软组织肿瘤病理学病例讨论五.ppt

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1、Sarcomas in Pediatric Age Group幼年较常见的肉瘤幼年较常见的肉瘤vvRhabdomyosarcoma Rhabdomyosarcoma 横纹肌肉瘤横纹肌肉瘤横纹肌肉瘤横纹肌肉瘤 (45%)(45%)vvPNET/extraskeletal Ewings sarcoma PNET/extraskeletal Ewings sarcoma 原始性神经原始性神经原始性神经原始性神经外胚层瘤或骨外尤文肉瘤外胚层瘤或骨外尤文肉瘤外胚层瘤或骨外尤文肉瘤外胚层瘤或骨外尤文肉瘤 (22%)(22%)vvMPNST MPNST 恶性神经鞘瘤恶性神经鞘瘤恶性神经鞘瘤恶性神经鞘瘤 (8

2、%)(8%)vvSynovial sarcoma Synovial sarcoma 滑膜肉瘤滑膜肉瘤滑膜肉瘤滑膜肉瘤 (5%)(5%)vvLeiomyosarcoma Leiomyosarcoma 平滑肌肉瘤平滑肌肉瘤平滑肌肉瘤平滑肌肉瘤 (3%)(3%)vvFibrosarcoma Fibrosarcoma 纤维肉瘤纤维肉瘤纤维肉瘤纤维肉瘤 (2%)(2%)vvExtrarenal rhabdoid tumor Extrarenal rhabdoid tumor 肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤 (2%)(2%)vvAlveolar soft part sarcoma

3、 Alveolar soft part sarcoma 腺泡状软组织肉瘤腺泡状软组织肉瘤腺泡状软组织肉瘤腺泡状软组织肉瘤(1%)(1%)vvOthersOthersS-100 proteinS-100 proteinCD34CD34VimentinVimentinCytokeratinCytokeratin (Cam5.2)(Cam5.2)CytokeratinCytokeratin (AE1/3)(AE1/3)CD99CD99Smooth muscle Smooth muscle actinactinNeuron-specific Neuron-specific enolaseenolase

4、ChromograninChromograninSynaptophysinSynaptophysinImmunohistochemistry免疫组化免疫组化vvCytokeratin+Cytokeratin+vvVimentin+Vimentin+vvCD99+CD99+vvNeuron-specific enolase/Neuron-specific enolase/chromogranin/synaptophysin+chromogranin/synaptophysin+(focal)(focal)vvSmooth muscle actin+Smooth muscle actin+(foc

5、al)(focal)vvS-100 protein/HMB45-S-100 protein/HMB45-vvCD34/CD 31-CD34/CD 31-vvbcl-2-bcl-2-vvDesmin-Desmin-vvp63-p63-vvCalretinin-Calretinin-Cytokeratin-positivity in Soft Tissue LesionsCytokeratin-positivity in Soft Tissue Lesions细胞角蛋白呈阳性的软组织病变细胞角蛋白呈阳性的软组织病变细胞角蛋白呈阳性的软组织病变细胞角蛋白呈阳性的软组织病变vvMetastatic c

6、arcinoma/mesothelioma Metastatic carcinoma/mesothelioma 转移性癌或间皮瘤转移性癌或间皮瘤转移性癌或间皮瘤转移性癌或间皮瘤vvChordoma Chordoma 脊索瘤脊索瘤脊索瘤脊索瘤vvSynovial sarcoma Synovial sarcoma 滑膜肉瘤滑膜肉瘤滑膜肉瘤滑膜肉瘤vvEpithelioid sarcoma Epithelioid sarcoma 上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤vvEpithelioid hemangioendothelioma/angiosarcoma Epithelioid hemang

7、ioendothelioma/angiosarcoma 上皮样血管上皮样血管上皮样血管上皮样血管内皮瘤或血管肉瘤内皮瘤或血管肉瘤内皮瘤或血管肉瘤内皮瘤或血管肉瘤vvDesmoplastic small round cell tumor Desmoplastic small round cell tumor 结缔组织增生性小细胞瘤结缔组织增生性小细胞瘤结缔组织增生性小细胞瘤结缔组织增生性小细胞瘤vvMixed tumor Mixed tumor 混合瘤混合瘤混合瘤混合瘤/myoepithelioma /myoepithelioma 肌上皮细胞瘤肌上皮细胞瘤肌上皮细胞瘤肌上皮细胞瘤 /parach

