小脑血管母细胞瘤2例临床病理分析
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摘要
目的:探讨小脑血管母细胞瘤(HB)临床影像学表现、病理学特征及免疫组化特点,为鉴别诊断的准确率提供依据。方法:回顾性分析2015年10月至2017年4月诊治的2例HB患者临床资料、影像学表现、病理学特征及免疫组化特点并复习相关文献。结果:2例HB患者均以头痛为首发症状,影像学检查提示小脑半球囊实性和实质性占位,镜下肿瘤主要以大间质细胞和丰富的毛细血管网构成,并根据间质细胞的丰富程度分为细胞亚型,其肿瘤组织内含大量的间质细胞,细胞呈圆形或多角形,胞浆丰满呈空泡状;网状亚型,肿瘤组织内含大量的毛细血管,网织染色示有丰富的网状纤维。HB间质细胞中Vimentin、S-100蛋白、Inhibin-a、CD56表达均阳性且NSE、VEGF高表达,EMA、CD34、CD31、GFAP、EGFR均呈阴性。血管内皮细胞均表达内皮细胞标记物CD34、CD31及FⅧAg。Ki-67增殖指数标记阳性率<5%。结论:HB是临床较为少见的中枢神经系统良性肿瘤(WHOⅠ级),影像学表现具有一定的特异性,结合病理学特征及免疫组化特点,有助于提高诊断的准确率。
Objective: To investigate the clinical imaging findings,pathology feature and immuno histochemistry characteristics of cerebellum hemangioblastoma( HB) and to provide evidence for the accuracy of differential diagnosis. Methods: Retrospectively analyse the clinical data of 2 patients with HB from October 2015 to April 2017 in imaging findings,histopathological features and immunohistochemistry,pathology feature and relevant literature was reviewed. Results: Headache was the first symptom in all patients with HB,and imaging examinations revealed solid cystic and substantial tumor occupancy in the cerebellar hemisphere,microscopically tumors consisted of large interstitial cells and a rich network of capillary vessels,and they were classified into cell subtypes according to the abundance of interstitial cells. The cells were round or polygonal,and the cytoplasm was full showed vacuole-shape. For reticulon subtype,tumor tissue contained a large number of capillaries,reticular fiber staining showed rich reticular fibers.The HB interstitial cells were positive for Vimentin,S-100 protein,Inhibin-a and CD56,and were highly expressed NSE and VEGF. EMA,CD34,CD31,EGFR and GFAP were negative. Vascular endothelial cell expressed endothelial markers CD34,CD31 and FVⅢAg. The positive rate of Ki-67 proliferation index was < 5%. Conclusion: HB is a clinically rare benign tumor of the central nervous system( WHO level Ⅰ). Imaging performance has a certain degree of specificity. Combined with pathological features and immunohistochemical features,it contributes to the accuracy of diagnosis.
Objective: To investigate the clinical imaging findings,pathology feature and immuno histochemistry characteristics of cerebellum hemangioblastoma( HB) and to provide evidence for the accuracy of differential diagnosis. Methods: Retrospectively analyse the clinical data of 2 patients with HB from October 2015 to April 2017 in imaging findings,histopathological features and immunohistochemistry,pathology feature and relevant literature was reviewed. Results: Headache was the first symptom in all patients with HB,and imaging examinations revealed solid cystic and substantial tumor occupancy in the cerebellar hemisphere,microscopically tumors consisted of large interstitial cells and a rich network of capillary vessels,and they were classified into cell subtypes according to the abundance of interstitial cells. The cells were round or polygonal,and the cytoplasm was full showed vacuole-shape. For reticulon subtype,tumor tissue contained a large number of capillaries,reticular fiber staining showed rich reticular fibers.The HB interstitial cells were positive for Vimentin,S-100 protein,Inhibin-a and CD56,and were highly expressed NSE and VEGF. EMA,CD34,CD31,EGFR and GFAP were negative. Vascular endothelial cell expressed endothelial markers CD34,CD31 and FVⅢAg. The positive rate of Ki-67 proliferation index was < 5%. Conclusion: HB is a clinically rare benign tumor of the central nervous system( WHO level Ⅰ). Imaging performance has a certain degree of specificity. Combined with pathological features and immunohistochemical features,it contributes to the accuracy of diagnosis.
引文
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[12]HUANG Changjian,HUANG Wei.Surgical treatment of 85 cases of central nervous system hemangioblastoma[J].Chin J Clin Neurosurg,2016,21(11):694-696.[黄常坚,黄玮.85例中枢神经系统血管母细胞瘤的手术治疗[J].中国临床神经外科杂志,2016,21(11):694-696.]
[13]WANG Zheng,WANG Yanfen,JIANG Shuwan,et al.Clinicopathological observation of 5 cases of hemangioblastoma[J].J Clin Exp Pathol,2017,33(8):915-917.[王正,王艳芬,蒋淑婉,等.血管母细胞瘤5例临床病理观察[J].临床与实验病理学杂志,2017,33(8):915-917.]
