[3] Primary cutaneous adenoid cystic carcinoma is a hard, slowly expanding, ill-defined tumor causing discomfort, itching, and secondary baldness, or may be asymptomatic.

Primary cutaneous adenoid cystic carcinoma frequently manifests as a hard, slowly expanding, ill-defined nodule or tumor that can cause symptoms including discomfort, itching, and secondary baldness, or it might be asymptomatic.

[7] Histopathology indicates that a common developmental mechanism is shared by salivary and primary cutaneous adenoid cystic carcinoma, since many of them exhibited high levels of MYB by immunohistochemistry or carried the fusion gene MYB-NFIB.

[8] Due to the lack of distinct clinical signs in primary cutaneous adenoid cystic carcinoma, the diagnosis is generally made based on the features of the tumor's histology.

A thorough clinical and radiographic examination must be performed in order to search for other indications of primary disease, particularly in the salivary glands.