腹膜后神经鞘瘤82例临床分析

目的通过临床病例分析,了解腹膜后神经鞘瘤的临床诊治状况,提高鉴别诊断能力.方法对本组病例行临床分析.结果本组82例均行手术治疗,肿瘤全切除60例(占73.2%),次全和大部分切除13例(占15.9%),无法切除9例(占11.0%);术中探查单发肿瘤78例(占95.1%),多发4例(占4.9%).肿瘤多位于脊柱旁,呈囊实性,有包膜,表面光滑,血管丰富,界清,包膜表面可与周围组织粘连.病理结果诊为神经鞘瘤81例(占98.8%),恶性神经鞘瘤1例(占1.2%);在多发肿瘤4例中,2例(占2.4%)诊为双原发,伴随病灶为升结肠腺癌及左上肺鳞癌,术后复发1例.结论腹膜后神经鞘瘤发病率低,术前诊断困难,绝大部分为良性,恶性少见,主要治疗方法为手术治疗,恶性神经鞘瘤对放化疗不敏感,预后差.     

颈部神经鞘瘤44例临床分析

[目的]探讨颈部神经鞘瘤的临床特征、诊断方法及治疗.[方法] 44例颈部神经鞘瘤均行手术切除.术前均行B超及CT检查.[结果]B超诊断符合率为72.7％(32/44).普通CT诊断符合率为81.9％(36/44),CTA诊断符合率为94.4％ (17/18),诊断性穿刺活检阳性率为80.0％(12/15).44例术后随访3个月～9年,39例无复发,1例位于锁骨上三角的肿块3年后复发.1例术后出现霍纳综合征,3个月后消失,1例术后出现声嘶.[结论]颈部神经鞘瘤术前鉴别诊断非常重要;一旦确诊应尽早手术摘除,术中尽可能保护神经功能,避免并发症的发生.     

纵隔神经鞘瘤31例临床分析

目的探讨纵隔神经鞘瘤的临床特点、诊断和治疗方法。方法回顾性分析1995年7月至2008年2月外科治疗的31例纵隔神经鞘瘤的临床资料。结果28例为良性神经鞘瘤（90.3%）,3例为恶性。肿瘤完整切除29例（93.5%）,姑息切除2例。无手术死亡者。术后出现喉返神经损伤1例,上肢深静脉血栓1例。3例恶性神经鞘瘤患者术后生存5～19个月。结论手术是治疗纵隔神经鞘瘤的主要方法,恶性预后不良。     

巨大低度恶性外周神经鞘瘤1例并文献回顾

恶性外周神经鞘瘤(MPNST)是一种起源于外周神经施万细胞或显示神经鞘分化的恶性肿瘤[1],属于WHOⅢ-Ⅳ级;2/3的病例发生于神经纤维瘤病I型的基础上,常见于头颈部。本文报道胸前区巨大低度恶性外周神经鞘瘤伴有色素沉积1例,复习临床病理学资料并进行组织化学及免疫组     

恶性外周神经鞘膜瘤中 TBX2 基因突变及相关蛋白 表达的临床意义

  目的  检测恶性外周神经鞘膜瘤基因组异常并探讨TBX2、CHK2和p53在恶性外周神经鞘膜瘤中的表达及其临床意义。  方法  收集天津医科大学肿瘤医院骨与软组织肿瘤科1991年1月至2011年12月手术切除并经病理证实的恶性外周神经鞘膜瘤组织标本63例。从中选取新鲜且DNA质量合格的肿瘤样本12例,采用第二代测序(next-generation sequencing,NGS) 方法,检测人恶性外周神经鞘膜瘤组织样本基因组异常情况。应用免疫组织化学方法检测63例恶性外周神经鞘膜瘤组织样本中TBX2、CHK2和p53的表达情况。  结果  12例恶性外周神经鞘膜瘤组织样本中,有1例TBX2基因突变。63例恶性外周神经鞘膜瘤组织样本中,TBX2、CHK2和p53的高表达率分别为60.3%(38/63) 、47.6%(30/63) 及30.2%(19/63) 。TBX2的高表达与AJCC分期、复发和转移有显著相关性(P＜0.05) ;TBX2的表达与CHK2的表达呈正相关(r=0.254,P=0.045) ,CHK2的表达与p53的表达呈正相关(r=0.343,P=0.006) 。高表达TBX2、CHK2和p53的无病生存时间及总生存时间均显著低于低表达组(P＜0.05) ,且TBX2、CHK2和p53均为恶性外周神经鞘膜瘤的独立预后因素。  结论  TBX2及其相关蛋白的表达可能在恶性外周神经鞘膜瘤发生发展过程中起重要作用,检测其表达有望为MPNST预后提供理论依据。       

恶性神经鞘瘤10例

目的提高对恶性神经鞘瘤的认识。方法 对该院2005年5月至2010年10月收治的10例恶性神经鞘瘤临床资料、病理免疫特征、治疗及预后进行回顺性分析。结果随访期内,全组患者死亡2例,均为肿瘤相关性死亡：2例发生复发,2例发生肺转移,1例发生颌下淋巴结转移,6例未见复发转移.结论组纵学形态是恶性神经鞘瘤诊断重要依据,免疫组织化学有助于辅助和鉴别诊断。完整手术切除是最有效的治疗方法.     

