长海进修读片骨关节-尤文

1、叶华教授 王铁功2014-10-24Ewing a Family of Tumors：Radiologic-Pathologic Correlation尤文氏家族肿瘤：影像与病理联系Mark D. Murphey et al. RadioGraphics 2013;33:803-831.Ewing a family of tumors Ewing a of bone （EWS） extraskeletal Ewing a primitive neuroectodermal tumor （PNET） Askin tumor2Mark D. Murphey etal. RadioGraphics

2、2013;33:803-831. In 1921, James Ewing first described the tumor of bone. In 1975, Angervall and Enzinger reported extraskeletal Ewing a. Subsequently，lesions in bone and soft tissue with histologic rosette formation and other pathologic features of neural differentiation , were described as primitiv

3、e neuroectodermal tumor (PNET). In 1979, Askin and colleagues reported a similar series of lesions involving the chest wall , referred to as Askin tumors.3Ewing a family of tumorsCytogenetic evaluation a translocation involving chromosomes 11 and 22 (t11;22q24;q12) is present in approximately 90% of

4、 these lesions. 4Ewing a family of tumors5Histology featurescrowded sheets of small round blue cells （密集的小圆细胞）Homer-Wright rosettes formation （假菊形团结构）Ewing a family of tumorsPrevalenceThe second most common primary malignant tumor of bone in children and adolescents , exceeded in prevalence only by

5、a （仅次于骨肉瘤）The fourth most frequent primary malignant tumor of bone after multiple myeloma, a,and a.（次于多发性骨髓瘤、骨肉瘤、软骨肉瘤）Account for approximately 3% of all pediatric cancers. Age 425Y，95% (the peak prevalence,1015Y)6osseous EWSLocationFemur 21% （股骨）Ilium 12% (髂骨)Tibia 8%-11% （胫骨）Humerus 10% (肱骨)Fibula

6、 7%-9% （腓骨）Ribs 8% （肋骨）long tubular bone （长管状骨）metadiaphyseal 44%-59% (骨干干骺端)， diaphyseal 33%-35% （骨干），metaphysis 5%-15% （干骺端）7osseous EWSClinical Characteristics （nonspecific）pain 82%-88% （疼痛）a mass or swelling 60% (肿块或软组织肿胀)fever 20%-49% （发热）ESR pathological fractures （首发症状病理性骨折少见）8osseous EWSPath

7、ologic Featuresa soft gray-to-white mass （灰白色肿块)well-defined margins （边界清晰）osseous lesions originate in the central medullary canal ,with soft tissues in 80%-100%. （肿瘤位于髓腔中央，伴软组织肿块）crowded sheets of small round blue cells or lobules of such cells divided by small amount of fibrous stroma （密集的小圆细胞）Ho

8、mer-Wright rosettes （假菊形团结构）hemorrhage or necrosis （出血、坏死）cytoplasm contains glycogen （胞质富含糖原）CD99，NSE，CD57，FL-1 protein，vimentin （+）9osseous EWS10The communication between the medullary canal and soft-tissue componentsosseous EWS X、CT findings bone destruction with a moth-eaten to permeative patter

9、n （虫蚀状、穿凿样骨质破坏，76%-82%）poor margination （边界不清，96%）soft-tissue mass： circumferential but asymmetric (肿块位于瘤骨周围，不对称)11osseous EWS MRI findingsmarrow replacement（骨髓占位，100%）cortical destruction（骨皮质破坏， 92%）associated soft-tissue mass （伴随软组织肿块，96%）Homogeneous（73%）and intermediate（95%）signal intensity on T1

10、WI ；Homogeneous （86%）and low to intermediate（68%） signal intensity on T2WI；Heterogeneity and high signal intensity hemorrhage or necrosis.edema (水肿)（Unusual before treatment，unless there is an associated pathologic fracture. ）contrast enhancement 12osseous EWSFDG PETSUV 5.3（no metastases at presenta

11、tion）SUV 11.3（metastases at presentation） 13osseous EWS Subtle cortical involvement （linear intermediate signal intensity channels that extend through the low signal intensity cortex）14 a small round blue cell tumor （EWS or lymphoma or leukemia）osseous EWSMRI sign： M，16Y，swelling，pain，enlarging mass

12、 in the left distal lower extremity. 15Figure 1osseous EWS 同一患者16Figure 1osseous EWST1+CT2WI EWS of the proximal femoral metadiaphysis in a 16Y boy with pain17Figure 2osseous EWS18Figure 2osseous EWS同一患者 EWS of the proximal humeral metadiaphysis in a 25Y man with a skip metastasis.19Figure 3osseous

13、EWS EWS of the pelvis with prominent sclerosis in a 20Y man.20Figure 4osseous EWST2WI EWS In the metatarsal with prominent sclerosis in a 12Y boy21Figure 5osseous EWST1WI Periosteal EWS of the femoral diaphysis in a 14Y boy with pain in the right thigh.22Figure 6osseous EWST1+CT1WIT2WIEWS , PNET , A

