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 | |
|---|---|
 | |
| 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 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
editPrimary 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
editIt'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
editDue 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
editIn 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
editReferences
edit- ^ 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.
Further reading
edit- Ramakrishnan, Rathi; Chaudhry, Iskander H.; Ramdial, Pratistadevi; Lazar, Alexander J.; McMenamin, Mairin E.; Kazakov, Dmitry; Brenn, Thomas; Calonje, Eduardo (2013). "Primary Cutaneous Adenoid Cystic Carcinoma: A Clinicopathologic and Immunohistochemical Study of 27 Cases". American Journal of Surgical Pathology. 37 (10): 1603–1611. doi:10.1097/PAS.0b013e318299fcac. ISSN 0147-5185. PMID 24025525.
- Coban, Deniz Turgut; Erol, Muhammet Kazim; Suren, Dinc; Tutus, Birsel (2015). "Primary cutaneous adenoid cystic carcinoma of the eyelid and literature review". Arquivos Brasileiros de Oftalmologia. 78 (5). GN1 Genesis Network: 323–325. doi:10.5935/0004-2749.20150085. ISSN 0004-2749. PMID 26466235.