颅内恶性蝾螈瘤1例并文献复习

恶性蝾螈瘤(malignant triton tumor,MTT)是恶性神经鞘膜瘤的一种亚型,为含有横纹肌肉瘤成分的恶性神经鞘膜瘤.这种含有混合成分的肿瘤由Masson等[1]在1932年首次报道,MTT的发生十分罕见,占全部恶性神经鞘膜瘤不到5%,患者年龄跨度较大,从11个月～80岁(平均35岁)[2].该肿瘤的发生部位十分广泛,据有关文献报道[2-5],常好发于头颈部、躯干和上、下肢,后腹膜、直肠、纵隔和皮下组织等处偶可发生,国内尚未有颅内恶性蝾螈瘤的报道.     

恶性神经鞘瘤25例临床分析

目的:对恶性神经鞘瘤的临床特征进行分析,以期提高对该病的认识。方法:对我院1993年1月~2006年2月收治的25例患者的临床表现、治疗模式及转移复发情况进行回顾性分析。结果:全组男性14例(56%),女性11例(44%),平均发病年龄53.7岁。表现为体表肿物16例(64%),腹腔及盆腔肿物7例(28%),腰背痛、下肢乏力1例(4%),体检发现肺转移结节1例(4%)。病理类型均为梭形细胞恶性肿瘤,S-100阳性者24例(96%),S-100阴性1例(4%)。23例给予手术完整或部分切除,1例未行手术,1例行剖腹探查发现广泛转移而无法切除。6例予术后放疗,2例行术前导管化疗。25例随访时间为7个月~5年,随访期内,4例(16%)发生肺转移,2例(8%)淋巴结转移,7例(28%)局部复发,2例失访,余10例(40%)未见复发及转移。结论:恶性神经鞘瘤可发生于全身各部位,体表肿块为常见症状,确诊依赖病理组织学检查。肿瘤易复发及转移,手术切除是最有效的治疗方法。     

原发性腹膜后神经源性肿瘤的治疗评价

目的探讨腹膜后原发性神经源性肿瘤的治疗.方法腹膜后原发性神经源性肿瘤较少见,本文共报道27例此类患者的治疗过程,并随访25例疗效.结果手术死亡率3.7%,良性肿瘤二年生存率89.4%,恶性肿瘤随访6例中,有5例于术后一年内死亡;共4例肿瘤复发,为神经纤维瘤、恶性神经鞘瘤和交感神经母细胞瘤.结论原发性神经源性肿瘤良性较恶性预后好,恶性肿瘤较易复发.     

椎管内神经鞘瘤的诊断和治疗

目的　探讨椎管内神经鞘瘤的早期诊断和治疗。方法　对 63例椎管内神经鞘瘤进行回顾性分析。结果　本组 63例病程为1个月～ 4年 ,其中 1年内确诊者 3 8例 ,占 60 3 %。全部手术治疗 ,病理证实为椎管内神经鞘瘤。无手术死亡病例。术后全部随访 ,随访时间为 3个月～ 2年 ,痊愈 43例 ;好转 18例 ;无效 2例。肿瘤复发 4例。结论　神经根性疼痛和进行性脊髓受压症状是椎管内神经鞘瘤的主要临床表现 ,磁共振检查可帮助早期诊断 ,手术全切是治疗关键     

CT导向下125Ⅰ粒子植入治疗晚期头颈肿瘤的近期疗效

目的:评价CT导向下¹²⁵Ⅰ放射性粒子植入治疗晚期头颈肿瘤的临床价值。方法:回顾分析2007年10月-2008年8月接受CT导向下¹²⁵Ⅰ放射性粒子植入治疗的10例晚期头颈癌患者的临床资料。采用计算机立体计划系统计算布源,在CT导向下¹²⁵Ⅰ粒子平面插植。手术结束后1-3个月复查CT。结果:10例患者中8例术后5-14天疼痛缓解,有效率80%,2例无效。10例患者局部肿瘤完全缓解8例（80%）,部分缓解1例,无效1例。无明显并发症发生。结论:¹²⁵Ⅰ粒子植入治疗晚期头颈肿瘤近期疗效好,安全性高,创伤小,并发症发生率低。     

