软组织肿瘤 病理学病例讨论 五.ppt

Sarcomas in Pediatric Age Group幼年较常见的肉瘤 Rhabdomyosarcoma 横纹肌肉瘤 (45%) PNET / extraskeletal Ewing’s sarcoma 原始性神经外胚层瘤或骨外尤文肉瘤 (22%) MPNST 恶性神经鞘瘤 (8%) Synovial sarcoma 滑膜肉瘤 (5%) Leiomyosarcoma 平滑肌肉瘤 (3%) Fibrosarcoma 纤维肉瘤 (2%) Extrarenal rhabdoid tumor 肾外横纹肌样瘤 (2%) Alveolar soft part sarcoma 腺泡状软组织肉瘤 (1%) Others Immunohistochemistry免疫组化 Cytokeratin + Vimentin + CD99 + Neuron-specific enolase / chromogranin / synaptophysin + (focal) Smooth muscle actin + (focal) S-100 protein / HMB45 - CD34 / CD 31 - bcl-2 - Desmin - p63 - Calretinin - Cytokeratin-positivity in Soft Tissue Lesions细胞角蛋白呈阳性的软组织病变 Metastatic carcinoma / mesothelioma 转移性癌或间皮瘤 Chordoma 脊索瘤 Synovial sarcoma 滑膜肉瘤 Epithelioid sarcoma 上皮样肉瘤 Epithelioid hemangioendothelioma / angiosarcoma 上皮样血管内皮瘤或血管肉瘤 Desmoplastic small round cell tumor 结缔组织增生性小细胞瘤 Mixed tumor 混合瘤 / myoepithelioma 肌上皮细胞瘤 / parachordoma 副脊索瘤 Reticulum cell sarcoma 网状细胞肉瘤 Adamantinoma of long bones 长骨釉质瘤 PNET / extraskeletal Ewing’s sarcoma 原始性神经外胚层瘤或骨外尤文肉瘤 Rare sarcomas with aberrant cytokeratin expression Electron Microscopy 电镜 Paranuclear whorls of intermediate filaments 成旋渦状的中丝聚于核周胞浆 (8 – 10 nm in diameter), associated with mitochondria 线粒体, lipid globules 脂滴, lysosomes 溶酶体, rER 粗面内浆网 free ribosomes 自由的核糖体 Short bundles of tonofilaments 短束状的张力微丝 Abortive cytoplasmic processes 不发达的胞浆突起 Primitive cell junctions 原始性的细胞接连 Histologic Differential Diagnosis 组织学鉴别诊断 Epithelioid sarcoma 上皮样肉瘤 (conventional 惯常型 vs. “proximal-type” 中央型) Extrarenal rhabdoid tumor 肾外横纹肌样瘤 Metastatic carcinoma 转移性癌 Epithelioid Sarcoma 上皮样肉瘤:Conventional 惯常型 vs. “Proximal-type” 中央型 Conventional epithelioid sarcoma 惯常型上皮样肉瘤: - Young adults 年轻成年患者 - Distal location 外周位置 (e.g. extremities 肢) - Superficial or deep-seated 表层或深层组织 - Necrobiotic granuloma-like morphology 肉芽肿样形态 - Repeated local recurrences 局部复发 with 5-year survival rate 存活率 = 50 – 80% “Proximal-type” epithelioid sarcoma 中央型上皮样肉瘤: - Older adults 年长成年患者 - Axial location 中轴位置 (e.g. pelvis 盘腔, perineum 会阴