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: 15 October 2020

Copyright: 2002-2020, PathologyOutlines.com, 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: 2,738
Cite this page: Tranesh G. Adenoid cystic carcinoma (primary cutaneous). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticACC.html. Accessed October 21st, 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)
Epidemiology
Sites
  • Usually head and neck (scalp), also chest / abdomen
  • Vulva / perigenital sites may correlate with more aggressive disease / metastases
Etiology
  • 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
Treatment
  • 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

Nodule with slight erythema

Scalp lesion with alopecia

Scalp lesion with alopecia

Erythematous patch on chest

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

Contributed by Mark R. Wick, M.D.



Images hosted on other servers:
Well defined deep dermal nodule

Well defined deep dermal nodule

Multiple glandular and ductal structures

Multiple glandular and ductal structures

Cribriform pattern

Cribriform pattern


Cytoplasmic blebbing

Cytoplasmic blebbing

Foci of ACC

Foci of ACC

Various images

Various images

EMA, AE1 / AE3

EMA, AE1 / AE3

Positive stains
Differential diagnosis
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