8、ordoma parachordoma 副脊索瘤副脊索瘤副脊索瘤副脊索瘤vvReticulum cell sarcoma Reticulum cell sarcoma 网状细胞肉瘤网状细胞肉瘤网状细胞肉瘤网状细胞肉瘤vvAdamantinoma of long bones Adamantinoma of long bones 长骨釉质瘤长骨釉质瘤长骨釉质瘤长骨釉质瘤vvPNET/extraskeletal Ewings sarcomaPNET/extraskeletal Ewings sarcoma 原始性神经外胚层瘤或原始性神经外胚层瘤或原始性神经外胚层瘤或原始性神经外胚层瘤或骨外尤文肉瘤骨

9、外尤文肉瘤骨外尤文肉瘤骨外尤文肉瘤vvRare sarcomas with aberrant cytokeratin expressionRare sarcomas with aberrant cytokeratin expressionElectron microscopy Electron microscopy 电镜电镜电镜电镜Paranuclear whorl of intermediate filamentsParanuclear whorl of intermediate filaments成旋渦状的中丝聚于核周胞浆成旋渦状的中丝聚于核周胞浆成旋渦状的中丝聚于核周胞浆成旋渦状的中丝聚

10、于核周胞浆Electron microscopy Electron microscopy 电镜电镜电镜电镜Intermediate filaments admixedIntermediate filaments admixedwith scanty free ribosomeswith scanty free ribosomes中丝混和自由的核糖体中丝混和自由的核糖体中丝混和自由的核糖体中丝混和自由的核糖体Electron microscopy Electron microscopy 电镜电镜电镜电镜Intermediate filaments Intermediate filaments a

11、dmixed with rERadmixed with rER中丝混和粗面内浆网中丝混和粗面内浆网中丝混和粗面内浆网中丝混和粗面内浆网Electron microscopy Electron microscopy 电镜电镜电镜电镜Whorl of intermediate filamentsWhorl of intermediate filaments中丝成旋渦状中丝成旋渦状中丝成旋渦状中丝成旋渦状Electron microscopy Electron microscopy 电镜电镜电镜电镜Short bundles of tonofilamentsShort bundles of tono

12、filaments短束状的张力微丝短束状的张力微丝短束状的张力微丝短束状的张力微丝Electron microscopy Electron microscopy 电镜电镜电镜电镜Tonofilaments&primitive cell junctionTonofilaments&primitive cell junction张力微丝与原始性的细胞接连张力微丝与原始性的细胞接连张力微丝与原始性的细胞接连张力微丝与原始性的细胞接连Electron microscopy Electron microscopy 电镜电镜电镜电镜Abortive cytoplasmic processesAbortiv

13、e cytoplasmic processes不发达的胞浆突起不发达的胞浆突起不发达的胞浆突起不发达的胞浆突起Electron Microscopy 电镜电镜vvParanuclear whorls of intermediate filaments Paranuclear whorls of intermediate filaments 成旋成旋成旋成旋渦状的中丝聚于核周胞浆渦状的中丝聚于核周胞浆渦状的中丝聚于核周胞浆渦状的中丝聚于核周胞浆 (8 10 nm in diameter)(8 10 nm in diameter),associated with mitochondria asso

14、ciated with mitochondria 线粒体线粒体线粒体线粒体,lipid,lipid globules globules 脂滴脂滴脂滴脂滴,lysosomes,lysosomes 溶酶体溶酶体溶酶体溶酶体,rER,rER 粗面内浆网粗面内浆网粗面内浆网粗面内浆网&free ribosomes free ribosomes 自由的核糖体自由的核糖体自由的核糖体自由的核糖体vvShort bundles of tonofilaments Short bundles of tonofilaments 短束状的张力微丝短束状的张力微丝短束状的张力微丝短束状的张力微丝vvAbortive

15、cytoplasmic processes Abortive cytoplasmic processes 不发达的胞浆突起不发达的胞浆突起不发达的胞浆突起不发达的胞浆突起vvPrimitive cell junctions Primitive cell junctions 原始性的细胞接连原始性的细胞接连原始性的细胞接连原始性的细胞接连Histologic Differential Diagnosis 组织学鉴别诊断组织学鉴别诊断vEpithelioid sarcoma Epithelioid sarcoma 上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤 (conventional(convent

