Pigmented villonodular synovitis of the hip presenting as a retroperitoneal mass

We present an unusual case of diffuse pigmented villonodular synovitis (PVNS) of the hip presenting as a large retroperitoneal mass in an asymptomatic 39-year-old man. Initial imaging characteristics and findings on core needle biopsy suggested soft tissue sarcoma. However, incisional biopsy showed findings of PVNS. The patient underwent radical synovectomy and total joint replacement, with no change in the large retroperitoneal component after 15 months of follow-up. Received: 15 June 2000 Revision requested: 8 September 2000 Revision received: 14 March 2001 Accepted: 15 March 2001     

髋关节色素沉着绒毛结节性滑膜炎的MRI诊断

目的：探讨髋关节色素沉着绒毛结节性滑膜炎的MRI表现。方法：对8例髋关节PVNS患者行常规MRI检查,结合术后病理结果进行对照分析。结果：8例MRI均显示滑膜不同程度肿胀、增厚以及关节间隙软组织肿胀,增生的滑膜组织于T1WI上呈等信号～低信号,T2WI上呈低信号夹杂混杂信号。结论：PVNS的MRI表现具有一定的特征性,MRI可显示病变的范围和程度,能准确反映关节内外受侵情况,对本病的临床诊治具有较高价值。     

Imaging of pigmented villonodular synovitis

Pigmented villonodular synovitis (PVNS) is a rare, benign, idiopathic proliferative disorder of the synovium that results in villous and/or nodular formation in joints, tendon sheaths, and bursae. The disease can be localized or diffuse. Patients with this condition typically present with symptoms of mild discomfort and associated stiffness of the involved joint; however, the spectrum of presentations is broad. Diagnosis of PVNS can be clinically difficult, and plain radiographs are usually nonspecific. Magnetic resonance (MR) imaging is a highly diagnostic modality in characterizing PVNS when it contains hemosiderin deposits exhibiting low signal intensity on all MR image pulse sequences. This article discusses the presentation, pathology, differential diagnosis, diagnostic modalities as well as various treatment options of PVNS.     

True bursal pigmented villonodular synovitis

We describe two cases of pigmented villonodular synovitis affecting true bursae. This study was also designed to discuss the term "pigmented villonodular bursitis", not confined to true synovial bursae, sometimes creating misunderstanding.     

Malignancy in pigmented villonodular synovitis

Malignant pigmented villonodular synovitis is an extremely rare and controversial disease. We describe malignant change in pigmented villonodular synovitis of the ankle in a patient with an unusually long clinical history. Symptoms began at age 21, metastatic disease developed at age 85, and the patient died 1 year later. The histologic appearance of the malignant tumor differed from that in most reported cases, in that spindle-shaped cells predominated. Lymph node metastasis also developed, a feature uncommon to soft tissue sarcomas.     

色素沉着绒毛结节性滑膜炎的MRI诊断

目的:探讨色素沉着绒毛结节性滑膜炎(PVNS)的MRI表现,提高本病的诊断水平。方法:回顾性分析5例经手术及病理证实的PVNS的MRI资料。结果:5例病变关节均见不同程度的滑膜增生,增生滑膜T1WI呈中等或稍低信号,T2WI为稍高或中等信号,其中可见散在含铁血黄素沉着结节状影,T1WI及T2WI均为低信号。5例病变关节均可见关节积液。2例病变关节有关节软骨及骨质破坏。结论:PVNS在MRI上有典型特征性的表现,可以准确显示其病变范围,做出定性诊断。     

色素沉着绒毛结节性滑膜炎的MRI与病理对照分析

目的　研究色素沉着绒毛结节性滑膜炎 (PVNS)MRI表现的病理基础及其在扩散加权像 (DWI)上异常信号改变的机制。方法　分析 7例PVNS的MRI表现 ,计算DWI上异常信号灶的表观扩散系数 (ADC) ,并与手术病理结果进行对照分析。结果　 7例PVNS中弥漫型 5例 (膝关节4例 ,肩关节 1例 )。MRI主要表现为关节滑膜广泛增生 ,呈长T1 、长T2 信号 ,其内见含铁血黄素沉着 ,呈长T1 、短T2 信号 ;4例见膝关节腔积液 ,呈长T1 、长T2 信号 ;半月板破坏 3例 ,前、后交叉韧带破坏 2例 ,呈结节状长T1 、长T2 信号 ;4例见关节内软骨和邻近骨质明显破坏 (膝关节 3例 ,肩关节 1例 ) ,1例股骨骨质呈小囊状破坏。局限型 2例 (膝关节、髋关节各 1例 ) ,主要表现为滑膜结节状增生 ,其内亦可见含铁血黄素沉着 ,呈长T1 、短T2 信号。 1例见关节腔内少许积液 ,邻近股骨骨质小囊状破坏。DWI上关节积液、滑膜增生及邻近骨质破坏表现为异常高信号 ,其平均ADC值分别为 2 .750× 1 0 - 3mm2 /s,0 .52 2× 1 0 - 3mm2 /s,0 .559× 1 0 - 3mm2 /s。结论　滑膜绒毛结节状增生及其内点状或结节状含铁血黄素沉着是PVNS的典型表现 ,在MRI上具有一定的特征性 ;DWI有助于区分PVNS病变的不同组织成分 ;病变组织的ADC值下降 ,其对鉴别诊断的意义有待进一     

A systematic literature review of synovial chondromatosis and pigmented villonodular synovitis of the hip

Objectives: Benign synovial diseases of the hip including Synovial Chondromatosis (SC) and Pigmented Villonodular Synovitis (PVNS) are devastating diseases. Initially, patients present with hip pain unrelieved by conservative measures. The diagnosis of PVNS and SC are often delayed, leading to progression of joint damage.