14、skin tumor are cytogenetically closely related, which is referred to as the Ewing a family of tumors. The typical cytogenetic aberration is the nonrandom reciprocal translocation between chromosomes 11 and 22 （t 11;22q24;q12）. Bone destruction with a moth-eaten to permeative pattern is seen in 76%-8

15、2% of lesions, and a wide zone of transition (poor margination) is seen in 96% of lesions.23Teaching Point:osseous EWSPrevalence 15%-20% of that of osseous EWSAge 20M30Y （85%）Clinical features a large, rapidly growing, solitary, superficial or deep soft-tissue mass measuring 5-10cm 92% deep seated，8

16、% subcutaneous（Angervall and Enzingers series,39 patients）Location paravertebral region(32%),lower extremities(26%),chest wall (18%), retroperitoneum(11%),pelvis and hip(11%),upper extremities(3%) (椎旁，下肢，胸壁，腹膜后，盆腔，上肢)24Extraskeletal EWSdifferences25Extraskeleta EWSmore equally distributed between th

17、e sexSlightly older by 5-10 yearsTrunk （躯干骨）osseous EWSMale 4Y25YLower limbs (下肢)Extraskeletal EWSDiagnosis criteria26No osseous involvement at MRINo increased uptake in bone orperiosteum adjacent on bone scintigraphyA lesion composed of small round blue tumor cells Demonstration of cytoplasmic glyc

18、ogenExtraskeletal EWSImaging findings （nonspecific）a large soft-tissue mass (50%) or normalMargin: well-defined (36%), ill-defined (45%)calcification (25%) （钙化）The presence of serpentine high-flow vascular channels（90%） （can be seen in higher-grade vascular lesions）(迂曲高流量血管信号)prominent contrast enha

19、ncement (CT&MRI)DSA lesions are typically hypervascular （富血供）27Extraskeletal EWSImaging findings （nonspecific）adjacent bone erosion, cortical thickening, osseous invasion, aggressive periosteal reaction (邻近骨质受侵，骨皮质增厚，骨质侵犯，骨膜反应，25%-42%)neurovascular involvement (73%) （神经血管侵犯）direct invasion of bone i

20、s more common in the terminal stage（直接骨侵犯）28Extraskeletal EWS Extraskletal EWS in a periscapular location in a 21Y man with an enlarging painless mass. 29Figure 7Extraskeletal EWST1+CT1WIT2WI Extraskeletal EWS in an 11Y boy with enlarging axillary soft-tissue mass.30Figure 8high-flow vascular channe

21、lsExtraskeletal EWST1WIT1+CT2WIThe presence of serpentine high-flow vascular channels, which have low signal intensity with all pulse sequences, is not unique to extraskeletal EWS.However, the presence of this feature in a young person with a large intramuscular mass should raise the possibility of

22、extraskeletal EWS.31Teaching Point:Extraskeletal EWSIn 1979，Askin and coworkers described a malignant small round blue cell tumor in the thoracopulmonary region.Rare，in young adults and children，famaleMalignant，develop from embryonal migrating cells of the neural crest (起源于原始神经外胚层)Typically feature

23、a large tumor involving the chest wall and pleura (发生于胸壁、胸膜的巨大肿瘤)32Askin TumorClinical presentationPainFeverAnorexia (神经性厌食症)Weight loss33Askin TumorRadiography features pleural-based mass with pleural fluid (胸膜肿块合并胸腔积液)Pulmonary parenchymal disease (25%) (肺实质)Calcification (10%) (钙化)Ipsilateral hil

24、ar and mediastinal adenopathy (同侧肺门和纵膈内淋巴结肿大)Rib destruction (63%) (骨质破坏) uptake 111In & 99mTc （negative after surgical resection） no uptake FDG PET34Askin TumorCT findingslarge (巨大)unilateral (单侧)heterogeneous (不均质)chest wall mass (胸壁肿块)rib destruction (肋骨破坏)pleural effusions (胸膜渗出)intrathoracic an

25、d extrathoracic components (同时累及胸廓内外)with pleural,pericardial.diaphragmatic,spinal extension and involvement (伴胸膜、心包、膈肌、脊柱侵犯)35Askin TumorMRI findingslarge (巨大)hererogeneous (不均质)T1WI intermediate 、T2WI high signal intensity （T1WI中等、T2WI高信号）high signal intensity hemorrhage or necrosisDirect invasion

26、 of the chest wall musculature, mediastinum or lung is common. (直接侵犯胸壁肌肉、纵膈、肺组织)prominent vascular enhancement (明显强化)36 Askin Tumor Askin tumor in a 20Y man with right lower rib pain and mild tenderness.37Figure 9Askin TumorAskin Tumor Large Askin tumor of the chest wall in a 26Y man with lower back pain and difficulty walking.38Figure 10the