gallery

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.
Note the subtle pink plaque on the left mid temple.

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Author

Nektarios I Lountzis, MD Dermatologist/Dermatopathologist, Dermatology Partners

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

Disclosure: Nothing to disclose.

Coauthor(s)

Kelley Cross Ramsauer, MD Resident Physician, Department of Dermatology, Geisinger Health System

Kelley Cross Ramsauer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for Dermatologic Surgery, Gold Humanism Honor Society, Women's Dermatologic Society

Disclosure: Nothing to disclose.

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 Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

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.

Microcystic Adnexal Carcinoma Clinical Presentation

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Physical Examination

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