Departments of Orthopedic Surgery (E.M.S., P.D.S.D., and M.A.J.v.d.S.), Histopathology (J.V.M.G.B.), and Pathology (P.C.W.H.), Leiden University Medical Center, Leiden, the Netherlands.
London Sarcoma Service, Royal National Orthopaedic Hospital, Stanmore, United Kingdom.
J Bone Joint Surg Am. 2020 Oct 7;102(19):1703-1713. doi: 10.2106/JBJS.19.01056.
Osteofibrous dysplasia-like adamantinoma (OFD-AD) and classic adamantinoma (AD) are rare, neoplastic diseases with only limited data supporting current treatment protocols. We believe that our retrospective multicenter cohort study is the largest analysis of patients with adamantinoma to date. The primary purpose of this study was to describe the disease characteristics and evaluate the oncological outcomes. The secondary purpose was to identify risk factors for local recurrence after surgical treatment and propose treatment guidelines.
Three hundred and eighteen confirmed cases of OFD-AD and AD for which primary treatment was carried out between 1985 and 2015 were submitted by 22 tertiary bone tumor centers. Proposed clinical risk factors for local recurrence such as size, type, and margins were analyzed using univariable and multivariate Cox regression analysis.
Of the 318 cases, 128 were OFD-AD and 190 were AD. The mean age at diagnosis was 17 years (median, 14.5 years) for OFD-AD and 32 years (median, 28 years) for AD; 53% of the patients were female. The mean tumor size in the OFD-AD and AD groups combined was 7.8 cm, measured histologically. Sixteen percent of the patients sustained a pathological fracture prior to treatment. Local recurrence was recorded in 22% of the OFD-AD cases and 24% of the AD cases. None of the recurrences in the OFD-AD group progressed to AD. Metastatic disease was found in 18% of the AD cases and fatal disease, in 11% of the AD cases. No metastatic or fatal disease was reported in the OFD-AD group. Multivariate Cox regression analysis demonstrated that uncontaminated resection margins (hazard ratio [HR] = 0.164, 95% confidence interval [CI] = 0.092 to 0.290, p < 0.001), pathological fracture (HR = 1.968, 95% CI = 1.076 to 3.600, p = 0.028), and sex (female versus male: HR = 0.535, 95% CI = 0.300 to 0.952, p = 0.033) impacted the risk of local recurrence.
OFD-AD and AD are parts of a disease spectrum but should be regarded as different entities. Our results support reclassification of OFD-AD into the intermediate locally aggressive category, based on the local recurrence rate of 22% and absence of metastases. In our study, metastatic disease was restricted to the AD group (an 18% rate). We advocate wide resection with uncontaminated margins including bone and involved periosteum for both OFD-AD and AD.
Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.
骨纤维结构不良样骨化性纤维瘤（OFD-AD）和经典骨化性纤维瘤（AD）是罕见的肿瘤性疾病，目前仅有限的数据支持当前的治疗方案。我们认为，我们的回顾性多中心队列研究是迄今为止对骨化性纤维瘤患者进行的最大分析。本研究的主要目的是描述疾病特征并评估肿瘤学结果。次要目的是确定手术治疗后局部复发的风险因素，并提出治疗指南。
22 个三级骨肿瘤中心提交了 1985 年至 2015 年间进行的原发性治疗的 318 例确诊的 OFD-AD 和 AD 病例。使用单变量和多变量 Cox 回归分析，分析了局部复发的临床风险因素，如大小、类型和切缘。
318 例患者中，128 例为 OFD-AD，190 例为 AD。OFD-AD 的诊断年龄平均为 17 岁（中位数 14.5 岁），AD 为 32 岁（中位数 28 岁）；53%的患者为女性。OFD-AD 和 AD 组联合的肿瘤平均大小为 7.8cm，组织学测量。16%的患者在治疗前发生病理性骨折。OFD-AD 组中有 22%的病例发生局部复发，AD 组中有 24%的病例发生局部复发。OFD-AD 组无一例复发进展为 AD。AD 组中有 18%的病例发生远处转移，11%的病例发生致命性疾病。OFD-AD 组未报告转移或致命性疾病。多变量 Cox 回归分析表明，无污染切缘（危险比[HR] = 0.164，95%置信区间[CI] = 0.092 至 0.290，p < 0.001）、病理性骨折（HR = 1.968，95%CI = 1.076 至 3.600，p = 0.028）和性别（女性与男性：HR = 0.535，95%CI = 0.300 至 0.952，p = 0.033）影响局部复发的风险。
OFD-AD 和 AD 是疾病谱的一部分，但应视为不同的实体。我们的结果支持根据局部复发率为 22%且无转移的情况，将 OFD-AD 重新分类为中间局部侵袭性类别。在我们的研究中，转移疾病仅限于 AD 组（占 18%）。我们主张广泛切除，包括无污染的骨和受累的骨膜，适用于 OFD-AD 和 AD。
预后 IV 级。有关完整的证据水平描述，请参见作者说明。
