Kunkel Martin, Ghalibafian Maryam, Radner Herbert, Reichert Torsten E, Fischer Berthold, Wagner Wilfried
Department of Oral and Maxillofacial Surgery, University Hospital of Mainz, Augustusplatz 2, 55101 Mainz, Germany.
Oral Oncol. 2004 Apr;40(4):444-9. doi: 10.1016/j.oraloncology.2003.09.010.
A biphasic odontogenic tumor with aggressive clinical behaviour is reported. The tumor arose posterior to the right mandibular third molar involving the angle of the mandible and the ascending ramus. Within a 5 years period the patient suffered from two episodes of local progression and final disease generalization occurred 6 years after initial surgical therapy. On histopathologic evaluation, both recurrences and the distant metastasis preserved the biphasic pattern seen in the primary tumor with both epithelial and mesenchymal components exhibiting clear cytological features of malignancy. There was no evidence of sarcomatous overgrowth within the progression of the tumor. By contrast, the proportion of the epithelial component was even enlarged in the second recurrence. Thus, both pathologic features and clinical behaviour clearly distinguishes this tumor from ameloblastic fibrosarcoma and demonstrates the clear morphological as well as clinical characteristics of a true malignant mixed type tumor with a definite sarcomatous and carcinomatous component. Thus, even though a very rare lesion, our case supports the consideration of the odontogenic carcinosarcoma as an individual entity.
报告了一例具有侵袭性临床行为的双相性牙源性肿瘤。该肿瘤发生于右下颌第三磨牙后方，累及下颌角和升支。在5年的时间里，患者经历了两次局部进展，初始手术治疗6年后出现了最终的疾病播散。组织病理学评估显示，复发灶和远处转移灶均保留了原发肿瘤的双相模式，上皮和间充质成分均表现出明显的恶性细胞学特征。在肿瘤进展过程中没有肉瘤样过度生长的证据。相比之下，第二次复发时上皮成分的比例甚至增大。因此，病理特征和临床行为均明确将该肿瘤与成釉细胞纤维肉瘤区分开来，并显示出具有明确肉瘤和癌成分的真正恶性混合性肿瘤的清晰形态学及临床特征。因此，尽管是一种非常罕见的病变，但我们的病例支持将牙源性癌肉瘤视为一个独立的实体。