The purpose of this review is to present the latest on the diagnosis, management, and prognosis of SC and PVNS of the hip.

Methods: An extensive systematic search of MEDLINE and PUBMED Databases was performed. Data parameters were set from 2005 to present day with set inclusion criteria. Systematic reviews were excluded.

Results: 427 abstracts were identified, with 12 articles meeting all inclusion criteria. Eight studies focused on SC, and 5 on PVNS. 233 patients with SC of the hip and 98 patients with PVNS of the hip were identified, a total of 331 patients.

Discussion: Benign Synovial disorders of the hip are rare. In patients with chronic hip pain secondary to benign synovial disorders, early diagnosis and surgical intervention demonstrate good outcomes, and patients may benefit due to prevention of morbidity from further joint destruction. There is no clear consensus between higher successes through open versus arthroscopic surgical debridement. In the final phase of benign synovial disorders of the hip, THA of different types based on the patient’s age should be considered.     

色素沉着绒毛结节性滑膜炎的影像学诊断

目的:探讨色素沉着绒毛结节性滑膜炎(PVNS)的影像学表现。方法:回顾性分析14例经手术及病理证实的PVNS患者的X线平片、CT、MRI等影像学资料。结果:14例X线检查者均见关节囊肿胀,其中8例骨质受侵蚀,1例可见软组织肿块。8例CT检查均示关节囊肿胀,其中6例可见关节腔积液及周围骨质受侵蚀,2例见软组织肿块影,2例增强扫描者均可见强化的滑膜结节和增厚的滑膜。10例MR检查者弥漫型8例,局限型2例,均可见到关节囊肿胀及数量不等的增生滑膜绒毛结节,其中9例在增生滑膜内可见含铁血黄素沉着,呈长T1、短T2低信号,8例关节腔积液及骨质受侵蚀,3例见软组织肿块,3例增强扫描者示增厚的滑膜和结节明显强化。结论:MRI对色素沉着绒毛结节性滑膜炎诊断及鉴别诊断具有重要价值,是一种简单、无创、有效的检查手段。     

膝关节色素沉着绒毛结节性滑膜炎影像学表现

目的 探讨膝关节色素沉着绒毛结节性滑膜炎(PVNS)的病理与影像学表现.方法 对21例膝关节PVNS的影像学表现进行回顾性分析,并与手术病理结果对照.结果 4例X线平片表现为关节间隙增宽,关节边缘骨侵蚀伴硬化;8例CT显示关节囊肿胀、软骨下边缘骨侵蚀伴硬化;14例MR显示关节内脂肪垫受侵,10例半月板及交叉韧带受累,13例软骨或骨受侵.所有病例MRI表现均有滑膜增厚、关节内软组织结节,T1WI呈等或低信号,T2WI呈低或高低混杂信号,以及不同程度关节积液.结论 与CT、X线相比,MRI对膝关节PVNS的显示具有特征性,是诊断PVNS最理想的影像学检查方法.     

色素沉着绒毛结节性滑膜炎的MRI表现

目的 探讨色素沉着绒毛结节性滑膜炎的MRI表现特征和MRI诊断价值。方法 回顾分析经手术和病理证实的5例色素沉着绒毛结节性滑膜炎的MRI图像，全部病例采用SE序列和注射钆喷酸葡胺增强扫描。结果 1例局限型表现为关节囊结节状不规则增厚；4例弥漫型表现为关节旁葡萄串状囊性或囊实性包块，内有不规则结节，结节在T1、T2WI表现为低信号，囊液T2WI呈高低混杂信号、T1WI呈等信号，包块半包匀节囊及邻近骨生长；增强扫描绒毛结节及囊壁均匀强化，囊液信号变低。结论 色素沉着绒毛结节性滑膜炎具特征性MRI表现，MRI可作出确诊。     

MRI of the knee in diffuse pigmented villonodular synovitis

Magnetic resonance imaging (MRI) was performed on 11 patients with surgically proven pigmented villonodular synovitis (PVNS) of the knee. PVNS was diagnosed on the basis of presence of hemosiderin, joint effusion, and hyperplastic synovium without significant joint destruction. MRI provided a detailed map of the distribution of the disease within the joint, emphasizing the common occurrence of the disease behind the cruciate ligaments and in synovial cysts in the popliteal fossa. MRI aided in preoperative planning and postoperative follow-up for residual and recurrent disease. Nine additional cases of joint hemorrhage, hemophilia, desmoplastic tumors, and synovial chondromatosis were included to delineate differential diagnostic criteria.     

膝关节色素沉着绒毛结节性滑膜炎MRI表现

目的:探讨膝关节色素沉着绒毛结节性滑膜炎(pigmented villonodular synovitis, PVNS)的MRI表现.方法:分析6例PVNS患者行MRI检查并经病理证实病例的MRI资料.结果:6例均为弥漫型PVNS,表现为不规则滑膜增厚及关节软骨的破坏,2例表现为关节腔积液,5例表现为关节滑膜呈不规则结节状增生,2例增强后病灶呈线状网格状强化,4例表现为骨质侵蚀破坏改变,1例表现为关节外侵犯.结论:MRI能准确反映PVNS中滑膜的绒毛结节状增生、增生滑膜及其对关节软骨的破坏以及增生滑膜对软骨下骨质及软骨下远部骨质侵蚀的不同时期绒毛结节的影像学特征,并可以准确评价病变对关节内外累及的范围,是诊断PVNS最佳检查方法.