16、ional 惯常型惯常型惯常型惯常型 vs.vs.“proximal-type”“proximal-type”中央型中央型中央型中央型)vExtrarenal rhabdoid tumor Extrarenal rhabdoid tumor 肾外肾外肾外肾外横纹肌样瘤横纹肌样瘤横纹肌样瘤横纹肌样瘤vMetastatic carcinoma Metastatic carcinoma 转移性癌转移性癌转移性癌转移性癌Epithelioid Sarcoma Epithelioid Sarcoma 上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤:Conventional Conventional 惯常型惯常

17、型惯常型惯常型 vs.“Proximal-type”vs.“Proximal-type”中央型中央型中央型中央型vvConventional epithelioid sarcoma Conventional epithelioid sarcoma 惯常型上皮样肉瘤惯常型上皮样肉瘤惯常型上皮样肉瘤惯常型上皮样肉瘤:-Young adults Young adults 年轻成年患者年轻成年患者年轻成年患者年轻成年患者-Distal location Distal location 外周位置外周位置外周位置外周位置 (e.g.extremities(e.g.extremities 肢肢肢肢)-Sup

18、erficial or deep-seated Superficial or deep-seated 表层或深层组织表层或深层组织表层或深层组织表层或深层组织-Necrobiotic granuloma-like morphology Necrobiotic granuloma-like morphology 肉芽肿样形态肉芽肿样形态肉芽肿样形态肉芽肿样形态-Repeated local recurrences Repeated local recurrences 局部复发局部复发局部复发局部复发 with 5-year survival with 5-year survival rate r

19、ate 存活率存活率存活率存活率 =50 80%=50 80%vv“Proximal-type”epithelioid sarcoma“Proximal-type”epithelioid sarcoma 中央型上皮样肉瘤中央型上皮样肉瘤中央型上皮样肉瘤中央型上皮样肉瘤:-Older adults Older adults 年长成年患者年长成年患者年长成年患者年长成年患者-Axial location Axial location 中轴位置中轴位置中轴位置中轴位置 (e.g.pelvis(e.g.pelvis 盘腔盘腔盘腔盘腔,perineum,perineum 会阴会阴会阴会阴&genital

20、 tract genital tract 阴道阴道阴道阴道)-Deep-seated Deep-seated 深层组织深层组织深层组织深层组织-More nuclear pleomorphism More nuclear pleomorphism 核多形性核多形性核多形性核多形性&prominent rhabdoid&prominent rhabdoid morphology morphology 明显的横纹肌样形态明显的横纹肌样形态明显的横纹肌样形态明显的横纹肌样形态-Aggressive clinical course Aggressive clinical course 预后差预后差预后

21、差预后差EpithelioidEpithelioid sarcoma(conventional)sarcoma(conventional)上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤(惯常型惯常型惯常型惯常型)Granuloma-like geographic necrosisGranuloma-like geographic necrosis肉芽肿样的坏死灶肉芽肿样的坏死灶肉芽肿样的坏死灶肉芽肿样的坏死灶EpithelioidEpithelioid sarcoma(“proximal-type”)sarcoma(“proximal-type”)上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤(中央型中央

22、型中央型中央型)Pleomorphic tumor cells with rhabdoid morphologyPleomorphic tumor cells with rhabdoid morphology多形性肿瘤细胞呈横纹肌样形态多形性肿瘤细胞呈横纹肌样形态多形性肿瘤细胞呈横纹肌样形态多形性肿瘤细胞呈横纹肌样形态“Proximal-type”Epithelioid Sarcoma“Proximal-type”Epithelioid Sarcoma 中央型中央型中央型中央型上皮样肉上皮样肉上皮样肉上皮样肉瘤瘤瘤瘤vs.Extrarenal Rhabdoid Tumor vs.Extrare

23、nal Rhabdoid Tumor 肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤vvBoth entities show:Both entities show:-Axial location Axial location 中轴位置中轴位置中轴位置中轴位置-Rhabdoid morphology Rhabdoid morphology 横纹肌样形态横纹肌样形态横纹肌样形态横纹肌样形态-Cytokeratin+Cytokeratin+细胞角蛋白阳性细胞角蛋白阳性细胞角蛋白阳性细胞角蛋白阳性-Similar ultrastructural findings Similar ultras

24、tructural findings 电镜下形态相似电镜下形态相似电镜下形态相似电镜下形态相似-Frequent aberrations of chromosome 22q Frequent aberrations of chromosome 22q 染色体变异相近染色体变异相近染色体变异相近染色体变异相近-Aggressive clinical course Aggressive clinical course 预后差预后差预后差预后差(5-year survival rate (5-year survival rate 30%)with multimodal therapy resista