头颈部恶性纤维组织细胞瘤21例临床分析

背景与目的;恶性纤维组织细胞瘤是一类组织结构较复杂的多形性肿瘤,其组织来源和是否应定为独立的疾病尚存在争议,既往文献常将其混同于其他类型恶性肿瘤。本文旨在研究恶性纤维组织细胞瘤的临床及病理特点,以探讨其最佳诊疗方法。方法：回顾性分析我科1984年6月至1999年6月收治的经病理检查证实的头颈部恶性纤维组织细胞瘤患者21例的病例资料。结果：本研究的病例中除有1例失访(按死亡计算)外,其余均已随访3年以上,3年生存率为42．9％(9／21)。采用非手术治疗的2例,因就诊时即已相对晚期,疗效不佳,分别在就诊后2个月、5个月死亡;采用手术治疗的19例,6例术后3年内无复发,13例术后2年内局部复发,9例发生转移,其中7例经再次手术,3例已生存3年以上。21例中,12例术后证实有颈淋巴结转移,颈部淋巴转移率为57．1％。结论：头颈部恶性纤维组织细胞瘤是一类复发率较高的恶性肿瘤,治疗以扩大根治术为首选方法,单独放疗或化疗效果不佳。对术后复发的病例,不应放弃再次手术的机会。     

CT导向下125I粒子植入治疗晚期头颈肿瘤的近期疗效

目的：评价CT导向下125I放射性粒子植入治疗晚期头颈肿瘤的临床价值。方法：回顾分析2007年10月-2008年8月接受CT导向下125I放射性粒子植入治疗的10例晚期头颈癌患者的临床资料。采用计算机立体计划系统计算布源,在CT导向下125I粒子平面插植。手术结束后1—3个月复查CT。结果：10例患者中8例术后5—14天疼痛缓解,有效率80％,2例无效。10例患者局部肿瘤完全缓解8例（80％）,部分缓解1例,无效1例。无明显并发症发生。结论：协I粒子植入治疗晚期头颈肿瘤近期疗效好,安全性高,创伤小,并发症发生率低。     

头颈部粘膜恶性黑色素瘤临床分析

 目的　探讨头颈部粘膜恶性黑色素瘤的诊断和治疗方法。方法　回顾性分析64例头颈部粘膜恶性黑色素瘤的病例资料,分组统计预后。结果　手术治疗组5年生存率为20%。以手术为主的综合治疗组为21%,非手术治疗组为0。结论　头颈部粘膜恶性黑色素瘤首选手术治疗。免疫组化病理有助确诊。     

Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal

BACKGROUND: Extraadrenal paragangliomas of the head and neck are tumors with variable clinical behavior. Because tumor growth as well as surgery can cause disabling loss of function, knowledge of the natural history of paragangliomas is important for the development of treatment strategies. METHODS: The tumor volume, growth rate, and tumor doubling time of 48 paragangliomas at different anatomic locations in the head and neck region were estimated retrospectively using sequential radiologic imaging. RESULTS: During a mean follow-up period of 4.2 years, a volume increase of > 20% was observed in 60% of the paragangliomas. In these cases the median growth rate was 1.0 mm/year and the median tumor doubling time was 4. 2 years. More growing tumors were observed in intermediate size tumors than in very small or large paragangliomas, suggesting a biphasic growth pattern. CONCLUSIONS: The majority of head and neck paragangliomas have a very low growth rate. Although management of paragangliomas also is determined by other parameters, preoperative estimation of the tumor doubling time may influence the treatment proposal. A "wait and scan" policy must be considered in all cases.     

Malignant melanoma of the head and neck--a clinical analysis of 46 patients

46 patients with malignant melanoma of the head and neck as treated in our hospital from March 1964 to December 1981 are reported. 29 were male and 17 were female. The sex ratio ws 1.7:1. 56.5% of patients were 40-60 years old. The tumor occurred most frequently in the nasal cavity, next in the oral cavity. Lesions originating from the eye and skin in the head and neck region comprised 15.2% of cases. Melanoma of the nasal cavity and eye rarely metastasized into the cervical lymph nodes but melanoma arising from the skin of head and neck had a metastatic rate of 57.1%. 50% of melanoma of the gingiva metastasized to the neck glands. In this series, 29 patients were treated by operation. The overall 3 and 5 year survival rates of these 46 patients are 38% and 15.7%. The 3 and 5 year survival rates of those without regional lymph node metastasis are 51% and 28%. The 3 and 5 year survivals of those with regional lymph node metastasis are 13% and 0%. Other methods, such as surgery plus chemotherapy, chemotherapy alone and radiotherapy plus surgery or chemotherapy were used only in a few cases which could not be evaluated.     

