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长骨原发性恶性纤维组织细胞瘤:长期随访

Primary malignant fibrous histiocytoma of long bones: long-term follow-up.

作者信息

Özkurt Bülent, Başarır Kerem, Yıldız Yusuf H, Kalem Mahmut, Sağlık Yener

机构信息

Department of Orthopedics and Traumatology, Ankara Numune Training and Research Hospital, 06100 Altındağ, Ankara, Turkey.

出版信息

Eklem Hastalik Cerrahisi. 2016 Aug;27(2):94-9. doi: 10.5606/ehc.2016.20.

DOI:10.5606/ehc.2016.20
PMID:27499321
Abstract

OBJECTIVES

This study aims to evaluate patients diagnosed with malignant fibrous histiocytoma and investigate the possible prognostic factors associated with duration of survival.

PATIENTS AND METHODS

The study, which was conducted between May 1994 and September 2013, included 14 patients diagnosed as malignant fibrous histiocytoma (12 males, 2 females; median age 48 years; range 17 to 64 years). We evaluated patients' demographic features, location of the pathology, histological findings, surgical margins, and treatment modalities and investigated the effects of these parameters on survival.

RESULTS

Femur was the most frequently involved bone, followed by tibia and humerus. The median follow-up duration of the patients was 129 months. We performed limb salvage surgeries in 13 patients and amputation in one patient. Surgical margins were marginal in three patients and postoperative radiotherapy was performed for local control of the disease. Although there was no local recurrence in these patients, distant metastasis developed in two patients, indicating the importance of surgical margin as a significant factor on survival. Five-year survival rate was 81.9% in patients with wide surgical margins and 33.3% in patients with marginal margins.

CONCLUSION

Surgical excision with wide margins and adjuvant chemotherapy provided adequate control of the disease and longer survival. The only prognostic factor statistically significantly associated with duration of survival was surgical margins. Neoadjuvant chemotherapy may be used when there is a suspicion of not obtaining adequately wide surgical margin perioperatively due to close association with neurovascular structures.

摘要

目的

本研究旨在评估被诊断为恶性纤维组织细胞瘤的患者,并调查与生存时间相关的可能预后因素。

患者与方法

该研究于1994年5月至2013年9月进行,纳入了14例被诊断为恶性纤维组织细胞瘤的患者(12例男性,2例女性;中位年龄48岁;范围17至64岁)。我们评估了患者的人口统计学特征、病变部位、组织学表现、手术切缘及治疗方式,并调查了这些参数对生存的影响。

结果

股骨是最常受累的骨骼,其次是胫骨和肱骨。患者的中位随访时间为129个月。我们对13例患者实施了保肢手术,1例患者进行了截肢。3例患者的手术切缘为边缘性,对其进行了术后放疗以局部控制疾病。尽管这些患者未出现局部复发,但有2例发生了远处转移,这表明手术切缘作为生存的一个重要因素的重要性。手术切缘为广泛性的患者5年生存率为81.9%,切缘为边缘性的患者5年生存率为33.3%。

结论

广泛切缘的手术切除及辅助化疗可充分控制疾病并延长生存期。与生存时间在统计学上显著相关的唯一预后因素是手术切缘。当怀疑因与神经血管结构关系密切而在围手术期无法获得足够宽的手术切缘时,可使用新辅助化疗。

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