Duan Yanfang, Qian Jing, Chen Kun, Zhang Zhuo
Dalian Medical University Department of Oncology Radiotherapy, the Second Hospital of Dalian Medical University, Dalian, Liaoning, China.
Medicine (Baltimore). 2018 Jan;97(3):e9637. doi: 10.1097/MD.0000000000009637.
Pigmented villonodular synovitis (PVNS) is an idiopathic, proliferative disorder lesion of synovial tissue, which is regarded as a benign disease, but has a local invasion. Up to now, these are no consensus about the etiology and pathogenesis of PVNS. Because of the lack of typical clinical features, misdiagnosis and delayed diagnosis are not uncommon, magnetic resonance imaging (MRI) can assist diagnosis and histopathological examination is recognized as the gold standard for the final diagnosis. Because this disease is so rare, there is no standard treatment. Surgical resection of the lesion is considered the preferred treatment, but postoperative recurrence is a problem that cannot be ignored. Postoperative radiotherapy is necessary, especially for patients with diffuse PVNS of the knee.
A 27-year-old female teacher presented with 3 years chronic pain of the right knee, and progressive swelling aggravated for 1 week. The range of motion of the knee was limited.
Clinical and laboratory examination failed to provide definitive diagnosis. Imaging can assist in diagnosis, and pathology is the gold standard. Erythrocyte sedimentation rate (ESR), antihemolytic streptococcus O (ASO), and rheumatoid factors (RF) were all negative. Joint puncture revealed giant cell tumor of the synovial membrane. PVNS was confirmed by postoperative pathology. The characteristic T2 weighted low signal of MRI suggests the recurrence of PVNS.
The patient underwent 2 stages of treatment: open synovectomy was performed in the first place and postoperative external radiotherapy was not considered. After 2 years of disease-free remission, she was diagnosed with a recurrence of the disease by MRI. Further, arthroscopic total synovectomy of the right knee was performed and external beam radiotherapy was carried out after the operation.
Up to now, the patient was followed up for 3 years without any sign of recurrence.
Adjuvant postoperative radiotherapy can improve the local control rate, it is a reliable treatment method for diffused PVNS.
色素沉着绒毛结节性滑膜炎(PVNS)是一种特发性滑膜组织增生性病变,虽被视为良性疾病,但具有局部侵袭性。目前,关于PVNS的病因和发病机制尚无共识。由于缺乏典型临床特征,误诊和延迟诊断并不少见,磁共振成像(MRI)有助于诊断,组织病理学检查被认为是最终诊断的金标准。由于这种疾病非常罕见,没有标准的治疗方法。手术切除病变被认为是首选治疗方法,但术后复发是一个不可忽视的问题。术后放疗是必要的,尤其是对于膝关节弥漫性PVNS患者。
一名27岁的女教师右膝关节慢性疼痛3年,渐进性肿胀加重1周。膝关节活动范围受限。
临床和实验室检查未能提供明确诊断。影像学可辅助诊断,病理是金标准。红细胞沉降率(ESR)、抗溶血性链球菌O(ASO)和类风湿因子(RF)均为阴性。关节穿刺显示滑膜巨细胞瘤。术后病理证实为PVNS。MRI特征性的T2加权低信号提示PVNS复发。
患者接受了两阶段治疗:首先进行了开放性滑膜切除术,未考虑术后外照射放疗。在无病缓解2年后,MRI诊断为疾病复发。进一步行右膝关节镜下全滑膜切除术,术后进行外照射放疗。
截至目前,患者随访3年无任何复发迹象。
术后辅助放疗可提高局部控制率,是弥漫性PVNS可靠的治疗方法。