软组织肿瘤病理学病例讨论四课件

Case 4EX05-146(04/S10052).Presented by Q H Fan.History.Female aged 12.Subcutaneous lesion extending down to bone on the dorsum of the right middle finger.软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四Diangosis?软组织肿瘤病理学病例讨论四 Diagnosis Plexiform fibrohistiocytic tumor,subcutis extending down to bone,dorsum of right middle finger.软组织肿瘤病理学病例讨论四 Plexiform fibrohistiocytic tumor,a tumor first described by Drs.Enzinger and Zhang in 1988 when they reported of 65 cases from the files of the Armed Forces Institute of Pathology.软组织肿瘤病理学病例讨论四It occurs chiefly in children and young adults.The most common location was the upper extremity(63.1%),especially the regions of shoulder and forearm.软组织肿瘤病理学病例讨论四The median age of their 65 patients was 14.5 years;two-thirds(67.7%)were younger than 20 years;there were 46 females and only 19 males.软组织肿瘤病理学病例讨论四The tumors presented as a slow-growing,poorly demarcated dermal or subcutaneous mass that rarely exceeded 3 cm in greatest diameter.软组织肿瘤病理学病例讨论四Histologically,lesions were characterized by a multinodular or plexiform proliferation of histiocyte-and fibroblast-like cells associated with multinucleate giant cells,morphologic features reminiscent of both a fibrous histiocytoma and a fibromatosis.软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四软组织肿瘤病理学病例讨论四The differential diagnosis includes:1.Cutaneous fibrous histiocytoma 2.Plexiform neurofibroma,3.Fibromatosis,4.Benign and malignant giant cell tumors 软组织肿瘤病理学病例讨论四PrognosisTwenty of their 32 cases(Good:62.5%)with follow-up information were alive and well after local excision,but the tumor recurred in 12 cases(Recurred:37.5%).In two patients with recurrence,the disease(Metastasized:6%)metastasized to regional lymph nodes 9 and 36 months after the initial excision,respectively.Enzinger and Zhangs original findings have been confirmed in subsequent papers.软组织肿瘤病理学病例讨论四TreatmentPlexiform fibrohistiocytic tumor is a low grade soft tissue tumor of intermediate malignancy which frequently recurs locally but rarely metastasizes.Wide excision is believed to be the best treatment.软组织肿瘤病理学病例讨论四Case 5(Slide Seminar Case 04BX5833)Presented by:Dr.Wai-Kuen NgPresented by:Dr.Wai-Kuen Ng讲者：吴伟权医生讲者：吴伟权医生讲者：吴伟权医生讲者：吴伟权医生Division of Anatomical Pathology,Division of Anatomical Pathology,Department of Pathology&Clinical Biochemistry,Department of Pathology&Clinical Biochemistry,Queen Mary Hospital,Hong Kong SAR,ChinaQueen Mary Hospital,Hong Kong SAR,China香港玛丽医院病理及临床生化学部门香港玛丽医院病理及临床生化学部门香港玛丽医院病理及临床生化学部门香港玛丽医院病理及临床生化学部门软组织肿瘤病理学病例讨论四Clinical History 病历病历vv10-year-old boy with 10-year-old boy with torticollistorticollis&bilateral upper&bilateral upper limb weakness for 3 monthslimb weakness for 3 months患者男，患者男，患者男，患者男，1010岁。