101例胃肠间质瘤的病理特征及其预后分析

目的 探讨胃肠间质瘤（GIST）的病理特征和影响预后的因素.方法 回顾性分析山西省肿瘤医院2002年1月到2008年12月收治的101例GIST患者的病理资料.结果 本组胃50例（49.5%）,小肠19例（18.8%）,其他部位32例（31.7%）.核分裂像中位数18个/50 HPF.90例（89.1%）患者CD117阳性.1、3、5年生存率分别为75%、62%和57%.单因素分析显示,GIST细胞丰富程度对生存率的影响差异有统计学意义（x2=7.167,P=0.028）.Cox回归分析结果提示,细胞密集（P=0.003）和肿瘤直径大于10 cm（P=0.028）预后差.结论 GIST细胞丰富程度和肿瘤大小是影响患者预后的重要因素.     

腹腔镜手术联合靶向治疗胃肠间质瘤28例疗效分析

目的探讨原发性局限性高危胃肠间质瘤(GIST)经腔镜手术切除联合靶向药物(甲磺酸伊马替尼)治疗的可行性及疗效。方法回顾性分析2013年2月至2018年2月间在武警浙江省总队医院接受腔镜手术的高危的胃GIST(20例)和小肠GIST(8例)患者的临床资料。结果 28例患者均在腹腔镜辅助下完成肿瘤切除手术,术后均接受甲磺酸伊马替尼辅助治疗。根据肿瘤生长浸润部位及方式,行胃局部切除术12例,胃远端部分切除5例,胃全切除3例,小肠部分切除8例。肿瘤直径2.5～11.8(7.7±2.5)cm。手术时间60～180(101.8±28.3)分钟,术中出血量20～120(75.3±24.8)ml。术后胃肠道功能恢复时间2.0～4.0(3.1±0.8)天,术后住院时间5.0～10.0(7.2±1.6)天。术后吻合口出血1例,吻合口瘘2例,经保守治疗后痊愈。术后病理按照改良NIH标准记录,肿瘤直径5cm者2例,直径5～10cm者18例,10 cm者8例,核分裂象数6～10/50 HPF者12例(42.9%),10/50 HPF者16例(57.1%)。免疫组织化学染色显示,CD117阳性率96.4%(27/28),CD34阳性率78.6%(22/28),DOG-1阳性率89.3%(25/28)。所有28例患者术后病理免疫组化均为高度复发风险。所有病例均获随访,术后共随访36个月。随访期间2例(7.1%)患者发生肿瘤复发和(或)转移。结论腹腔镜手术和靶向治疗的结合以及密切随访可以在高危GIST患者中实现长期生存。     

小肠间质瘤预后因素分析

目的探讨小肠间质瘤预后相关因素。方法复阅病理切片(苏木精-伊红染色),重新诊断;两点取材构建组织微阵列,免疫组织化学染色检测CD117、CD34、SMA、Desmin、S-100、Ki-67、P53及bcl-2蛋白等8种抗体表达;单因素分析各变量与患者预后的关系。结果58例患者1、3、5年生存率分别为98.3%、69.7%、50.9%。单因素分析,患者预后仅与肿瘤大小及性别有关(P<0.05)。结论对男性小肠间质瘤患者应加以关注,小肠间质瘤直径大于5cm,应高度警惕其复发转移。     

胃间质瘤70例临床与预后分析

目的 探讨胃间质瘤（GST）的临床特征、生物学表现及预后相关因素.方法 回顾性分析经手术治疗及病理证实的70例GST患者相关临床资料,并对其进行电话和信件随访.结果 （1）全组GST中男女性别比例相当,年龄范围在50 ～ 69岁之间,平均年龄59.1岁,主要临床表现为腹部不适（42.9％）,其次为腹部疼痛（37.1％）和上消化道出血（7.1％）,临床表现无特异性.（2） GST的预后与患者性别、年龄、首发症状、肿瘤原发部位等因素无相关性（P＞0.05）,肿瘤大小、核分裂像计数和恶性潜能分级与预后相关,其中肿瘤的大小（肿瘤最大径＞5 cmvs ＜2cm,HR =21.3,95％ CI：5.9 ～77.0;肿瘤最大径2～5 cmvs＜2 cm,HR =2.3,95％CI：1.2～7.8）和核分裂像计数（核分裂像计数＞10/50 HPF vs≤5/50 HPF,HR=22.5,95％ CI：8.6～58.6;核分裂像计数6～ 10/50 HPF vs≤5/50 HPF,HR=11.1,95％ CI：9.9～12.3）是影响GST预后的独立影响因素.结论 GST中肿瘤的大小、核分裂像计数和恶性潜能分级与预后相关,其中肿瘤的大小、核分裂像计数是影响GST预后的独立影响因素.肿瘤越大、核分裂数越多、恶性潜能分级越高,GST的预后越差.     

