Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations

Clear cell odontogenic carcinoma (CCOC) is a low-grade malignant neoplasm that affects the jaws. We report an 18 cm massive case of mandibular CCOC in a 43-year-old female. The tumor was composed of nests and cords of round to polygonal monomorphic clear cells separated by prominent stromal hyaliniz...

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Main Authors: Adepitan A. Owosho, Chukwubuzor Okwuosa, Donald I. Obi, Robinson O. Okiti, Kurt F. Summersgill
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Dentistry
Online Access:http://dx.doi.org/10.1155/2021/5558019
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author Adepitan A. Owosho
Chukwubuzor Okwuosa
Donald I. Obi
Robinson O. Okiti
Kurt F. Summersgill
author_facet Adepitan A. Owosho
Chukwubuzor Okwuosa
Donald I. Obi
Robinson O. Okiti
Kurt F. Summersgill
author_sort Adepitan A. Owosho
collection DOAJ
description Clear cell odontogenic carcinoma (CCOC) is a low-grade malignant neoplasm that affects the jaws. We report an 18 cm massive case of mandibular CCOC in a 43-year-old female. The tumor was composed of nests and cords of round to polygonal monomorphic clear cells separated by prominent stromal hyalinization. Immunohistochemically, the tumor cells showed focal cytokeratin 5/6 positivity and intracytoplasmic PAS-positive granules and were negative for S100 and after diastase treatment (PAS-D). Molecularly, this case was positive for EWSR1 rearrangement by FISH. The following should be included in the histopathological differential diagnosis: hyalinizing clear cell carcinoma of the salivary gland, clear cell variant of central mucoepidermoid carcinoma, clear cell variant of calcifying epithelial odontogenic tumor, and metastatic renal cell carcinoma. CCOC is a rare entity, with only 79 cases reported in the mandible. This case highlights the propensity for CCOC to exhibit invasiveness, destructive nature, and facial disfigurement if left untreated.
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publisher Wiley
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spelling doaj-art-6d5c2316360e4fd5ab9d09ef092268b62025-08-20T02:06:44ZengWileyCase Reports in Dentistry2090-64472090-64552021-01-01202110.1155/2021/55580195558019Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic ConsiderationsAdepitan A. Owosho0Chukwubuzor Okwuosa1Donald I. Obi2Robinson O. Okiti3Kurt F. Summersgill4Missouri School of Dentistry and Oral Health, A.T. Still University, Kirksville, Missouri, USAUniversity of Nigeria Teaching Hospital, Nsukka, Enugu, NigeriaUniversity of Nigeria Teaching Hospital, Nsukka, Enugu, NigeriaUniversity College Hospital, Ibadan, Oyo, NigeriaDepartment of Diagnostic Sciences, School of Dental Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USAClear cell odontogenic carcinoma (CCOC) is a low-grade malignant neoplasm that affects the jaws. We report an 18 cm massive case of mandibular CCOC in a 43-year-old female. The tumor was composed of nests and cords of round to polygonal monomorphic clear cells separated by prominent stromal hyalinization. Immunohistochemically, the tumor cells showed focal cytokeratin 5/6 positivity and intracytoplasmic PAS-positive granules and were negative for S100 and after diastase treatment (PAS-D). Molecularly, this case was positive for EWSR1 rearrangement by FISH. The following should be included in the histopathological differential diagnosis: hyalinizing clear cell carcinoma of the salivary gland, clear cell variant of central mucoepidermoid carcinoma, clear cell variant of calcifying epithelial odontogenic tumor, and metastatic renal cell carcinoma. CCOC is a rare entity, with only 79 cases reported in the mandible. This case highlights the propensity for CCOC to exhibit invasiveness, destructive nature, and facial disfigurement if left untreated.http://dx.doi.org/10.1155/2021/5558019
spellingShingle Adepitan A. Owosho
Chukwubuzor Okwuosa
Donald I. Obi
Robinson O. Okiti
Kurt F. Summersgill
Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations
Case Reports in Dentistry
title Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations
title_full Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations
title_fullStr Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations
title_full_unstemmed Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations
title_short Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations
title_sort clear cell odontogenic carcinoma of the mandible harboring ewsr1 rearrangement report of a massive jaw tumor and review of diagnostic considerations
url http://dx.doi.org/10.1155/2021/5558019
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