色素沉着绒毛结节性滑膜炎影像学诊断6例报告

2000～2004年,我们收治色素沉着绒毛结节性滑膜炎（PVNS）患者6例。现将其影像学诊断体会报告如下。     

肩关节色素沉着绒毛结节性滑膜炎精确放疗1例

<正>色素沉着绒毛结节性滑膜炎(PVNS)是一种发生于黏液滑囊、关节滑膜及腱鞘的罕见良性增生性疾病,以滑膜增生、黄棕色绒毛结节突起和含铁血黄素沉着为特点〔1〕。这种增生紊乱非常罕见,年发生率为百万分之1.8〔2〕。好发于青壮年,发病无明显性别差异。PVNS最常累及单个大关节,尤其以膝关节常见,约占80%〔3〕,其次为髋关节、踝关节、手和脚等小关节,肩关节极为少见。现将1例罕见的老年PVNS病例报道如下。     

膝关节色素沉着绒毛结节性滑膜炎MRI表现特点

目的分析膝关节色素沉着绒毛结节性滑膜炎（PVNS）的MRI表现特点,提高对本病诊断的准确率。方法回顾性分析8例经手术或关节镜及病理证实的膝关节PVNS,总结其MRI表现特点。结果8例患者均为弥漫型,主要MRI表现为滑膜不规则结节状增厚或弥漫性增生,T1WI呈等/低信号,T2WI呈等/稍高信号,增生的滑膜内可见含铁血黄素沉着（T2WI低信号）、邻近半月板和（或）骨侵蚀、关节腔积液等,关节内外结构均可不同程度受侵。结论PVNS所致的滑膜绒毛结节样增生以及含铁血黄素沉着在MRI上具有特征性的表现和信号,对早期诊断本病提供重要依据。     

关节镜下滑膜全切术治疗膝关节色素沉着绒毛结节性滑膜炎疗效观察

目的观察关节镜下滑膜全切除术治疗膝关节色素沉着绒毛结节性滑膜炎（PVS）的效果。方法对19例膝关节PVS患者在关节镜下行滑膜全切除术。结果本组手术顺利,术后无并发症发生。术后随访6～21个月,行膝关节MRI检查未见滑膜炎复发。按KDC膝关节功能主观评分为（87．9±3．1）分,Lysholm膝关节评分为（86．7±1．7）分,与术前的（55．3±2．5）、（53．9±3．1）分比较,P均〈0．05。结论关节镜下对膝关节实施滑膜全切除,手术创伤小,膝关节功能恢复好。     

色素沉着绒毛结节状腱鞘滑膜炎2例报告

色素沉着绒毛结节状滑膜炎多发生于人体各关节,发生于腱鞘者少见。我们收治手指腱鞘色素沉着绒毛结节状滑膜炎患者2例,现择其1例报告如下。     

膝关节色素沉着绒毛结节性滑膜炎的诊治体会

色素沉着绒毛结节性滑膜炎是一种介于炎症和良性肿瘤之间的滑膜疾病。1999年 1月至 2 0 0 2年 12月 ,我院收治该病患者 8例 ,均经关节镜检查确诊 ,并在镜下进行手术治疗 ,疗效满意。现报告如下。资料与方法 :本组 8例中男 3例、女 5例 ,年龄 37～ 5 2岁、平均 46岁 ,病程 3～ 15年、平均 7年。左侧 3例 ,右侧 5例。均有膝关节疼痛、肿胀 ,触有海棉样感觉 ,浮髌试验阳性 ,关节穿刺抽出血性咖啡色液体 ,膝关节活动轻度受限 2例 ,有外伤史 5例。 X线检查示 ,6例膝关节腔积液 ,滑膜增厚 ,局部软组织肿胀 ,尤以髌上囊为著 ;2例围绕关节周围见软…     

原发性色素沉着结节性肾上腺皮质病

Chute等首先报道了一种导致柯兴综合征的少见原因——原发性色素沉着结节性肾上腺皮质病(primary pigmented nodular adrenocortical disease,PPNAD)。此病以双侧肾上腺皮质多发性自主分泌的色素沉着结节和结节间皮质组织萎缩为特征。其发病原因和治疗与其他类型柯兴综合征迥异。为引起临床医生注意,现将近年有关此病的研究进展综述如下。     

色素沉着绒毛结节性滑膜炎3例

例1:女性,42岁。因右膝关节肿胀1年,伴疼痛、功能受限半年于1996年3月5日入院。无外伤史。体检:右膝关节局部皮温增高但不红,髌上可触及一15cm×12cm类圆形肿块,质韧,有波动感,压痛不明显,移动性差。实验室检查无特殊。右膝关节X线照片髌上囊区可见椭圆形密度增高阴影。临床疑诊色素沉着绒毛结节性滑膜炎予硬膜外麻醉下行右膝关节镜检术及镜下滑膜切除术。术中引出100ml暗红色血性关节液。关节镜下见滑膜呈黄褐色,弥漫性增生呈绒毛状,伴充血、水肿,关节软骨及半月板未见明显破坏。滑膜病理报告为右膝关节色素沉着     