胃间质瘤临床诊治和预后分析:附35例报告

目的:探讨胃间质瘤的临床特点,诊治经验和预后。方法:回顾性分析我院诊治的35例胃GIST病人的临床资料,并对临床特点、诊断、治疗以及预后进行报道,探讨该病复发转移的特点及影响预后的因素。结果:术前诊断正确率为68.6%肿瘤,中位直径7.0(1.5～14.0)cm。行胃楔形切除9例,胃部分切除23例,全胃切除3例;术后服用甲磺酸伊马替尼9例。CD117(+)100%,CD34(+)90.9%,Vim(+)79.3%,SMA(+)42.9%,S-100(+)26.9%。术后有6例出现复发转移,其中1例进行了再次手术。2例出现肝转移,3例后腹膜转移,1例腹腔广泛转移;2例死亡,4例带瘤生存。单因素分析发现,肿瘤直径≥5 cm、核分裂象≥5/50 HPF以及高危病人为胃GIST复发转移的预测因素。结论:胃GIST高危病人术后出现复发或转移的可能性大,即使出现复发、转移,也应积极再次手术,同时高危和复发转移者应配合甲磺酸伊马替尼辅助治疗。     

单中心220例小肠肿瘤临床病理学特征和预后因素分析

目的分析小肠肿瘤患者临床病理学特征及预后相关因素。方法采用回顾性观察性研究方法。收集2012年1月至2017年9月期间, 四川大学华西医院胃肠外科行手术切除的原发于空肠和(或)回肠肿瘤患者的临床病理资料。病例纳入标准:(1)年龄≥18岁;(2)接受小肠肿瘤切除手术;(3)发病部位为空肠或回肠者;(4)术后病理证实为恶性肿瘤或具有恶性潜能的肿瘤;(5)临床病理学资料及随访数据完整。排除有恶性肿瘤病史和同时并发其他恶性肿瘤者, 以及仅行小肠肿瘤活检手术而未行小肠肿瘤切除手术者。分析小肠肿瘤患者的临床病理学特征、预后情况及影响预后的相关因素。结果本研究共纳入220例小肠肿瘤患者, 其中136例为小肠胃肠间质瘤(GIST), 47例为小肠腺癌, 35例为小肠淋巴瘤。小肠GIST主要临床表现为消化道出血(61.0%, 83/136)和腹痛(38.2%, 52/136), 淋巴结和远处转移率分别为0.7%(1/136)和11.8%(16/136)。中位随访81.0(75.9～86.1)个月, 3年总生存率(OS)为96.3%。Cox多因素回归分析结果显示, 肿瘤远处转移是影响小肠GIST患者OS的...     

原发性胃肠道间质瘤73例的外科治疗

目的 探讨原发性胃肠道间质瘤(GIST)的外科治疗方法及预后.方法 回顾性分析1997年4月至2007年12月手术切除治疗的73例原发性GIST的临床病理资料和治疗方法,并对其预后进行评价.结果 73例GIST施行肿瘤完全切除者68例(其中12例在腹腔镜下完成肿瘤切除术),行肿瘤不完全切除仅取活检者5例,两组生存率差异有统计学意义(P=0.000).66例获随访的患者1、3、5年总体生存率分别为91.0%、78.2%、74.1%,根据肿瘤直径和核分裂象计数分级的肿瘤恶性程度风险分级与术后生存率密切相关(P=0.002),极低度及低度风险组与高度风险组间生存率差异有统计学意义(P<0.05).结论 应高度重视原发性GIST的初次手术治疗,积极行肿瘤完全切除以提高疗效,对肿瘤恶性程度风险较高者需扩大切除范围,应强调腹腔镜下GIST切除术适应证的选择和肿瘤完全切除.     