因斜颈及双手无力达岁。因斜颈及双手无力达岁。因斜颈及双手无力达岁。因斜颈及双手无力达3 3个月而就诊。个月而就诊。个月而就诊。个月而就诊。vvSpine X-ray:Spine X-ray:OsteolyticOsteolytic lesion over C2 vertebral body lesion over C2 vertebral body脊柱脊柱脊柱脊柱X X线：有一溶骨性病变在线：有一溶骨性病变在线：有一溶骨性病变在线：有一溶骨性病变在C2C2颈椎骨中。颈椎骨中。颈椎骨中。颈椎骨中。vvBone scan:Bone scan:Increased uptake in C1 to C3 regionIncreased uptake in C1 to C3 region骨扫描：骨扫描：骨扫描：骨扫描：C1C1至至至至C3C3位置摄取量增加。位置摄取量增加。位置摄取量增加。位置摄取量增加。vvMRI:MRI:Tumor mass arising from C2 vertebral body&Tumor mass arising from C2 vertebral body&compressing on spinal cordcompressing on spinal cord磁脊振显像：有一磁脊振显像：有一磁脊振显像：有一磁脊振显像：有一C2C2颈椎骨肿瘤，并压著脊髓。颈椎骨肿瘤，并压著脊髓。颈椎骨肿瘤，并压著脊髓。颈椎骨肿瘤，并压著脊髓。vvTumor Tumor debulkingdebulking (04BX5833)(04BX5833)performed performed肿瘤主体切除术标本送检。肿瘤主体切除术标本送检。肿瘤主体切除术标本送检。肿瘤主体切除术标本送检。软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells infiltrating Tumor cells infiltrating intertrabecular spaceintertrabecular space肿瘤细胞於骨肿瘤细胞於骨肿瘤细胞於骨肿瘤细胞於骨板间浸润板间浸润板间浸润板间浸润软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells infiltrating Tumor cells infiltrating intertrabecular spaceintertrabecular space肿瘤细胞於骨板间浸润肿瘤细胞於骨板间浸润肿瘤细胞於骨板间浸润肿瘤细胞於骨板间浸润软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells with coagulative necrosisTumor cells with coagulative necrosis肿瘤细胞伴有坏死灶肿瘤细胞伴有坏死灶肿瘤细胞伴有坏死灶肿瘤细胞伴有坏死灶软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells in cohesive sheetTumor cells in cohesive sheet肿瘤细胞成紧密片状肿瘤细胞成紧密片状肿瘤细胞成紧密片状肿瘤细胞成紧密片状软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Epithelioid tumor cells admixedEpithelioid tumor cells admixedwith inflammatory cellswith inflammatory cells上皮样肿瘤细胞混和炎性细胞上皮样肿瘤细胞混和炎性细胞上皮样肿瘤细胞混和炎性细胞上皮样肿瘤细胞混和炎性细胞软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells with pleomorphic nuclei,prominentTumor cells with pleomorphic nuclei,prominentnucleoli&abundant eosinophilic cytoplasmnucleoli&abundant eosinophilic cytoplasm肿瘤细胞含多形性核、巨核仁及大量嗜酸性胞浆肿瘤细胞含多形性核、巨核仁及大量嗜酸性胞浆肿瘤细胞含多形性核、巨核仁及大量嗜酸性胞浆肿瘤细胞含多形性核、巨核仁及大量嗜酸性胞浆软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Polygonal tumor cells with abundant Polygonal tumor cells with abundant eosinophilic cytoplasmeosinophilic cytoplasm多边形肿瘤细胞含大量嗜酸性胞浆多边形肿瘤细胞含大量嗜酸性胞浆多边形肿瘤细胞含大量嗜酸性胞浆多边形肿瘤细胞含大量嗜酸性胞浆软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells