The clinical and molecular characteristics of osteofibrous dysplasia (OFD)-like adamantinoma (AD) differ from those of classic AD. Most reports about OFD-like AD are case reports or small case series. More cases from different centers are still warranted. The orthopedic oncology database of our institution was searched to identify patients with AD. The cases of OFD-like and classic AD of the long bones were retrospectively analyzed. Between December 1999 and August 2016, 23 patients were treated for AD, comprising seven with OFD-like AD and 16 with classic AD. The outcomes were compared between AD subtypes. In the OFD-like AD group, four lesions were treated with extensive curettage, while three were treated with wide resection. The median follow-up duration in the OFD-like AD group was 66 months (range 43–131 months). At the end of follow-up, there was only one case of local recurrence (LR) in the OFD-like AD group, giving a LR rate of 14.3% (1/7). No distant metastasis or progression to classic AD was detected in the OFD-like AD group. In the classic AD group, the treatments were below-the-knee amputation in one patient with extensive tibial and fibular lesions, curettage with a bone graft in one patient who was diagnosed with OFD based on a core needle biopsy, hemi-cortical excision and reconstruction in two patients, and segmental resection and reconstruction in 12 patients. At the end of follow-up, there were three cases of LR in the classic AD group, giving a LR rate of 18.8% (3/16); two patients developed lung metastasis after LR and died of the disease at 88 and 126 months after the first surgery in our hospital, respectively. The classic AD group had a metastatic rate of 12.5% (2/16), a final limb salvage rate of 75%, and estimated 5- and 10-year survival rates of 88.9% and 77.1%, respectively. OFD-like AD has a better outcome than classic AD. For OFD-like AD, extensive curettage is suggested if the tumor extent allows. For classic AD, aggressive resection with wide margins is essential to achieve local control. A long-term follow-up is necessary due to the possibility of late complications.

中文翻译：

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骨纤维异常增生型与经典长骨金刚瘤相比的结果：单机构经验。

骨纤维异常增生（OFD）样金刚烷瘤（AD）的临床和分子特征不同于经典AD。关于类似于OFD的AD的大多数报告都是案例报告或小案例系列。仍需要更多来自不同中心的案件。搜索我们机构的骨科肿瘤数据库以识别患有AD的患者。回顾性分析长骨OFD样和经典AD病例。在1999年12月至2016年8月之间，有23位患者接受了AD治疗，包括7位OFD样AD和16位经典AD。比较AD亚型的结局。在OFD样AD组中，有4处病灶接受了广泛的刮除术，有3处病灶经过了广泛的切除术。OFD样AD组的中位随访时间为66个月（范围43-131个月）。在跟进结束时，类似OFD的AD组中只有1例局部复发（LR），其LR率为14.3％（1/7）。在类似OFD的AD组中未检测到远处转移或进展为经典AD。在经典的AD组中，治疗方法是：对一名胫骨和腓骨广泛病变的患者行膝下截肢术，对一名根据核心穿刺活检，半皮质切除术和诊断为OFD的患者行植骨刮除术。 2例患者进行了重建，而12例患者进行了节段切除和重建。随访结束时，经典AD组中有3例LR，LR率为18.8％（3/16）。两名患者在LR术后发生肺转移，分别在我院首次手术后88和126个月死于该病。经典的AD组的转移率为12。5％（2/16），最终肢体抢救率为75％，估计的5年和10年生存率分别为88.9％和77.1％。类似于OFD的AD的疗效要优于经典AD。对于OFD样AD，如果肿瘤范围允许，建议广泛刮除。对于经典的AD，进行宽范围的积极切除对于实现局部控制至关重要。由于可能会出现后期并发症，因此需要长期随访。