Primary cutaneous adenoid cystic carcinoma
From Wikipedia, the free encyclopedia
| Primary cutaneous adenoid cystic carcinoma | |
|---|---|
 | |
| Primary cutaneous adenoid cystic carcinoma (PCACC) of the right lower eyelid. The micrograph shows cells covering cystic spaces showing arrangement in two cell layers. | |
| Specialty | Dermatology |
Primary cutaneous adenoid cystic carcinoma is a cutaneous condition characterized by a tumor that usually presents on the chest, scalp, or vulva of middle- to older-aged persons.[1]: 670 It is a hard, slow growing, ill-defined tumor causing discomfort, itching, and secondary baldness, or may be asymptomatic. The condition is rare and believed to be caused by somatic mutations. It was characterized in 1975.[2]
Diagnosis relies on tumor histology features, but a comprehensive clinical and radiographic examination is necessary to identify other primary disease indications, especially in salivary glands. The condition is treated with broad surgical excision with a 2 cm safety margin, and lymphadenectomy if nodal involvement is suspected.
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.[3] The locations of predilection have been the chest and scalp (at least 40%).[4]
Causes
It's uncertain what causes primary cutaneous adenoid cystic carcinoma. They most likely stem from somatic mutations.[5] Somatic mutation patterns haven't been studied, though.[6] 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.[7]
Diagnosis
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.[5]
Treatment
In order to prevent the tumor's frequent recurrence, the usual therapy for primary cutaneous adenoid cystic carcinoma involves a broad surgical excision with at least a 2 cm safety margin from the tumor.[8] When there is a surgical or clinical suspicion of nodal involvement, lymphadenectomy is carried out.[9]