Surgical Outcome and Oncological Survival of Osteofibrous Dysplasia-Like and Classic Adamantinomas: An International Multicenter Study of 318 Cases.,The Journal of Bone & Joint Surgery

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Surgical Outcome and Oncological Survival of Osteofibrous Dysplasia-Like and Classic Adamantinomas: An International Multicenter Study of 318 Cases.
The Journal of Bone & Joint Surgery ( IF 5.3 ) Pub Date : 2020-10-07 , DOI: 10.2106/jbjs.19.01056
E M Schutgens _{1,

2} , P Picci ₃ , D Baumhoer ₄ , R Pollock ₂ , J V M G Bovée ₁ , P C W Hogendoorn ₁ , P D S Dijkstra ₁ , A J Rueten-Budde ₅ , P C Jutte ₆ , F Traub ₇ , A Leithner ₈ , P-U Tunn ₉ , P Funovics ₁₀ , G Sys ₁₁ , M San-Julian ₁₂ , G R Schaap ₁₃ , H R Dürr ₁₄ , J Hardes ₁₅ , J Healey ₁₆ , R Capanna ₁₇ , D Biau ₁₈ , A Gomez-Brouchet ₁₉ , J Wunder ₂₀ , T D A Cosker ₂₁ , M K Laitinen ₂₂ , X Niu ₂₃ , V Kostiuk ₂₄ , M A J van de Sande ₁ ,

Affiliation

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.
Medical Oncology, Musculoskeletal Oncology Department, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.
Bone Tumour Reference Centre, Institute of Pathology, University Hospital and University of Basel, Basel, Switzerland.
Mathematical Institute, Leiden University, Leiden, the Netherlands.
Department of Orthopedics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Orthopedic Surgery, University of Tübingen, Tübingen, Germany.
Department of Orthopedics and Trauma, Medical University of Graz, Graz, Austria.
Orthopedic Surgery, Helios-Clinics, Berlin, Germany.
Orthopedic Surgery, Medical University of Vienna, Vienna, Austria.
Orthopedic Surgery, Ghent University Hospital, Ghent, Belgium.
Orthopedic Surgery, University of Navarra, Pamplona, Spain.
Orthopedic Surgery, Academic Medical Center, Amsterdam, the Netherlands.
Department of Tumor Orthopedics and Sarcoma Surgery, University Hospital Essen, Essen, Germany.
Musculoskeletal Oncology, Department of Orthopedic Surgery, Physical Medicine and Rehabilitation, University Hospital, LMU Munich, Munich, Germany.
Orthopedic Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.
Department of Orthopaedics, S. Chiara University Hospital, University of Pisa, Italy.
Orthopedic Surgery, Cochin Hospital, Paris, France.
Department of Histopathology, University Medical Center, Toulouse, France.
University Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, Ontario, Canada.
Orthopedic Surgery, Nuffield Orthopedic Center, Oxford, United Kingdom.
Orthopedic Surgery, Helsinki University Hospital, Helsinki, Finland.
Department of Orthopedic Oncology, Beijing Jishuitan Hospital, Beijing, People's Republic of China.
Orthopedic Surgery, National Cancer Institute Ukraine, Kiev, Ukraine.

Background:

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.

Methods:

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.

Results:

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.

Conclusions:

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.

Level of Evidence:

Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

中文翻译：

骨纤维发育不良样和经典精金瘤的手术结果和肿瘤生存：一项 318 例国际多中心研究。

背景：

骨纤维发育不良样金刚质瘤 (OFD-AD) 和经典金刚质瘤 (AD) 是罕见的肿瘤性疾病，支持当前治疗方案的数据有限。我们相信，我们的回顾性多中心队列研究是迄今为止对金刚质瘤患者最大规模的分析。本研究的主要目的是描述疾病特征并评估肿瘤学结果。第二个目的是确定手术治疗后局部复发的危险因素并提出治疗指南。

方法：

1985年至2015年间，22个三级骨肿瘤中心提交了318例确诊的OFD-AD和进行初级治疗的AD病例。使用单变量和多变量 Cox 回归分析来分析局部复发的拟议临床危险因素，例如大小、类型和边缘。

结果：

在 318 例病例中，128 例为 OFD-AD，190 例为 AD。OFD-AD 的平均诊断年龄为 17 岁（中位数为 14.5 岁），AD 的平均诊断年龄为 32 岁（中位数为 28 岁）；53%的患者是女性。组织学测量显示，OFD-AD 组和 AD 组的平均肿瘤大小合计为 7.8 厘米。百分之十六的患者在治疗前患有病理性骨折。22% 的 OFD-AD 病例和 24% 的 AD 病例出现局部复发。OFD-AD 组中没有任何复发进展为 AD。18% 的 AD 病例发现转移性疾病，11% 的 AD 病例发现致命性疾病。OFD-AD 组未报告转移性或致命性疾病。多变量 Cox 回归分析表明，未污染的切除边缘（风险比 [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。

结论：

OFD-AD 和 AD 是疾病谱的一部分，但应被视为不同的实体。基于 22% 的局部复发率和无转移，我们的结果支持将 OFD-AD 重新分类为中等局部侵袭性类别。在我们的研究中，转移性疾病仅限于 AD 组（发生率 18%）。对于 OFD-AD 和 AD，我们主张广泛切除未污染的边缘，包括骨骼和受累骨膜。