MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland

Abstract Background Oncocytic mucoepidermoid carcinoma is a rare variant of mucoepidermoid carcinoma. Only a few cases are reported involving mostly the parotid gland with only four cases reported in the submandibular gland. Mastermind-like 2 (MAML2) translocation is detected in about 66% mucoepider...

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Main Authors: Maria Kamal, Anoshia Afzal, Elizabeth Gillies
Format: Article
Language:English
Published: BMC 2022-08-01
Series:Surgical and Experimental Pathology
Subjects:
Online Access:https://doi.org/10.1186/s42047-022-00119-5
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author Maria Kamal
Anoshia Afzal
Elizabeth Gillies
author_facet Maria Kamal
Anoshia Afzal
Elizabeth Gillies
author_sort Maria Kamal
collection DOAJ
description Abstract Background Oncocytic mucoepidermoid carcinoma is a rare variant of mucoepidermoid carcinoma. Only a few cases are reported involving mostly the parotid gland with only four cases reported in the submandibular gland. Mastermind-like 2 (MAML2) translocation is detected in about 66% mucoepidermoid carcinoma and many oncocytic mucoepidermoid carcinoma. Case presentation We present a rare case of MAML2 negative oncocytic mucoepidermoid carcinoma of the submandibular gland in a 73-year-old female. CT revealed a large left submandibular gland mass. Submandibular gland resection with marginal mandibulectomy and ipsilateral neck dissection was performed. Grossly, a solid-cystic submandibular gland tumor was identified. Microscopic examination revealed an infiltrative proliferation of oncocytes. Foci of squamoid and intermediate cells with rare mucocytes were present. Two ipsilateral cervical lymph nodes and mandible were involved. Immunohistochemistry and special stains demonstrated positivity for p63, p40, CK5/6, mucicarmine and alcian blue. No MAML2 gene rearrangement was identified. Conclusion An awareness that MAML2 negative mucoepidermoid carcinoma exists will prevent misdiagnosis and incorrect treatment as many of its differential diagnoses are benign.
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spelling doaj.art-0fbea52faa214a678f0792a303a1da6a2022-12-22T02:34:49ZengBMCSurgical and Experimental Pathology2520-84542022-08-01511610.1186/s42047-022-00119-5MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular glandMaria Kamal0Anoshia Afzal1Elizabeth Gillies2Department of Pathology, University of Oklahoma Health Sciences CenterDepartment of Pathology, University of Oklahoma Health Sciences CenterDepartment of Pathology, University of Oklahoma Health Sciences CenterAbstract Background Oncocytic mucoepidermoid carcinoma is a rare variant of mucoepidermoid carcinoma. Only a few cases are reported involving mostly the parotid gland with only four cases reported in the submandibular gland. Mastermind-like 2 (MAML2) translocation is detected in about 66% mucoepidermoid carcinoma and many oncocytic mucoepidermoid carcinoma. Case presentation We present a rare case of MAML2 negative oncocytic mucoepidermoid carcinoma of the submandibular gland in a 73-year-old female. CT revealed a large left submandibular gland mass. Submandibular gland resection with marginal mandibulectomy and ipsilateral neck dissection was performed. Grossly, a solid-cystic submandibular gland tumor was identified. Microscopic examination revealed an infiltrative proliferation of oncocytes. Foci of squamoid and intermediate cells with rare mucocytes were present. Two ipsilateral cervical lymph nodes and mandible were involved. Immunohistochemistry and special stains demonstrated positivity for p63, p40, CK5/6, mucicarmine and alcian blue. No MAML2 gene rearrangement was identified. Conclusion An awareness that MAML2 negative mucoepidermoid carcinoma exists will prevent misdiagnosis and incorrect treatment as many of its differential diagnoses are benign.https://doi.org/10.1186/s42047-022-00119-5Oncocytic mucoepidermoid carcinomaSubmandibular glandMAML2
spellingShingle Maria Kamal
Anoshia Afzal
Elizabeth Gillies
MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
Surgical and Experimental Pathology
Oncocytic mucoepidermoid carcinoma
Submandibular gland
MAML2
title MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
title_full MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
title_fullStr MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
title_full_unstemmed MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
title_short MAML2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
title_sort maml2 negative oncocytic mucoepidermoid carcinoma of submandibular gland
topic Oncocytic mucoepidermoid carcinoma
Submandibular gland
MAML2
url https://doi.org/10.1186/s42047-022-00119-5
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AT anoshiaafzal maml2negativeoncocyticmucoepidermoidcarcinomaofsubmandibulargland
AT elizabethgillies maml2negativeoncocyticmucoepidermoidcarcinomaofsubmandibulargland