[14]Denis Migliorini,Sven Haller,Doron Merkler,et al.Recurrent multiple CNS hemangioblastomas with VHL disease treated with pazopanib:A case report and literature review[J].CNS Oncol,2015(45):92-95.
[15]Betty Kim,Eric Jonasch,Ian Mc Cutcheon.Pazopanib therapy for cerebellar hemangioblastomas in von Hippel-Lindau disease[J].Targeted Oncology,2012(2):36-38.
[2]Gwang Jun Lee,Tae Young Jung,In Young Kim,et al.The clinical experience of recurrent central nervous system hemangioblastomas[J].Clinical Neurology and Neurosurgery,2014,40(2):917-922.
[3]DONG Bing,WANG Changbin.MRI features and pathological character of cerebellar hemangioblastoma[J].J Med Imaging,2012,22(11):1083-1085.[董冰,王长彬.小脑血管母细胞瘤的MRI表现及其病理探讨[J].医学影像学杂志,2012,22(11):1083-1085.]
[4]LIU Yan,GUO Da,JING Zonglin.Intracranial solid hemangioblastoma report of 1 cases[J].Journal of Military Surgeon in Southwest China,2017,19(5):497-499.[刘艳,郭达,敬宗林.颅内实性血管母细胞瘤1例[J].西南军医,2017,19(5):497-499.]
[5]LIU Chong,WANG Zhen,LI Hai,et al.The clinical Pathology observation of cerebellar hemangioblastoma[J].Chinese Journal of Pathology,2016,45(2):113-114.[刘冲,王震,李海,等.小脑血管母细胞瘤临床病理学观察[J].中华病理学杂志,2016,45(2):113-114.]
[6]GUO Liping,LI Wenfei,NIU Chen,et al.The MRI and MRS characteristics of hemangioblastoma[J].Modern Oncology,2016,24(7):1125-1129.[郭丽萍,李文菲,牛晨,等.血管母细胞瘤的MRI及MRS特点分析[J].现代肿瘤医学,2016,24(7):1125-1129.]
[7]SUN Xianyong,LU Shanshan,ZHANG Yongdi.MR diagnosis and pathological analysis of cerebellar hemangioblastoma[J]Chinese Journal of CT and MRI,2015,13(7):15-17.[孙献勇,鲁珊珊,张永娣.小脑血管母细胞瘤MR表现及病理分析[J].中国CT和MRI杂志,2015,13(7):15-17.]
[8]ZHENG Hongwei,PENG Xiaobo,ZHENG Lingyun,et al.MRI features and pathological analysis of intracalvarium hemangioblastomas[J].Chin J Interv Imaging,2017,14(10):613-617.[郑红伟,彭晓博,郑凌云,等.颅内血管母细胞瘤的MRI表现及病理分析[J].中国介入影像与治疗学,2017,14(10):613-617.]
[9]YU Juanhan,SONG Min,QIU Xueshan.Clinicopatholigical analysis of central nervous system hemangioblastoma[J].Modern Oncology,2016,24(4):545-548.[于涓瀚,宋敏,邱雪杉.中枢神经系统血管母细胞瘤临床病理特征[J].现代肿瘤医学,2016,24(4):545-548.]
[10]CONG Yuwei,JIANG Jie,WANG Xuefeng,et al.Pilocytic astrocytoma[J].Chinese Journal of Contemporary Neurology and Neurosurgery,2015,15(2):148-151.[丛玉玮,姜杰,王雪峰,等.毛细胞型星形细胞瘤[J].中国现代神经疾病杂志,2015,15(2):148-151.]
[11]Pierre-Jean Reste,Pierre-Louis Henaux,Xavier Morandi,et al.Sporadic intracranial haemangioblastomas:Surgical outcome in a single institution series[J].Acta Neurochirurgica,2013(6):73-82.
[12]HUANG Changjian,HUANG Wei.Surgical treatment of 85 cases of central nervous system hemangioblastoma[J].Chin J Clin Neurosurg,2016,21(11):694-696.[黄常坚,黄玮.85例中枢神经系统血管母细胞瘤的手术治疗[J].中国临床神经外科杂志,2016,21(11):694-696.]
[13]WANG Zheng,WANG Yanfen,JIANG Shuwan,et al.Clinicopathological observation of 5 cases of hemangioblastoma[J].J Clin Exp Pathol,2017,33(8):915-917.[王正,王艳芬,蒋淑婉,等.血管母细胞瘤5例临床病理观察[J].临床与实验病理学杂志,2017,33(8):915-917.]
[14]Denis Migliorini,Sven Haller,Doron Merkler,et al.Recurrent multiple CNS hemangioblastomas with VHL disease treated with pazopanib:A case report and literature review[J].CNS Oncol,2015(45):92-95.
[15]Betty Kim,Eric Jonasch,Ian Mc Cutcheon.Pazopanib therapy for cerebellar hemangioblastomas in von Hippel-Lindau disease[J].Targeted Oncology,2012(2):36-38.