25、nce&early tumor-30%)with multimodal therapy resistance&early tumor-related deathsrelated deaths-Frequent metastasis to lung,lymph nodes&liver Frequent metastasis to lung,lymph nodes&liver 肺、淋肺、淋肺、淋肺、淋巴结和肝转移巴结和肝转移巴结和肝转移巴结和肝转移“Proximal-type”Epithelioid Sarcoma“Proximal-type”Epithelioid Sarcoma 中央型中央型中

26、央型中央型上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤vs.Extrarenal Rhabdoid Tumor vs.Extrarenal Rhabdoid Tumor 肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤vv“Proximal-type”epithelioid sarcoma“Proximal-type”epithelioid sarcoma 中央型中央型中央型中央型上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤:-Adult age group Adult age group 成年患者成年患者成年患者成年患者(20 40 years)(20 40 years)-Involves

27、pelvis Involves pelvis 盘腔盘腔盘腔盘腔,perineum,perineum 会阴会阴会阴会阴&genital tract&genital tract 阴道阴道阴道阴道 (pubis(pubis 耻骨耻骨耻骨耻骨,vulva,vulva 外阴外阴外阴外阴&penis&penis 阴茎阴茎阴茎阴茎)-Size:1 20 cmSize:1 20 cm-CD34+(50%cases)CD34+(50%cases)vvExtrarenal rhabdoid tumor Extrarenal rhabdoid tumor 肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤:-

28、Pediatric age group Pediatric age group 幼年患者幼年患者幼年患者幼年患者(10 years,sometimes (10 years,sometimes congenital congenital 偶见先天性偶见先天性偶见先天性偶见先天性)-Involves CNSInvolves CNS 中枢神经中枢神经中枢神经中枢神经,liver,liver 肝肝肝肝,heart,heart 心心心心,GI tract,GI tract 胃肠胃肠胃肠胃肠,thymus thymus 胸腺胸腺胸腺胸腺,paraspinal soft tissue,paraspinal

29、soft tissue 脊周软组织脊周软组织脊周软组织脊周软组织&skin&skin 皮肤皮肤皮肤皮肤-Size:5 cmSize:5 cm-CD34 (but neuroectodermal markers CD34 (but neuroectodermal markers 神经外胚层标记神经外胚层标记神经外胚层标记神经外胚层标记 sometimes+)sometimes+)Controversies RegardingControversies RegardingExtrarenal Rhabdoid Tumor&Extrarenal Rhabdoid Tumor&“Proximal-ty

30、pe”Epithelioid Sarcoma“Proximal-type”Epithelioid Sarcoma关于肾外横纹肌样瘤及中央型上皮样肉瘤的争议关于肾外横纹肌样瘤及中央型上皮样肉瘤的争议关于肾外横纹肌样瘤及中央型上皮样肉瘤的争议关于肾外横纹肌样瘤及中央型上皮样肉瘤的争议vvSome tumors reported as extrarenal rhabdoid Some tumors reported as extrarenal rhabdoid tumors are examples of“proximal-type”tumors are examples of“proximal-t

31、ype”epithelioid sarcoma epithelioid sarcoma(Fletcher CDM,et al.)(Fletcher CDM,et al.)vvRhabdoid tumor is not a distinct entityRhabdoid tumor is not a distinct entity (Fletcher CDM,et al.)(Fletcher CDM,et al.)vv“Proximal-type”epithelioid sarcoma more likely“Proximal-type”epithelioid sarcoma more like

32、ly represents a variant of extrarenal rhabdoid represents a variant of extrarenal rhabdoid tumor tumor(Weiss SW,et al.)(Weiss SW,et al.)vvNot yet convinced that“proximal-type”Not yet convinced that“proximal-type”epithelioid sarcoma represents a distinct entityepithelioid sarcoma represents a distinc

33、t entity (Weiss SW,et al.)(Weiss SW,et al.)Final Pathologic Diagnosis病理学诊断结论病理学诊断结论vExtrarenal rhabdoid tumor 肾外横纹肌样瘤肾外横纹肌样瘤/“Proximal-type”epithelioid sarcoma 中央型上皮样肉瘤中央型上皮样肉瘤Extrarenal Rhabdoid TumorExtrarenal Rhabdoid Tumor 肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤:Histogenesis Histogenesis 发病机制发病机制发病机制发病机制vv

