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Primary cutaneous adenoid cystic carcinoma
Medical condition From Wikipedia, the free encyclopedia
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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 Primary cutaneous adenoid cystic carcinomas have been misinterpreted as metastatic lesions.[2] It was characterized in 1975.[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 is a rare condition that is believed to be caused by somatic mutations.
Primary cutaneous adenoid cystic carcinoma 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.
Primary cutaneous adenoid cystic carcinoma therapy involves broad surgical excision with a 2 cm safety margin, and lymphadenectomy if nodal involvement is suspected.
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Signs and symptoms
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.[4] The locations of predilection have been the chest and scalp (at least 40%).[5]
Causes
It's uncertain what causes primary cutaneous adenoid cystic carcinoma. They most likely stem from somatic mutations.[6] Somatic mutation patterns haven't been studied, though.[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]
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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.[6]
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.[9] When there is a surgical or clinical suspicion of nodal involvement, lymphadenectomy is carried out.[10]
See also
References
Further reading
External links
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