Primary synovial chondromatosis and synovial chondrosarcoma: a pictorial review

This article illustrates the imaging characteristics of primary synovial chondromatosis (PSC) using 20 cases referred to a tertiary orthopaedic oncology centre. Three quarters of patients presented with a large intra-articular soft tissue mass and a suspected clinical and radiological diagnosis of malignancy made in the referring centres. Radiographs demonstrated fine cartilaginous mineralisation in the soft tissue masses in 85% cases and bone erosions were shown on MR imaging in 80%. Malignant transformation to chondrosarcoma was proven in 2 cases with longstanding disease. There were no specific MR features to distinguish these cases with malignant change from PSC alone. Primary synovial chondromatosis should be considered in the diagnosis of the monarticular presentation of an intra-articular soft tissue mass, particularly in the presence of superficial bone erosions and signal voids due to the mineralisation.     

原发性前列腺滑膜肉瘤1例并文献复习

1 病例资料 患者 男,28岁.于3月前无明显诱因出现肛周疼痛,大便变细,10余天前,小便次数增多,血清前列腺特异抗原(PSA)0.05 ng/mL. MRI检查示:前列腺区不规则团块状等T1长T2信号影(图1),内可见低信号分隔,DWI扩散受限呈明显高信号,ADC图呈低信号,边界欠清,累及直肠前壁、膀胱后壁.注射Gd-DTPA后,病灶呈明显不均匀强化(图2),直肠前壁明显增厚(图3).CT检查:前列腺区不规则软组织密度团块(图4),边界不清,增强呈不均匀明显强化(图5).患者行手术治疗,术中可见盆腔巨大肿块,与膀胱壁、直肠、精囊关系密切.     

Synovial chondrosarcoma: report of a case and review of the literature

Primary synovial chondromatosis is a relatively uncommon condition. Synovial chondrosarcoma is considered to be very rare and it is not always clear whether the sarcoma develops by malignant transformation of synovial chondromatosis or whether it arises de novo. Differentiation of the two conditions on clinical and radiographic features is not possible and on histological criteria can be difficult. We report the case of a 59-year-old man who, 4 years after a synovectomy for synovial chondromatosis, developed a synovial chondrosarcoma of the hip. The literature is reviewed.     

髋关节不典型滑膜软骨瘤病1例

患者女,52岁。左髋部进行性肿痛伴活动受限8月余加重2周。查体：左髋关节半屈位,髋关节周围肿胀饱满,腹部沟区压痛明显,有反跳痛。实验室检查：血、尿、粪、血沉、结核抗体、生化全项、类风湿因子、C-反应蛋白未见异常。     

宫颈原发黑色素瘤1例报告并文献回顾

患者 女,64岁,绝经14年,阴道分泌物增多15d就诊,全身皮肤黏膜无黑痣糜烂、破溃.妇科检查:宫颈表面呈紫黑色,凹凸不平,有接触性出血,外阴、阴道无异常.盆腔CT示宫颈增宽,密度不均,CT值25～35 HU左右,无钙化,宫颈周围脂肪间隙模糊(图1).3月后查盆腔MRI示宫颈结构消失,被140 mm×92 mm×91 mm大小混杂信号肿物取代.肿块内见片状短T1、短T2信号(图2,3),无液一液平面,余部以等T1、长T2信号为主.     

Dedifferentiated chondrosarcoma in patients with multiple osteochondromatosis: report of a case and review of the literature

 Multiple osteochondromatosis (MOS) is a familial disorder of autosomal dominant transmission characterized by the development of multiple exostoses and often derangements of epiphyseal cartilage, sometimes resulting in long bone growth retardation. Patients with the disorder appear to be at increased risk for developing secondary chondrosarcomas. Rarely, dedifferentiated chondrosarcomas may also occur. We report a single case of a 27-year-old man with multiple osteochondromatosis who developed a fatal dedifferentiated chondrosarcoma. Radiographically, the neoplasm arose from the pelvis completely destroying the left pubic ramus. Subsequently, the patient underwent preoperative chemotherapy followed by a left external hemipelvectomy. On pathologic examination, the tumor was characterized by high-grade pleomorphic sarcoma sharply juxtaposed to a low-grade chondrosarcoma. The patient ultimately died of widespread metastatic sarcoma.     

Bilateral asymmetric hip dislocation: case report and literature review

Simultaneous anterior and posterior hip dislocation is an unusual injury. A unique case is presented, consisting of bilateral asymmetric hip dislocation with associated femoral head, femoral shaft, and acetabular fractures resulting from a motorcycle collision. The mechanisms of injury, management, role of imaging, and complications of this injury complex are discussed, with a review of the relevant literature.     

MRI of mesenchymal chondrosarcoma of the orbit: case report and review of the literature

Extraskeletal mesenchymal chondrosarcoma is a relatively uncommon entity, an orbital location being extremely rare. A review of the literature revealed 16 reported cases of primary orbital mesenchymal chondrosarcoma demonstrated by plain film and CT. To the best of our knowledge, the MRI features of orbital extraskeletal mesenchymal chondrosarcoma have not been previously reported. We present the case of an 18-year-old man with a 2-year history of progressive proptosis of the right eye who underwent CT, dynamic CT, MRI without and with gadolinium enhancement, and magnetic resonance angiography of the orbits. CT of orbital mesenchymal chondrosarcoma demonstrates a well-defined mass with multiple areas of fine and coarse calcification and shows moderate contrast enhancement. The noncalcified portions of the mass demonstrate signal intensity lower than or equal to gray matter on T1-weighted images and are isointense to the gray matter on T2-weighted images. Dynamic CT reveals delayed contrast enhancement. MRI has proven to be a valuable diagnostic tool in the diagnosis and differentiation of well-defined intraorbital masses. By a combination of CT and MRI, it appears mesenchymal chondrosarcoma can be differentiated from other intraorbital lesions, such as cavernous hemangioma, hemangiopericytoma, orbital amyloidosis and fibrous histiocytoma. Received: 29 February 1996 Accepted: 18 June 1996     

