Puchner S E, Varga R, Hobusch G M, Kasparek M, Panotopoulos J, Lang S, Windhager R, Funovics P T
Department of Orthopedics, Medical University of Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
Department of Orthopedics, Medical University of Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
Orthop Traumatol Surg Res. 2016 Nov;102(7):925-932. doi: 10.1016/j.otsr.2016.08.010. Epub 2016 Oct 10.
Adamantinoma (AD) is an ultimately rare, low-grade malignant bone tumor. In most cases it occurs in the tibia of young adults. Osteofibrous dysplasia (OFD) is a rare, benign, lesion that is typically seen in children. Histopathology, ultrastructure, and cytogenetics indicate that these lesions are closely related. Yet, etiology remains a matter of debate. Local recurrence rates are high for both entities as published in literature and long-term outcomes are scarce, due to the rarity of the disease.
AD should be treated by En-Bloc resection while ODF can be treated by curettage or by observation. Consequently, the aim of the present study was to answer following questions: Were local recurrence rates of both entities different based on a retrospective review within a tertiary referral center for orthopedic oncology?
In a retrospective cohort study, 10 patients with AD and 5 patients with OFD (including 1 patient with OFD-like-AD) were reviewed. Primary surgeries for patients with AD were: En-bloc resection in 7, curettage in 2 and amputation in 1. In the OFD group, only 2 patients underwent surgery by curettage. Mean follow-up was 16 years (range: 2-47 years). Nine patients had a minimum follow-up of 10 years (mean: 23 years; range: 10-47 years).
Four patients with AD (40%) and 2 patients with OFD (40%) - all of them following surgical removal - suffered from local recurrence. In the "En bloc" resection group of AD, there were 2 LR (29%). All patients of both groups treated with curettage showed LR. One patient with AD had metastasis at time of diagnosis and died of disease. Another patient with AD was diagnosed with metastasis 67 months after surgery and was still alive with disease at latest follow-up (77 month).
The overall prognosis of AD and OFD is good, yet local recurrence rates are high, irrespective of surgical strategy. While an internationally standardized treatment regime is still missing, a more radical surgical approach should be considered, especially when treating AD.
Retrospective study; Level IV.
骨样骨瘤（AD）是一种极其罕见的低度恶性骨肿瘤。多数情况下发生于年轻成人的胫骨。骨纤维结构不良（OFD）是一种罕见的良性病变，多见于儿童。组织病理学、超微结构及细胞遗传学表明这些病变密切相关。然而，病因仍存在争议。正如文献所报道，这两种疾病的局部复发率均较高，且由于疾病罕见，长期预后资料匮乏。
AD应采用整块切除治疗，而ODF可采用刮除术或观察处理。因此，本研究的目的是回答以下问题：基于在一家骨科肿瘤三级转诊中心进行的回顾性研究，这两种疾病的局部复发率是否存在差异？
在一项回顾性队列研究中，对10例AD患者和5例OFD患者（包括1例OFD样AD患者）进行了分析。AD患者的初次手术方式为：7例行整块切除，2例行刮除术，1例行截肢术。在OFD组中，仅2例患者接受了刮除术。平均随访时间为16年（范围：2 - 47年）。9例患者的最短随访时间为10年（平均：23年；范围：10 - 47年）。
4例AD患者（40%）和2例OFD患者（40%）——均在手术切除后——出现局部复发。在AD的“整块”切除组中，有2例局部复发（29%）。两组中所有接受刮除术治疗的患者均出现局部复发。1例AD患者在诊断时发生转移并死于疾病。另1例AD患者在术后第67个月被诊断为转移，在最近一次随访（第77个月）时仍存活且患有疾病。
AD和OFD的总体预后良好，但无论手术策略如何，局部复发率均较高。尽管仍缺乏国际标准化的治疗方案，但应考虑采用更激进的手术方法，尤其是在治疗AD时。
回顾性研究；IV级。