MRI features of pigmented villonodular synovitis (PVNS)

The aim of this study was to characterize the magnetic resonance imaging (MRI) features of PVNS. The radiographs and MR images of 23 pathologically proven cases of PVNS were retrospectively reviewed, with emphasis on MR images. There were 9 males and 14 females, mean age 36 years. Of 23 cases, 9 occurred in the hip, 8 in the knee, 3 in the ankle, 2 in the elbow and 1 in the wrist. Typical MRI findings included variable extent of nodular synovial proliferation, from mild proliferation to extensive masses, joint effusion in all cases, and multiple bony erosions in 15. Owing to the tight joint space, bone involvement was frequently seen in the hip, ankle, elbow and wrist. Although the knee joint had a loose capsule, bone involvement was rarely seen. Hemosiderin is a magnetic material, its deposit on proliferative synovial tissue resulting in a spotty low signal or extensive low signal area within the proliferative synovial masses on T₁- (T1WI) and T₂-weighted (T2WI) images, best seen on fast field echo (FFE) sequence MRI images. Fat-suppressed sequences obscured the deposit . This is diagnostic of PVNS. The MRI features of PVNS include variable extent of synovial proliferation, joint effusion and erosion of bone, and in particular the deposit of hemosiderin within the synovial masses. The deposit of hemosiderin, appearing as a low signal area best seen on FFE sequence, is diagnostic for PVNS.Abbreviations FFE Fast field echo - PVNS Pigmented villonodular synovitis     

Computed tomography in pigmented villonodular synovitis of the hip

Pigmented villonodular synovitis is an unusual cause of monoarticular symptoms in young adults. This report describes the ability of computed tomography to accurately demonstrate the lesions of pigmented villonodular synovitis affecting the hip joint when conventional methods are equivocal. The diagnosis is thereby facilitated allowing appropriate management, including biopsy and surgery, to be undertaken.     

Intraarticular adalimumab in a patient with pigmented villonodular synovitis

Pigmented villonodular synovitis is a chronic inflammatory disease progressing with histological villonodular hyperplasia in synovium, fibrosis and accumulation of hemosiderin. Knee joint is frequently involved leading to severe joint damage and disability in untreated cases. Treatment options include surgical intervention or radio-synovectomy with intraarticular yttrium-90. The case presented here includes significant clinical and radiological disease regression after intraarticular adalimumab in a patient diagnosed with radiologically and histologically confirmed pigmented villonodular synovitis who did not consent to surgical intervention.     

Cell populations involved in pigmented villonodular synovitis of the knee

OBJECTIVE: Pigmented villonodular synovitis (PVNS) of the knee is a tumor-like process of uncertain nature. We analyzed the involved cell populations, iron deposition, and cell proliferation in PVNS to propose a pathogenetic concept of this still elusive disease entity. METHODS: The study was performed on a series of 14 cases of localized PVNS of the knee. Histology and histochemistry were used to evaluate basic morphology and iron deposit distribution. Immunohistochemistry was performed to characterize the inflammatory cell infiltrate and to identify the proliferating cell compartments. In situ hybridization analysis using a cDNA probe against type I collagen was utilized to further characterize the mononuclear cell infiltrate. RESULTS: In addition to the classic features (mononuclear cell infiltrate, multinuclear giant cells, iron deposits, and stromal fibrosis) we observed a chronic inflammatory cell infiltrate in all PVNS samples, in which CD8 positive T cells were conspicuous. A high portion of non-phagocytotic cells resorbed iron and became CD68 positive. A proportion of mononuclear cells expressed type I collagen, thus resembling B synoviocytes. CONCLUSION: Our results suggest that preexisting chronic inflammation plays an important pathogenetic role in the PVNS disease process. Chronic inflammation increases the risk of articular bleeding and probably deranges the iron processing capacity of local synovial macrophages. The resulting iron overload could lead to a shift of iron storing cells from synovial macrophages to B synoviocytes and fibroblasts. A perpetuated proliferation and activation of these cells can explain why PVNS behaves like a neoplastic process.     

Erosive intervertebral joint lesions. A case of pigmented villonodular synovitis

Pigmented villonodular synovitis (PVNS) is a rare proliferative lesion that can affect synovial membranes, tendon sheaths, and bursae. It is usually a monarticular disease of the lower extremities, and so far fewer than 30 cases of spinal involvement have been reported in the literature. We describe a patient with progressive lumbar pain and spinal claudication, in whom a CT scan of the lumbar spine revealed destruction fo facet joints L3 to L5. An open biopsy was performed, which led to the diagnosis fo PVNS. The patient underwent successful surgical resection of the tumour mass and stabilization of segments L3 to S1. Two years after surgery the patient has no signs of recurrence. Differential diagnosis of erosive vertebral joint disease is discussed.