Ectomesenchymal chondromyxoid tumor: a comprehensive updated review of the literature and case report.
10.1038/s41368-017-0003-9
- Author:
Astrid TRUSCHNEGG
1
;
Stephan ACHAM
2
;
Lumnije KQIKU
3
;
Norbert JAKSE
2
;
Alfred BEHAM
4
Author Information
1. Department of Dental Medicine and Oral Health, Division of Oral Surgery and Orthodontics, Medical University Graz, Billrothgasse 4, 8010, Graz, Austria. astrid.truschnegg@medunigraz.at.
2. Department of Dental Medicine and Oral Health, Division of Oral Surgery and Orthodontics, Medical University Graz, Billrothgasse 4, 8010, Graz, Austria.
3. Department of Dental Medicine and Oral Health, Division of Preventive an Operative Dentistry, Endodontics, Periodontology, Prosthodontics, Restorative Dentistry and Implantology, Medical University Graz, Billrothgasse 4, 8010, Graz, Austria.
4. IMAH, Institute of Morphological Analytics and Human Genetics, Grabenstraße 23, 8010, Graz, Austria.
- Publication Type:Case Reports
- MeSH:
Biomarkers, Tumor;
analysis;
Chondroma;
pathology;
surgery;
Diagnosis, Differential;
Female;
Humans;
Immunophenotyping;
Mesenchymoma;
pathology;
surgery;
Middle Aged;
Myoepithelioma;
pathology;
surgery;
Palatal Neoplasms;
pathology;
surgery
- From:
International Journal of Oral Science
2018;10(1):4-4
- CountryChina
- Language:English
-
Abstract:
Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.
