Pathology of Primary Cutaneous Adenoid Cystic Carcinoma

Dr Sampurna Roy MD                        2023

This is a rare form of sweat gland carcinoma which has a striking resemblance to the adenoid cystic carcinoma of the salivary glands. 

It was first described by Boggio in the 1975.

Adenoid cystic carcinomas are regarded by some authors as apocrine in origin.

This tumour affects middle-aged and older individuals (mean age 59) and has predilection for women.

It is characterized by indolent course and local aggressive behaviour.

Site: 

This tumour is most common in the scalp. It may be located on the chest and abdomen.

Rarely the tumour arises from the skin of the eyelid.

If the scalp is involved, alopecia is generally an associated finding.

Differential diagnoses: 

1. Adenoid basal cell carcinoma:

[Adenoid cystic carcinoma is characterized by:  Absence of retraction artefact , absence of contiguity with the epidermis or hair sheaths ,lack of peripheral palisading of the nuclei, and occasional presence of central apoptotic or even necrotic cells. Pseudocysts stain positively to Alcian blue and periodic acid-Schiff ].

2. Primary cutaneous cribriform carcinoma: 

Primary cutaneous cribriform carcinoma: a rare apocrine tumour.

Unlike most of the sweat gland carcinomas, this tumour tends to recur locally and rarely spreads to lymph nodes or distant organs.

Dermatopathology Quiz Case 7

Cutaneous Adenoid Cystic Carcinoma with Perineural Invasion Treated by Mohs Micrographic Surgery—A Case Report with Literature Review

A primary cutaneous adenoid-cystic carcinoma in a young woman. Differential diagnosis and clinical implications

Adenoid cystic carcinoma of the oral cavity: immunohistochemical study of four cases

Perineural spread of adenoid cystic carcinoma in the oral and maxillofacial regions: evaluation with contrast-enhanced CT and MRI

Primary cutaneous adenoid cystic carcinoma: a clinicopathologic and immunohistochemical study of 27 cases.

Primary Adenoid Cystic Carcinoma of the Skin Metastatic to the Lymph Nodes: Immunohistochemical Study of a New Case and Literature Review.