Takeda Y, Kaneko R, Suzuki A
Virchows Arch A Pathol Anat Histopathol. 1984;404(3):253-63. doi: 10.1007/BF00694891.
This report presents a fatal case of ameloblastic fibrosarcoma arising from an ameloblastic fibroma, originating in the maxilla of 19-year-old Japanese male. An analysis of previously reported fatal cases of ameloblastic fibrosarcoma is included. In the course of the disease, the mesenchymal component of ameloblastic fibroma showed a dramatic histopathological transformation into sarcoma following multiple recurrence and the patient died of uncontrollable local infiltration of the cranial base. Although many cases have seemed to show disappearance of the epithelial component as malignant transformation progressed, many benign appearing ameloblastoid epithelial masses were scattered throughout the sarcomatous area even in the fatal stage in the present case. No distant metastases were found at autopsy. During multiple recurrences of the lesion, a little dysplastic dentin which was closely associated with both epithelial and mesenchymal components was found, though it could not be observed in autopsy material. Ultrastructural findings in autopsy material showed that the mesenchymal component consisted of undifferentiated mesenchymal cells, fibroblastic and fibrocytic cells with marked cellular and nuclear pleomorphism and that the epithelial component closely resembled the enamel organ.
本报告介绍了一例起源于成釉细胞纤维瘤的成釉细胞纤维肉瘤致死病例，该病例发生于一名19岁日本男性的上颌骨。报告还纳入了对先前报道的成釉细胞纤维肉瘤致死病例的分析。在疾病过程中，成釉细胞纤维瘤的间充质成分在多次复发后发生了显著的组织病理学转变，恶变为肉瘤，患者最终死于颅底无法控制的局部浸润。尽管随着恶性转化的进展，许多病例似乎显示上皮成分消失，但在本病例的致死阶段，即使在肉瘤区域也散在分布着许多看似良性的成釉器样上皮团块。尸检未发现远处转移。在病变多次复发期间，发现了少量与上皮和间充质成分均密切相关的发育异常牙本质，不过在尸检材料中未观察到。尸检材料的超微结构发现显示，间充质成分由未分化的间充质细胞、具有明显细胞和核多形性的成纤维细胞和纤维细胞组成，上皮成分与成釉器极为相似。
