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Primary cutaneous adenoid cystic carcinoma with MYB aberrations: report of three cases and comprehensive review of the literature
Author(s) -
PrietoGranada Carlos N.,
Zhang Lei,
Antonescu Cristina R.,
Henneberry Jean M.,
Messina Jane L.
Publication year - 2017
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.12856
Subject(s) - myb , adenoid cystic carcinoma , pathology , adenoid , lymph node , biology , biopsy , fluorescence in situ hybridization , carcinoma , medicine , chromosome , gene , gene expression , biochemistry
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.