PDF(Pubmed)
Abstract：
BACKGROUND: Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone.
METHODS: A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up.
CONCLUSIONS: Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.
METHODS: A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up.
CONCLUSIONS: Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.
摘要：
背景：成釉细胞纤维肉瘤（AFS）是一种罕见的恶性牙源性肿瘤，常见于年轻人，通常影响下颌区域。我们报告了一名来自上颌骨的老年女性患者中异常罕见且高度不典型的AFS病例。
方法：一名66岁女性入院，有2周的左上磨牙肿块病史。CT扫描提示上颌骨有囊肿。切开活检显示梭形细胞肿瘤。MRI显示左侧上颌骨异常,表明可能的肿瘤病变。病人接受了上颌骨次全切除术,广泛的肿瘤切除，口内上皮瓣移植,和拔牙。组织学鉴定了具有可见有丝分裂图的非典型肿瘤细胞。免疫组化显示PCK和CD34表达阴性，但波形蛋白和SMA表达呈阳性。Ki-67增殖指数为30～50%。这些发现提示左上颌骨有一个潜在的恶性软组织肿瘤，倾向于AFS的诊断。患者接受术后放疗。随访6个月无复发。
结论：基于重复的病理证据，我们报告了一例罕见的老年女性AFS源自上颌骨的病例。手术和术后放疗结果良好。
方法：一名66岁女性入院，有2周的左上磨牙肿块病史。CT扫描提示上颌骨有囊肿。切开活检显示梭形细胞肿瘤。MRI显示左侧上颌骨异常,表明可能的肿瘤病变。病人接受了上颌骨次全切除术,广泛的肿瘤切除，口内上皮瓣移植,和拔牙。组织学鉴定了具有可见有丝分裂图的非典型肿瘤细胞。免疫组化显示PCK和CD34表达阴性，但波形蛋白和SMA表达呈阳性。Ki-67增殖指数为30～50%。这些发现提示左上颌骨有一个潜在的恶性软组织肿瘤，倾向于AFS的诊断。患者接受术后放疗。随访6个月无复发。
结论：基于重复的病理证据，我们报告了一例罕见的老年女性AFS源自上颌骨的病例。手术和术后放疗结果良好。
