CAJ | 학술논문

背景与目的:腺泡状软组织肉瘤颅内转移少见,容易与颅内其他原发性肿瘤混淆.本研究探讨4例颅内转移性腺泡状软组织肉瘤的临床病理特点、鉴别诊断以及预后情况.方法:复习4例颅内转移性腺泡状软组织肉瘤的病理切片及临床资料,并做免疫组化染色及PAS特殊染色,抗体包括Actin、Vimentin、Myosin、Myoglobin、S-100、Desmin、CgA、Syn、NSE、CK.结果:4例患者临床症状均主要表现为头痛及视物模糊等.颅内转移性病变均位于后颅窝,与脑膜关系密切.显微镜下肿瘤细胞胞浆透亮或嗜伊红,核仁明显,呈腺泡状排列,周围绕以血窦样结构.免疫组化染色2例表达Actin、Desmin和S-100,1例Vimentin和NSE弱表达.PAS染色4例均为阳性.1例术后1年内死亡,1例术后27个月死亡,1例30个月死亡,另1例术后已生存半年,目前无肿瘤复发.结论:颅内转移性腺泡状软组织肉瘤多数为转移性,需要与脑膜瘤、黑色素肿瘤、腺泡状横纹肌肉瘤以及副节瘤鉴别.患者预后差,术后5年生存率较低.
배경여목적:선포상연조직육류로내전이소견,용역여로내기타원발성종류혼효.본연구탐토4례로내전이성선포상연조직육류적림상병리특점、감별진단이급예후정황.방법:복습4례로내전이성선포상연조직육류적병리절편급림상자료,병주면역조화염색급PAS특수염색,항체포괄Actin、Vimentin、Myosin、Myoglobin、S-100、Desmin、CgA、Syn、NSE、CK.결과:4례환자림상증상균주요표현위두통급시물모호등.로내전이성병변균위우후로와,여뇌막관계밀절.현미경하종류세포포장투량혹기이홍,핵인명현,정선포상배렬,주위요이혈두양결구.면역조화염색2례표체Actin、Desmin화S-100,1례Vimentin화NSE약표체.PAS염색4례균위양성.1례술후1년내사망,1례술후27개월사망,1례30개월사망,령1례술후이생존반년,목전무종류복발.결론:로내전이성선포상연조직육류다수위전이성,수요여뇌막류、흑색소종류、선포상횡문기육류이급부절류감별.환자예후차,술후5년생존솔교저.
Background and Objective:Metastatic alveolar soft tissue sarcoma (ASTS)of the central nervous system is rare and is easy to be misdiagnosed as other primary tumors of central nervous system. This study was to analyze the clinical and pathological features of four patients with ASTS of the central nervous system and to clarify their differential diagnosis as well as prognosis. Methods:HE slices and clinical data of the four cases were reviewed and immunohistochemical staining was performed. Antibodies included Vimentin,Myosin,Myoglobin,S-100,Actin,Desmin,CgA,Syn,NSE,and CK.Results:All four patients had a skin nodule of the extremities removed previously.Clinical symptoms included headache and sight blurring.The metastatic lesions were located in the posterior cranial fossa,closely associated with the meninges.The tumor cells had clear or eosinophilic cytoplasm and prominent nucleoli,arranged in alveolar structures,which were surrounded by delicate blood sinuses.The immunohistochemical staining results showed that the positive stainings of Actin,Desmin and S-100 were in 2 cases;the weakly positive stainings of NSE and Vimentin were in 1 case;the positive staining of PAS was in all four cases. The follow-up data showed that one case died during one year after surgery,two cases died during three years.The fourth case had half year after operation and had been alive without tumour.Conclusion:ASTS of the central nervous system was mostly metastatic and should be differentiated from other CNS tumors such as meningioma, melonocytic tumor,rhabdomyosarcoma and paraganglioma.Metastatic ASTS of the central nervous system had poor prognosis and the five-year survival rate was low.