with rhabdoid morphologyTumor cells with rhabdoid morphology肿瘤细胞呈横纹肌样形态肿瘤细胞呈横纹肌样形态肿瘤细胞呈横纹肌样形态肿瘤细胞呈横纹肌样形态软组织肿瘤病理学病例讨论四Debulking specimen of vertebral tumorDebulking specimen of vertebral tumor颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本颈椎骨肿瘤主体切除术标本Tumor cells with rhabdoid morphology Tumor cells with rhabdoid morphology(i.e.inclusion-like eosinophilic cytoplasm)(i.e.inclusion-like eosinophilic cytoplasm)肿瘤细胞呈横纹肌样形态，即含包肿瘤细胞呈横纹肌样形态，即含包肿瘤细胞呈横纹肌样形态，即含包肿瘤细胞呈横纹肌样形态，即含包涵涵涵涵体体体体样的嗜酸性胞浆样的嗜酸性胞浆样的嗜酸性胞浆样的嗜酸性胞浆软组织肿瘤病理学病例讨论四Histologic Diagnosis?组织学诊断组织学诊断?软组织肿瘤病理学病例讨论四Histologic Differential Diagnosis组织学鉴别诊断组织学鉴别诊断vvTumor with epithelioid cells Tumor with epithelioid cells 含上皮样细胞肿瘤含上皮样细胞肿瘤含上皮样细胞肿瘤含上皮样细胞肿瘤:-Epithelioid MPNST Epithelioid MPNST 上皮样恶性神经鞘瘤上皮样恶性神经鞘瘤上皮样恶性神经鞘瘤上皮样恶性神经鞘瘤-Epithelioid sarcoma Epithelioid sarcoma 上皮样肉瘤上皮样肉瘤上皮样肉瘤上皮样肉瘤-Epithelioid angiosarcoma Epithelioid angiosarcoma 上皮样血管肉瘤上皮样血管肉瘤上皮样血管肉瘤上皮样血管肉瘤-Epithelioid leiomyosarcoma Epithelioid leiomyosarcoma 上皮样平滑肌肉瘤上皮样平滑肌肉瘤上皮样平滑肌肉瘤上皮样平滑肌肉瘤-Epithelioid synovial sarcoma Epithelioid synovial sarcoma 上皮样滑膜肉瘤上皮样滑膜肉瘤上皮样滑膜肉瘤上皮样滑膜肉瘤-Metastatic carcinoma/mesothelioma/melanoma Metastatic carcinoma/mesothelioma/melanoma 转移性癌、间皮瘤或黑色素瘤转移性癌、间皮瘤或黑色素瘤转移性癌、间皮瘤或黑色素瘤转移性癌、间皮瘤或黑色素瘤软组织肿瘤病理学病例讨论四vvTumor with rhabdoid cells Tumor with rhabdoid cells 含横纹肌样细胞肿瘤含横纹肌样细胞肿瘤含横纹肌样细胞肿瘤含横纹肌样细胞肿瘤:-Rhabdomyosarcoma Rhabdomyosarcoma 横纹肌肉瘤横纹肌肉瘤横纹肌肉瘤横纹肌肉瘤-Meningioma Meningioma 脑膜瘤脑膜瘤脑膜瘤脑膜瘤-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-Desmoplastic small round cell tumor Desmoplastic small round cell tumor 结缔组织增生性小结缔组织增生性小结缔组织增生性小结缔组织增生性小细胞瘤细胞瘤细胞瘤细胞瘤-Extrarenal 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-Lymphoma Lymphoma 淋巴瘤淋巴瘤淋巴瘤淋巴瘤-Myxoid chondrosarcoma Myxoid chondrosarcoma 粘液样软骨肉瘤粘液样软骨肉瘤粘液样软骨肉瘤粘液样软骨肉瘤-Metastatic carcinoma/mesothelioma/melanoma Metastatic carcinoma/mesothelioma/melanoma 转移转移转移转移性癌、间皮瘤或黑色素瘤性癌、间皮瘤或黑色素瘤性癌、间皮瘤或黑色素瘤性癌、间皮瘤或黑色素瘤Histologic Differential DiagnosisHistologic Differential Diagnosis组织学鉴别诊断组织学鉴别诊断软组织肿瘤病理学病例讨论四Epithelioid MPNSTEpithelioid MPNST上皮样恶性神经鞘瘤上皮样恶性神经鞘瘤上皮样恶性神经鞘瘤上皮样恶性神经鞘瘤Sheets of epithelioid tumor cells with glassy cytoplasmSheets of epithelioid tumor cells with glassy cytoplasm成片的上皮样肿瘤细胞含玻璃样胞浆成片的上皮样肿瘤细胞含玻璃样胞浆成片的上皮样肿瘤细胞含玻璃样胞浆成片的上皮样肿瘤细胞含玻璃样胞浆软组织肿瘤病理学病例讨论四Embryonal rhabdomyosarcomaEmbryonal rhabdomyosarcoma胚胎性横纹肌肉瘤胚胎性横纹肌肉瘤胚胎性横纹肌肉瘤胚胎性横纹肌肉瘤Rhabdomyoblasts with cross-striationsRhabdomyoblasts with cross-striations横纹肌母细胞现肌横纹横纹肌母细胞现肌横纹横纹肌母细胞现肌横纹横纹肌母细胞现肌横纹软组织肿瘤病理学病例讨论四