34、Pure form Pure form 单纯型单纯型单纯型单纯型:-Likely a distinct entityLikely a distinct entity-Multidirectional differentiation Multidirectional differentiation 多向性分化多向性分化多向性分化多向性分化(or (or simply non-specific cross-reactive immunophenotype simply non-specific cross-reactive immunophenotype 交叉染色反应交叉染色反应交叉染色反应交叉染

35、色反应)-Mutations&homozygous deletions of Mutations&homozygous deletions of SMAR-CB1 SMAR-CB1(hSNF5hSNF5 or or INI1INI1)tumor suppressor gene in)tumor suppressor gene in chromosome 22q(similar to renal&CNS rhabdoid chromosome 22q(similar to renal&CNS rhabdoid tumor)tumor)染色体变异与肾或中枢神经系統横纹肌样瘤相似染色体变异与肾或中枢

36、神经系統横纹肌样瘤相似染色体变异与肾或中枢神经系統横纹肌样瘤相似染色体变异与肾或中枢神经系統横纹肌样瘤相似vvComposite form Composite form 合成型合成型合成型合成型:-Heterogeneous entitiesHeterogeneous entitiesExtrarenal Rhabdoid TumorExtrarenal Rhabdoid Tumor 肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤肾外横纹肌样瘤:Treatment 治疗方法治疗方法vvEarly radical local excision Early radical local excision

37、 局部全切除局部全切除局部全切除局部全切除术术术术®ional lymph node dissection®ional lymph node dissection 区区区区内淋巴结切除内淋巴结切除内淋巴结切除内淋巴结切除vvAdjuvant radiotherapy Adjuvant radiotherapy 放射治療放射治療放射治療放射治療&multiagent chemotherapy multiagent chemotherapy 化学治療化学治療化学治療化学治療Diagnostic Algorithm ofExtrarenal Rhabdoid Tumor肾外横纹肌样瘤的诊

38、断要诀肾外横纹肌样瘤的诊断要诀vvProminent rhabdoid morphology Prominent rhabdoid morphology 明显的横明显的横明显的横明显的横纹肌样形态纹肌样形态纹肌样形态纹肌样形态vvVimentin&epithelial markers Vimentin&epithelial markers(e.g.(e.g.cytokeratin)cytokeratin)+波形蛋白和上皮标记呈阳性波形蛋白和上皮标记呈阳性波形蛋白和上皮标记呈阳性波形蛋白和上皮标记呈阳性vvExclusion of alternative line of Exclusion of

39、 alternative line of differentiation differentiation 并无其他分化并无其他分化并无其他分化并无其他分化Case 6(2148 胜利油田中心医院 043976)女 59岁 会阴部肿块会阴部肿块疼痛4月余，渐大。查体：会阴部左侧坐骨结节内前侧可触及一56cm大 小肿块，实性，质硬，与周围分界清。标本检查：送检卵圆形肿物结节一个，体积：53.53.5cm，切面灰白，质硬韧，一边缘有编织状纹理，界清。讨 论会诊意见华西：(会阴部左侧坐骨结节内）恶性外周神经鞘膜瘤（可见肿瘤围绕小神经支）。廖松林：(坐骨结节内侧）梭形或短梭形细胞肉瘤，细胞比较一致，考虑

40、以下可能，1、梭形细胞恶黑（可能是透明细胞肉瘤）2、单向型滑膜肉瘤3、平滑肌肉瘤4、MPNST。建议染免疫组化进行鉴别诊断。朱雄增：(坐骨结节内侧）平滑肌肉瘤，中度分化。免疫组化HMB45S100MBP CKEMASMADESCD34Melan AHMB-45Melan AMBPS-100Case 7(SP-5 Lung Nodules)41岁女患，呼吸短促。该患有子宫出血病史现子宫切除术后。CTCT检查发现其肺脏多发的小病灶。进行活检以明确病变性质。大体标本大体标本示两块大小分别为1.5 和 1.8 cm的楔形肺组织.最大切面可见三个境界清晰的直径0.5 到1.2 cm质硬结节。切片示具代表性的病灶SP-5Diagnosis?Differential DiagnosisMetastatic leiomyosarcoma from uterusPrimary leiomyomatosis of the lungMetastatic spindle cell malignancyBenign metastasizing leiomyoma(of the lung)