骨外间叶性软骨肉瘤2例临床病理观察
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摘要
目的探讨骨外间叶性软骨肉瘤(MC)的临床病理、免疫表型及鉴别诊断。方法对2例MC进行组织病理学及免疫组化观察。结果 2例MC分别位于胰腺和肺。临床表现主要为腹痛和咳嗽、气短。影像学表现为边缘清晰的高密度肿瘤。组织学显示肿瘤由原始未分化小细胞和分化较成熟的透明软骨小岛构成。免疫组化显示未分化小细胞胞膜CD99呈不同程度(+),vimentin(+);软骨S-100蛋白(+)。结论 MC是一种少见的高度恶性肿瘤,结合组织病理形态特征及免疫组化染色对诊断有帮助。
Objective To investigate the clinicopathological and immunohistochemical features of extrskeletal mesenchymal chondrosarcoma and its differential diagnosis. Methods Two cases of extrskeletal mesenchymal chondrosarcoma were studied with histopathological observation and immunohistochemical staining. Results The tumors located at pancreas and lung,clinical features were abdominal pain and cough. Enhanced CT scan revealed sharp-edged high intensity tumors. Microscopically, the tumors were composed of the primitive undifferentiated cells and welldifferentiated hyaline cartilage islets. Immunohistochemically,the primitive undifferentiated cells were positive for CD99 and Vim,the cartilage islets were positive for S-100. Conclusions Mesenchymal chondrosarcoma is a rare and highly malignant tumor; its histopathological features and immunohistochemicalstaining help make a definite diagnosis.
Objective To investigate the clinicopathological and immunohistochemical features of extrskeletal mesenchymal chondrosarcoma and its differential diagnosis. Methods Two cases of extrskeletal mesenchymal chondrosarcoma were studied with histopathological observation and immunohistochemical staining. Results The tumors located at pancreas and lung,clinical features were abdominal pain and cough. Enhanced CT scan revealed sharp-edged high intensity tumors. Microscopically, the tumors were composed of the primitive undifferentiated cells and welldifferentiated hyaline cartilage islets. Immunohistochemically,the primitive undifferentiated cells were positive for CD99 and Vim,the cartilage islets were positive for S-100. Conclusions Mesenchymal chondrosarcoma is a rare and highly malignant tumor; its histopathological features and immunohistochemicalstaining help make a definite diagnosis.
引文
[1]Lichtenstein L,Bernstein D.Unusual benign and malignant chondroid tumors of bone.A survey of some mesenchymal cartilage tumors and malignant chondrobla-stic tumors,including a few malticontric ones,as well as many atypical benign chondroblastomas and chondromyxold fibromas[J].Cancer,1959,12(6):1142-1157.
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[4]杨莹,边莉,潘国庆,等.骨和软组织间叶性软骨肉瘤6例临床病理分析[J].诊断病理学杂志,2013,20(8):449-453.
[5]庞宗国,何兴状,吴兰雁,等.间叶性软骨肉瘤23例临床病理和免疫表型分析[J].中华病理学杂志,2011,40(6):368-372.
[6]Fanburg-Smith JC,Auerbach A,JMarwaha JS,et al.Immunoprofile of mesenchymal chondrosarcoma:aberrant desmin and EMA expression,retention of INI1,and negative estrogen receptor in 22female-predominant central nervous system and musculoskeletal cases[J].Ann Diagn Pathol,2010,14(1):8-14.
[7]Salvati M,Caroli E,Frati A,et al.Central nervous system mesenchymal chondrosarcoma[J].J Exp Clin Cancer Res,2005,24(2):317-324.
[8]Hoang MP,Suarez PA,Donner LR,et al.Mesenchymal chondrosarcoma:a small cell neoplasm with polyphenotypic differentiation[J].Int J Surg Pathol,2000,8(4)291-301.
[9]Wehrli BM,Huang W,de Crombrugge B,et al.Sox9,a master regulator of chondrogenesis,distinguishes mesenchymal chondrosarcoma from other small blue round cell tumors[J].Hum Pathol,2003,34(3):263-269.
[10]Frezza AM,Cesari M,Baumhore D,et al.Mesenchymal chondrosarcoma:prognostic factors and outcome in 113 pationts:a European Musculoskeletal Oncology Society Study[J].Eur J Cancer,2015,5(13):374-381.
[11]Kawaguchi S,Weiss I,Lin PP,et al.Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma[J].Clin Orthop Relat Res,2014,472(3):856-864.
[12]Van Oosterwijk JG,Meijer D,van Ruler MA,et al.Screening for potential targets for therapy in mesenchymal,clear cell,and dedifferentiated chondrosarcoma reveals Bcl-2 family members and TGFβas potential targets[J].Am J Pathol,2013,182(4):1347-1356.
[2]Dowling EA.Mesenchymal chondrosarcoma[J].J Bone Joint Surg Ame,1964,46:747-754.
[3]Nakashima Y,Unni KK,Shives TC,et al.Mesenchymal chondrosarcoma of bone and soft tissue:a review of 111 cases[J].Cancer,1986,57(12):2444-2453.
[4]杨莹,边莉,潘国庆,等.骨和软组织间叶性软骨肉瘤6例临床病理分析[J].诊断病理学杂志,2013,20(8):449-453.
[5]庞宗国,何兴状,吴兰雁,等.间叶性软骨肉瘤23例临床病理和免疫表型分析[J].中华病理学杂志,2011,40(6):368-372.
[6]Fanburg-Smith JC,Auerbach A,JMarwaha JS,et al.Immunoprofile of mesenchymal chondrosarcoma:aberrant desmin and EMA expression,retention of INI1,and negative estrogen receptor in 22female-predominant central nervous system and musculoskeletal cases[J].Ann Diagn Pathol,2010,14(1):8-14.
[7]Salvati M,Caroli E,Frati A,et al.Central nervous system mesenchymal chondrosarcoma[J].J Exp Clin Cancer Res,2005,24(2):317-324.
[8]Hoang MP,Suarez PA,Donner LR,et al.Mesenchymal chondrosarcoma:a small cell neoplasm with polyphenotypic differentiation[J].Int J Surg Pathol,2000,8(4)291-301.
[9]Wehrli BM,Huang W,de Crombrugge B,et al.Sox9,a master regulator of chondrogenesis,distinguishes mesenchymal chondrosarcoma from other small blue round cell tumors[J].Hum Pathol,2003,34(3):263-269.
[10]Frezza AM,Cesari M,Baumhore D,et al.Mesenchymal chondrosarcoma:prognostic factors and outcome in 113 pationts:a European Musculoskeletal Oncology Society Study[J].Eur J Cancer,2015,5(13):374-381.
[11]Kawaguchi S,Weiss I,Lin PP,et al.Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma[J].Clin Orthop Relat Res,2014,472(3):856-864.
[12]Van Oosterwijk JG,Meijer D,van Ruler MA,et al.Screening for potential targets for therapy in mesenchymal,clear cell,and dedifferentiated chondrosarcoma reveals Bcl-2 family members and TGFβas potential targets[J].Am J Pathol,2013,182(4):1347-1356.