Primary cutaneous adenoid cystic carcinoma

Primary cutaneous adenoid cystic carcinoma
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 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.

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]

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]

References

^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 1710. ISBN 978-1-4160-2999-1.
^ Naylor E, Sarkar P, Perlis CS, Giri D, Gnepp DR, Robinson-Bostom L (April 2008). "Primary cutaneous adenoid cystic carcinoma". J. Am. Acad. Dermatol. 58 (4): 636–41. doi:10.1016/j.jaad.2007.12.005. PMID 18342709.
^ Tiwari, Raja; Agarwal, Savita; Sharma, Manik; Gaba, Sunil (2018). "Primary cutaneous adenoid cystic carcinoma: A clinical and histopathological mimic: A case report". Oral and Maxillofacial Surgery Cases. 4 (4). Elsevier BV: 175–179. doi:10.1016/j.omsc.2018.09.002. ISSN 2214-5419.
^ Ko, Y. H.; Lee, M. A.; Hong, Y. S.; Lee, K. S.; Jung, C.-K.; Kim, Y. S.; Sun, D.-I.; Kim, B. S.; Kim, M. S.; Kang, J. H. (2007-10-10). "Prognostic Factors Affecting the Clinical Outcome of Adenoid Cystic Carcinoma of the Head and Neck". Japanese Journal of Clinical Oncology. 37 (11). Oxford University Press (OUP): 805–811. doi:10.1093/jjco/hym119. ISSN 0368-2811. PMID 18057012.
^ ^a ^b Temnithikul, Bhakinai; Rungrunanghiranya, Suthat; Limtanyakul, Piyakan; Jerasuthat, Suthep; Paige, David G. (2022-04-28). "Primary cutaneous adenoid cystic carcinoma of the scalp: A case report, immunohistochemistry and review of the literature". Skin Health and Disease. 2 (2). Wiley: e118. doi:10.1002/ski2.118. ISSN 2690-442X. PMC 9168016. PMID 35677929.
^ Rütten, Arno; Hegenbarth, Wolfgang; Kohl, Peter K.; Hillen, Uwe; Redler, Silke (2018). "Primary cutaneous adenoid cystic carcinoma mimicking dermal cylindroma: histology of the complete surgical excision as the key to diagnosis". JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 16 (8). Wiley: 1016–1018. doi:10.1111/ddg.13600. ISSN 1610-0379. PMID 30094921.
^ North, Jeffrey P.; McCalmont, Timothy H.; Fehr, André; van Zante, Annemieke; Stenman, Göran; LeBoit, Philip E. (2015). "Detection of MYB Alterations and Other Immunohistochemical Markers in Primary Cutaneous Adenoid Cystic Carcinoma". American Journal of Surgical Pathology. 39 (10). Ovid Technologies (Wolters Kluwer Health): 1347–1356. doi:10.1097/pas.0000000000000463. ISSN 0147-5185. PMID 26076064.
^ Raychaudhuri, Sujata; Santosh, Kv; Satish Babu, Hv (2012). "Primary cutaneous adenoid cystic carcinoma of the chest wall: A rare entity". Journal of Cancer Research and Therapeutics. 8 (4): 633. doi:10.4103/0973-1482.106583. ISSN 0973-1482. PMID 23361287.
^ Lestouquet, Fabienne Robuschi; Moya, Ana Isabel Sánchez; Guerra, Silvia Honorato; Alzate, Cristina Janeth Cardona (2013). "Primary cutaneous adenoid cystic carcinoma: An unusual case". Dermatology Online Journal. 19 (1). California Digital Library (CDL). doi:10.5070/d38w41t9kb. ISSN 1087-2108.