Primitive neuroectodermal tumour in a 60-year-old man: a case report and literature review

Primitive neuroectodermal tumour (PNET) is very rare, especially in adults. We report a 60-year-old man presented with a PNET. The symptoms at the time of diagnosis were intense headache, Broca's aphasia and right hemiparesis. Only an open biopsy was performed. Irradiation of the primary tumour was the main treatment (total tumour dose 59.8 Gy) because of serious haematological side effects due to chemotherapy. The patient tolerated radiation therapy extremely well and his neurological symptoms were improved. 1 month after completion of radiotherapy, MRI showed no regression of the tumour. Clinical deterioration was observed 10 months after the initial diagnosis and the patient died 2 months later. In cases of PNET, initial therapy is surgical bulk reduction whenever possible. Irradiation of the cerebrospinal axis is justified as a routine treatment but, owing to the radioresistance of the tumour, the addition of multiregimen chemotherapy appears to improve survival, according to the literature.     

一例左肺上叶间叶性软骨肉瘤的影像表现

患者女,32岁.因左侧胸痛、咯痰伴痰中带血1个月,加重伴气短半个月于2009年5月11日入院.既往无肝炎及结核病史.入院体检:气管尚居中,左侧胸廓内陷,肋间隙变窄.     

Primary paraganglioma simulating pituitary macroadenoma: a case report and review of the literature

A 76-year-old woman presented with an intrasellar and suprasellar mass which caused deterioration of visual acuity and bitemporal visual field defects. Trans-sphenoidal and transcranial partial resection revealed a primary chemodectoma. This tumour is very rare in the sellar area, where there are normally no paraganglionic cells. We review the literature and discuss possible mechanisms for the development of this tumour. Received: 1 June 1997 Accepted: 27 October 1997     

原发性肺隐球菌病1例

患者女性,41岁。因＂反复头晕、视物旋转伴恶心2年,乏力1年入住心内科。原有高血压病1年。近期偶有少量咳嗽,无畏寒、发热,无消瘦、夜间盗汗。入院查体：T36.8°C,P100次/min,R 20次/min,BP 130/80mmHg。神清,精神可,浅表淋巴结未及肿大。双肺呼吸音清,未闻及干湿性罗     

Isolated synovial chondromatosis of the subacromial bursa: report of a new case and review of the literature

Synovial chondromatosis is a rare metaplasia of the synovium of unknown etiology that may involve occasionally the subacromial bursa. We report a new case diagnosed by ultrasound in a 30-year-old man and we present pathogenetic, diagnostic and therapeutic features of this disease with a literature review.     

Presacral ganglioneuroma in an 8-year-old child: case report,and literature review

Ganglioneuromas are benign tumors of the sympathetic nervous system, rarely found in the presacral region. In this study, we report the case of presacral ganglioneuroma in an 8-year-old girl, who complained of abdominal pain with diarrhea and abdominal distension. Ultrasound showed a large hypoechoic pelvic mass complicated by right ureter hydronephrosis. the CT and MRI confirm the presence of a presacral tissue mass with heterogeneous enhancement after contrast injection. The child underwent a complete surgical resection, and the anatomopathological study returned in favor of a ganglioneuroma. The presacral ganglioneuroma is an extremely rare tumor in that only twenty cases have been reported in the literature. 3 of which were less than 8-years-old. Through our case, we will review the epidemiological, clinical, radiological and therapeutic characteristics of this type of tumor.     

Primary alveolar soft part sarcoma of vertebra: a case report and literature review

Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor, which rarely occurs in bone. We present a case of ASPS in a 23-year-old man with a 2-month history of back pain. Computed tomography scanning and magnetic resonance images demonstrated a destructive process in the 12th thoracic vertebra associated with a unilateral soft tissue mass. The tumor showed evidence of hypervascularity on MRI; it obviously was enhanced on T1-weighted images after injection of Gd-GDPA, and signal voids were shown on all pulse sequences which may help to differentiate ASPS from other tumors of the vertebra. We believe that this is the first case of ASPS arising in a vertebra.     

Primary aortoenteric fistula: A case report and brief review of the literature

Aortoenteric fistula is a life-threatening emergency and is associated with high morbidity and mortality. Prompt surgical intervention before the aneurysm ruptures lowers the mortality rate to about 50%. Potential imaging mimics for aortoenteric fistula include retroperitoneal fibrosis, mycotic aortic aneurysm, and infectious aortitis. Secondary aortoenteric fistula has relative higher incidence compared to primary and is more common with open aortic repair versus endovascular stent graft repair. Ectopic gas in the aneurysm sac and extravasation of enteric contrast into the aneurysm sac is diagnostic for aortoenteric fistula. However, enteric contrast is not recommended for routine evaluation of aortoenteric because the aforementioned finding is extremely rare. More common imaging findings include bowel loop appearing adherent to aneurysm sac with associated inflammatory stranding and foci or ectopic gas within the aneurysm sac or interposed between the bowel and aneurysm sac. Here we present a case of 52-year-old male who presents with incidental primary aortoenteric fistula.     

Primary malignant mesenchymoma of bone: case report,literature review,and distinction of this entity from mesenchymal and dedifferentiated chondrosarcoma

The seventh case of primary malignant mesenchymoma of bone is reported. It is compared to the other six cases in the literature and a clinicopathologic pattern of this entity is developed. Primary malignant mesenchymoma of bone is compared to mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Differences as well as similarities are discussed and a more current classification scheme for malignant mesenchymoma of bone is proposed.