A CLINICAL ANALYSIS OF 7878 PATIENTS WITH TUMORS IN THE HEAD AND NECK

7878 patients with tumors in the head and neck were analyzed in a period of twenty years. All the diagnosis were confirmed by pathologic examination. There were 5485 cases (69.62%) of malignant tumors. Nearly an half of malignant tumors were in the nasopharynx (49.32%). Among the malignant tumors of the nasopharynx, 2698 were carcinoma (99.34%) and only 18 (0.66%) sarcoma. Nine of them were under 10 years of age, the youngest patient was 3 years old, and this is rare in our county. The incidence of head and neck tumors was 37.66% and that of the head and neck malignancies was 26.22% of tumors in the whole body. This study indicates that incidence of malignant tumor in head and neck was rather higher, it is worth to improve ths procedure of treatment and prevention.     

Iridium afterloading curietherapy in the treatment of pediatric malignancies. The Institut Gustave Roussy experience

Between 1972 and 1980, 58 children with malignant tumors underwent iridium 192 (192Ir) curietherapy at the Institut Gustave Roussy. Data from only 45 of them were reviewed (bilateral retinoblastoma and salvage procedures were excluded). The pathologic diagnosis was rhabdomyosarcoma in 75% of cases, yolk sac tumor in 13% of cases, malignant mesenchymoma in two cases, clear cell carcinoma in two cases, and undifferentiated sarcoma in one case. The two major sites were pelvis-perineum (64.5%) and head and neck (31%). The treatment was essentially based on a combination of chemotherapy, which was adapted to the histologic type of the tumor, and curietherapy. Partial surgery was carried out in certain cases, either before chemotherapy for diagnostic purposes, or after chemotherapy to remove infiltrative residual tumor. External beam radiotherapy was used in a few cases in lymph node irradiation. The radioactive material was 192Ir, using afterloading techniques, either intracavitary or interstitially or both. The tumor dose, calculated by computerized dosimetry, was 60 to 65 Gy delivered over 5 to 7 days in the majority of cases. Two patients were lost to follow-up. Thirty-five patients (78%) are alive with no evidence of disease after a mean follow-up period of 5 years. Eight patients died, one of complications related to chemotherapy and seven of the evolution of their disease. The late-complication rate, studied in 33 patients with a minimum follow-up period of 3 years, is 18% (6/33). Curietherapy seems to be a preferable conservative treatment in children with localized and accessible malignant tumors.     

头颈部肿瘤组织中PD-L1的表达及临床意义分析

目的 探讨头颈部肿瘤组织中细胞程序性死亡配体-1（PD-L1）的表达情况及临床意义。方法 将2015 年1 月至2016年6月收治的40例头颈部恶性肿瘤组织及20例头颈部囊肿组织的手术标本经包埋制作成蜡块,采用免疫组织化学SP二步法检测以上组织中的PD-L1蛋白表达情况,分析PD-L1表达水平与临床病理参数（年龄、性别、病理分化程度和临床分期）的关系。结果PD-L1在头颈部囊肿组织中不表达,头颈部肿瘤组织中的阳性表达率为65.0%（26/40）,高于头颈部囊肿组织,差异有统计学意义（P<0.05）。PD-L1表达与年龄、性别及病理分化程度无关（P>0.05）,而与患者的临床分期有关（P<0.05）。结论 PD-L1在头颈部恶性肿瘤组织中表达升高,可为头颈部恶性肿瘤的免疫靶向治疗提供参考。     

耳鼻颌面部恶性黑色素瘤(附11例分析)

目的：探讨发生于耳鼻颌面部恶性黑色素瘤(恶黑)的诊疗方法。方法：对1986～2001年收治的11例恶黑患的临床资料进行临床病理分析。结果：男性6例、女性5例，年龄6个月～83岁；原发部位：牙龈粘膜2例，外耳道l例，耳廓2例，鼻腔筛蝶窦l例，鼻中隔l例，中耳l例，下睑腮腺l例，上唇2例；手术切除9例，术后化疗2例，术后放疗3例，未治疗2例。0．5～10年随访，死亡6例，生存期6个月～3年；健在5例，生存期1．0—15年。结论：恶黑在皮肤粘膜交界处的色素层或粘膜黑色素斑的基础上发生，肿块多呈黑褐色且常伴溃疡，诊断较易，而对于不含黑色素的恶黑容易误漏诊，须待病理确诊。瘤细胞内含大量黑色素颗粒为其病理特点，免疫组化显示VIM—HMB45和S-100蛋白及Vs38阳性反应均有助于不含色素的恶黑的确诊。本病恶性度极高，可采用手术、放化疗或免疫治疗，但预后差。