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J Cutan Pathol. 2017 Feb;44(2):201-209. doi: 10.1111/cup.12856. Epub 2016 Dec 2.

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

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Department of Pathology, University of Alabama at Birmingham (UAB), Birmingham, AL, USA.
Department of Pathology, Memorial Sloan Kettering Cancer Center (MSKCC), New York, NY, USA.
Department of Pathology, Baystate Medical Center, Springfield, MA, USA.
Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.


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 three additional cases of PCACC harboring MYB aberrations. The tumors presented in three males aged 43, 81 and 55 years old and affected the extremities in the first two 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 two cases showed MYB rearrangements while one case showed loss of one MYB signal. None of the cases showed NFIB rearrangements. We contribute with three additional cases of PCACC exhibiting MYB aberrations, the apparent driving genetic abnormality in these tumors.


MYB; NFIB; fluorescence in situ hybridization; primary cutaneous adenoid cystic carcinoma

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