Background: It is generally accepted that there is a spectrum ranging from Osteofibrous Dysplasia (OFD) to Osteofibrous dysplasia-like Adamantinoma (OFD-AD) to “classic” Adamantinoma (AD). However, it is controversi...Background: It is generally accepted that there is a spectrum ranging from Osteofibrous Dysplasia (OFD) to Osteofibrous dysplasia-like Adamantinoma (OFD-AD) to “classic” Adamantinoma (AD). However, it is controversial whether OFD may progress into OFD-AD and AD or these pathologic changes reflect sampling issues. We herein report the fifth case of late development of AD from OFD-AD to underline the importance of long-term follow-up. Case: At 6 years bone biopsy of the tibia revealed the diagnosis of OFD-AD, confirmed by repeat biopsies until the age of 18 years. At age 45 years a progressive focal osteolysis was biopsied and showed histologically a classic AD. The affected tibial diaphysis was resected and pasteurized. Reconstruction was performed adapting the “Capanna technique” of inserting the ipsilateral mobilized fibula into the replanted autologous, pasteurized tibial segment as biologic stabilizer. At 1.5 years follow-up, the reconstruction has healed allowing full weight bearing.展开更多
Adamantinoma is a primary low grade malignant bone tumor that is predominantly located in the mid-portion of the tibia. The tumor is of interest for two reasons： first, there still exists considerable dispute as to t...Adamantinoma is a primary low grade malignant bone tumor that is predominantly located in the mid-portion of the tibia. The tumor is of interest for two reasons： first, there still exists considerable dispute as to the origin of the lesion and recent reports reveal that the condition is more malignant than had previously been supposed. Although cases of adamanti- noma located to the axial skeleton have been reported, this is the first case of adamantinoma located to pelvic bone in Iran. Here we present the clinical, radiological ＆ histopathological features of a 19 year-old male with painful lesion located to the right pelvic bone which was morphologically and immunohistochemically diagnosed as adamantinoma. In general, metastasis is seen in 15%-20% of patients. The spread can occur to regional nodes, lung and infrequently to skeleton, liver and brain . Several weeks after surgery, our patient＇s condition gradually worsened. A CT-scan of abdomen revealed widespread liver metastasis and the patient died due to acute liver failure. This case demonstrates that the mortality rate from adamantinoma is not always low.展开更多
目的 探讨长骨造釉细胞瘤（adamantinoma of long bone,ALB）的临床病理特征、诊断和鉴别诊断及其组织发生。方法 对5例ALB进行病理形态学观察及免疫组化分析。结果 5例发病年龄为12-48岁,平均33岁,女性3例,男性2例。上皮性和骨纤维成分...目的 探讨长骨造釉细胞瘤（adamantinoma of long bone,ALB）的临床病理特征、诊断和鉴别诊断及其组织发生。方法 对5例ALB进行病理形态学观察及免疫组化分析。结果 5例发病年龄为12-48岁,平均33岁,女性3例,男性2例。上皮性和骨纤维成分为ALB的特征性改变,两者以不同的比例和形态相互交织排列。3例上皮细胞巢较明显,巢周细胞呈栅栏状排列,2例在骨纤维结构不良样病变背景中散在少量成簇的上皮细胞巢。免疫组化：上皮细胞表达CK和34βE12,部分表达vimentin,不表达CD34;纤维间质表达vimentin。结论 ALB是以形态多样为特征的低级别双向分化的恶性肿瘤,诊断和鉴别诊断需结合肿瘤的组织形态和免疫组化特点。展开更多
文摘Background: It is generally accepted that there is a spectrum ranging from Osteofibrous Dysplasia (OFD) to Osteofibrous dysplasia-like Adamantinoma (OFD-AD) to “classic” Adamantinoma (AD). However, it is controversial whether OFD may progress into OFD-AD and AD or these pathologic changes reflect sampling issues. We herein report the fifth case of late development of AD from OFD-AD to underline the importance of long-term follow-up. Case: At 6 years bone biopsy of the tibia revealed the diagnosis of OFD-AD, confirmed by repeat biopsies until the age of 18 years. At age 45 years a progressive focal osteolysis was biopsied and showed histologically a classic AD. The affected tibial diaphysis was resected and pasteurized. Reconstruction was performed adapting the “Capanna technique” of inserting the ipsilateral mobilized fibula into the replanted autologous, pasteurized tibial segment as biologic stabilizer. At 1.5 years follow-up, the reconstruction has healed allowing full weight bearing.
文摘Adamantinoma is a primary low grade malignant bone tumor that is predominantly located in the mid-portion of the tibia. The tumor is of interest for two reasons： first, there still exists considerable dispute as to the origin of the lesion and recent reports reveal that the condition is more malignant than had previously been supposed. Although cases of adamanti- noma located to the axial skeleton have been reported, this is the first case of adamantinoma located to pelvic bone in Iran. Here we present the clinical, radiological ＆ histopathological features of a 19 year-old male with painful lesion located to the right pelvic bone which was morphologically and immunohistochemically diagnosed as adamantinoma. In general, metastasis is seen in 15%-20% of patients. The spread can occur to regional nodes, lung and infrequently to skeleton, liver and brain . Several weeks after surgery, our patient＇s condition gradually worsened. A CT-scan of abdomen revealed widespread liver metastasis and the patient died due to acute liver failure. This case demonstrates that the mortality rate from adamantinoma is not always low.
文摘目的 探讨长骨造釉细胞瘤（adamantinoma of long bone,ALB）的临床病理特征、诊断和鉴别诊断及其组织发生。方法 对5例ALB进行病理形态学观察及免疫组化分析。结果 5例发病年龄为12-48岁,平均33岁,女性3例,男性2例。上皮性和骨纤维成分为ALB的特征性改变,两者以不同的比例和形态相互交织排列。3例上皮细胞巢较明显,巢周细胞呈栅栏状排列,2例在骨纤维结构不良样病变背景中散在少量成簇的上皮细胞巢。免疫组化：上皮细胞表达CK和34βE12,部分表达vimentin,不表达CD34;纤维间质表达vimentin。结论 ALB是以形态多样为特征的低级别双向分化的恶性肿瘤,诊断和鉴别诊断需结合肿瘤的组织形态和免疫组化特点。
