Skin nonmelanocytic tumor
Adnexal tumors
Sweat gland derived (apocrine & eccrine glands)
Adenoid cystic carcinoma (primary cutaneous)
Authors: Ghassan A. Tranesh, M.D., Hong Qu, M.D.
Editorial Board Member: Sara C. Shalin, M.D., Ph.D.
Last author update: 1 October 2014
Last staff update: 6 November 2020
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Adenoid cystic carcinoma [title] skin
Table of Contents
Definition / general | Epidemiology | Sites | Etiology | Clinical features | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosis | Additional referencesCite this page: Tranesh G. Adenoid cystic carcinoma (primary cutaneous). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticACC.html. Accessed May 29th, 2023.
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
- Commonly arises in seventh decade, mean age 59 years, 53% women (J Am Acad Dermatol 2008;58:636)
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
- 32 and 33 year old women (Rare Tumors 2011;3:e3, Dermatol Online J 2013;19:5)
- 55 year old man with primary cutaneous adenoid cystic carcinoma with distant metastasis (Indian J Dermatol Venereol Leprol 2010;76:176)
- 70 year old man with groin nodule (JAMA Dermatol 2013;149:1343)
- Primary cutaneous adenoid cystic carcinoma (J Am Acad Dermatol 2008;58:636)
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
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
Contributed by 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
- EMA, AE1 / AE3 (Dermatol Online J 2013;19:5)
- CD117 (c-kit) has diffuse expression, as seen in its salivary gland counterpart
- Often a myoepithelial component is focally present, which is immunoreactive for S100 protein and calponin, with smooth muscle actin expression in cells surrounding the tumor cells
Differential diagnosis
- Basal cell carcinoma (adenoid variant)
- Cylindroma or spiradenoma
- Metastases from salivary gland tumor (adenoid cystic carcinoma)
- Mucinous carcinoma
- Primary cutaneous cribriform apocrine carcinoma
Additional references
