Giant Primary Cutaneous Adenoid Cystic Carcinoma of the Perineum: Histological and Radiological Correlations

Adenoid cystic carcinoma (ACC) is a relatively rare slow growing and often-aggressive epithelial-myoepithelial neoplasm that arises in multiple organs including the skin. The t(6;9) (q22–23;p23–24) translocation, resulting in a MYB-NFIB gene fusion has been found in ACCs from the salivary glands and other organs. Recently, MYB aberrations occurring in a subset (40%) of primary cutaneous ACC (PCACC) examples was described. Herein, we report 3 additional cases of PCACC harboring MYB aberrations. The tumors presented in 3 males aged 43, 81 and 55 years old and affected the extremities in the first 2 patients and the scalp in the third one. None of the patients had history of prior or concurrent ACC elsewhere. Lesions exhibited the classic ACC morphology of nests of basaloid cells arranged in cribriform and adenoid patterns. Sentinel lymph node biopsy was performed in two cases with one case showing lymph node positivity. Fluorescence in situ hybridization with break-apart probes for MYB and NFIB loci revealed that 2 cases showed MYB rearrangements while one case showed loss of one MYB signal. None of the cases showed NFIB rearrangements. We contribute with 3 additional cases of PCACC exhibiting MYB aberrations, the apparent driving genetic abnormality in these tumors.

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

hi@scite.ai

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Giant Primary Cutaneous Adenoid Cystic Carcinoma of the Perineum: Histological and Radiological Correlations

Abstract: This article does not have an abstract.

Cited by 4 publications

References 11 publications

Primary cutaneous adenoid cystic carcinoma in the United States: Incidence, survival, and associated cancers, 1976 to 2005

Primary cutaneous adenoid cystic carcinoma in the United States: Incidence, survival, and associated cancers, 1976 to 2005

Quiste epidérmico vs. carcinoma adenoideo quístico

Primary cutaneous adenoid cystic carcinoma with MYB aberrations: report of three cases and comprehensive review of the literature

Contact Info

Product

Resources

About