Skin nonmelanocytic tumor
Adnexal tumors
Sweat gland derived (apocrine & eccrine glands)
Adenoid cystic carcinoma (primary cutaneous)

Editorial Board Member: Sara C. Shalin, M.D., Ph.D.
Ghassan A. Tranesh, M.D.
Hong Qu, M.D.

Topic Completed: 1 October 2014

Minor changes: 13 December 2019

Copyright: 2002-2016,, Inc.

PubMed Search: Adenoid cystic carcinoma [title] skin

Ghassan A. Tranesh, M.D.
Hong Qu, M.D.
Page views in 2019: 4,112
Page views in 2020 to date: 2,391
Cite this page: Tranesh G. Adenoid cystic carcinoma (primary cutaneous). website. Accessed September 20th, 2020.
Definition / general
  • Primary cutaneous adenoid cystic carcinoma is a rare skin malignancy, first reported by Boggio in 1975 (J Am Acad Dermatol 2008;58:636)
  • Lesions are generally slow growing, and often present as a crusted nodule or plaque
  • More indolent than salivary gland counterpart (Am J Surg Pathol 2013;37:1603)
  • Usually head and neck (scalp), also chest / abdomen
  • Vulva / perigenital sites may correlate with more aggressive disease / metastases
  • Unclear; previously thought to originate from eccrine glands, now thought to arise from apocrine or modified apocrine glands (Dermatol Online J 2013;19:5)
Clinical features
  • Commonly recurs locally, only rarely has distant metastases
  • Must rule out cutaneous extension from salivary gland malignancy or metastasis from other sites
Case reports
  • Treated with wide local excision to reduce local recurrence
  • Recent publications advocate Moh micrographic surgery for better control of surgical margins (JAMA Dermatol 2013;149:1343)
Clinical images

Images hosted on other servers:

Nodule with slight erythema

Scalp lesion with alopecia

Erythematous patch on chest

Gross description
  • Up to 1.5 cm, smooth surfaced with a firm consistency
  • Slowly expanding, skin colored nodule
Microscopic (histologic) description
  • Deep dermal tumor, often with subcutaneous extension
  • Epidermal involvement unusual
  • Nests of basaloid cells with cribriform and tubular patterns and abundant mucin in cysts and between cells
  • Deposition of basement membrane material on the intraluminal aspect of cystic spaces
  • Mitotic activity usually low
  • Perineural invasion (76% of cases) is associated with recurrence (J Am Acad Dermatol 2008;58:636)
Microscopic (histologic) images

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.

Images hosted on other servers:

Well defined deep dermal nodule

Multiple glandular and ductal structures

Cribriform pattern

Cytoplasmic blebbing

Foci of ACC

Various images

EMA, AE1 / AE3

Positive stains
Differential diagnosis
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