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Primary cutaneous adenoid cystic carcinoma

Field	Value
image	Micrograph of adenoid cystic carcinoma.jpg
caption	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. Primary cutaneous adenoid cystic carcinomas have been misinterpreted as metastatic lesions. It was characterized in 1975.

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.

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. The locations of predilection have been the chest and scalp (at least 40%).

Causes

It's uncertain what causes primary cutaneous adenoid cystic carcinoma. They most likely stem from somatic mutations. Somatic mutation patterns haven't been studied, though. 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.

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.

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. When there is a surgical or clinical suspicion of nodal involvement, lymphadenectomy is carried out.

References

James, William D.. (2006). "Andrews' Diseases of the Skin: Clinical Dermatology". Saunders Elsevier.
Rapini, Ronald P.. (2007). "Dermatology: 2-Volume Set". Mosby.
(April 2008). "Primary cutaneous adenoid cystic carcinoma". J. Am. Acad. Dermatol..
(2018). "Primary cutaneous adenoid cystic carcinoma: A clinical and histopathological mimic: A case report". Elsevier BV.
(2007-10-10). "Prognostic Factors Affecting the Clinical Outcome of Adenoid Cystic Carcinoma of the Head and Neck". Oxford University Press (OUP).
(2022-04-28). "Primary cutaneous adenoid cystic carcinoma of the scalp: A case report, immunohistochemistry and review of the literature". Wiley.
(2018). "Primary cutaneous adenoid cystic carcinoma mimicking dermal cylindroma: histology of the complete surgical excision as the key to diagnosis". Wiley.
(2015). "Detection of MYB Alterations and Other Immunohistochemical Markers in Primary Cutaneous Adenoid Cystic Carcinoma". Ovid Technologies (Wolters Kluwer Health).
(2012). "Primary cutaneous adenoid cystic carcinoma of the chest wall: A rare entity". Journal of Cancer Research and Therapeutics.
(2013). "Primary cutaneous adenoid cystic carcinoma: An unusual case". California Digital Library (CDL).

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

epidermal-nevi,-neoplasms,-and-cysts

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