胃肠道间质瘤的临床研究

目的探讨胃肠道间质瘤（GIST）临床病理特点、手术治疗及预后。方法对1997年4月至2008年6月间84例手术切除的GIST患者的临床病理资料进行回顾性分析,并评价预后。结果84例GIST位于胃42例,小肠24例,食管5例,直肠6例,胃肠道外7例;肿瘤直径（5．6±4．8）cm,肿瘤组织免疫组织化学检测CD117阳性表达率为96．4％。79例行肿瘤完全切除,5例行姑息切除或肿瘤活检。78例获随访患者1、3、5年生存率分别为92．0％、79．2％和72．0％,根据肿瘤直径和核分裂像计数分级的Fletcher恶性程度风险分级与生存率有密切关系（P〈0．01）,极低度风险组、低度风险组生存率与高度风险组生存率间差异有统计学意义（P值分别为0．003及0．000）。结论GIST的Fletcher分级与患者术后生存率有关。对高度风险者,需积极施行切除范围较大的手术。     

35例胃肠道间质瘤临床诊治分析

目的探讨胃肠道间质瘤(GIST)的临床表现及诊治方法。方法回顾性分析2003～2008年期间我院收治的35例GIST患者的临床表现和随访资料。结果 GIST发生部位:胃22例(62.8%),小肠8例(22.9%),结直肠2例(5.7%),腹膜后或肠系膜3例(8.6%)。免疫组织化学检测显示,CD117(+)32例(91.4%),CD34(+)18例(51.4%),SMA(+)4例(11.4%),S100(+)2例(5.7%)。35例患者均接受了手术治疗,其中根治性手术30例,非根治性手术5例,联合脏器切除10例。随访中位时间34个月,随访率为100%(35/35)。全组患者1、3、5年累积生存率分别为95.4%、87.2%和77.9%。本组患者中有3例术后复发转移患者服用甲磺酸伊马替尼的患者至今仍带瘤生存。患者的5年累积生存率与肿瘤大小、有无肿瘤坏死、核分裂数、肿瘤细胞核异型性、肿瘤部位及手术方式有关(P<0.05)。结论 GIST主要通过术后病理和免疫组织化学检测明确诊断,完整的局部手术切除是最有效的治疗手段,复发转移病例需要手术与药物治疗的结合。     

巨大胃肠道间质瘤39例临床分析

目的:探讨巨大胃肠道间质瘤(GIST)的临床特征、诊断治疗方法及预后。方法:回顾性分析2012年1月—2015年4月间在川北医学院附属医院收治的179例GIST患者资料,其中39例为巨大GIST(直径≥10 cm)。结果:39例巨大GIST患者的发病部位分别为胃部14例(35.6%)、消化道外(肠系膜、网膜、腹膜)10例(25.6%)、回肠7例(17.9%)、空肠4例(10.3%)、十二指肠3例(7.7%)、直肠1例(2.6%);与普通GIST(直径10 cm)比较,巨大GIST患者中核分裂像、包膜破损、多发、胃肠道外GIST、术前贫血及并发症比例明显增高(均P0.05)。39例患者中,单纯性肿瘤切除8例(20.5%),合并胃肠等器官部分切除30例(76.9%),1例(2.6%)肿瘤未能切除;39例巨大GIST均为高危风险度,32例患者获得有效随访,4例(10.3%)术后服用伊马替尼治疗;术后1、2年无进展生存率分别为92.8%、79.6%。结论:巨大GIST可根据临床表现、影像学及病理检查结果诊断,其临床特征表现出较高的危险度,完整肿瘤切除合并胃肠等器官部分切除以及规范的术前与术后靶向治疗是改善患者预后的有效手段。     

Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up

Gastrointestinal (GI) stromal tumors (GISTs), the specific KIT- or PDFGRA-signaling driven mesenchymal tumors, are the most common mesenchymal tumors of the GI tract. This study analyzed 1091 tumors originally classified as smooth muscle tumors of the small intestine (including jejunum or ileum and excluding duodenum), and found that 906 (83%) of these were GISTs. The GIST patients had 55:45 male-to-female ratio with a median age of 59 years (range, 13-94 years). Only 0.6% of tumors occurred before the age of 21 years and 13.6% before the age of 40 years. The tumors varied from 0.3 to 40 cm (median, 7.0 cm) and most commonly presented with GI bleeding or acute abdomen; 18% were incidentally detected. Histologically, the tumors were relatively monotypic with spindle cell (86%), epithelioid (5%), or mixed patterns (9%). Skeinoid fibers were present in 44% of cases, and their presence was associated with a favorable course. Most epithelioid tumors were malignant, and this morphology sometimes emerged from less cellular and less mitotically active spindle cell tumors, suggesting that it represented a transformation. KIT was immunohistochemically detected in 98%, CD34 in 40%, smooth muscle actin in 34%, desmin in 0.2%, and S-100 protein in 14% of the tumors tested. Outcome was strongly dependent on tumor size and mitotic activity, with an overall 39% tumor-related mortality, twice that for gastric GISTs. Only <3% of tumors <5 cm and < or = 5 mitoses/50 HPF metastasized, whereas 86% of tumors >10 cm and >5 mitoses/50 HPF metastasized. In stark contrast to corresponding gastric tumors, tumors >10 cm with mitotic activity < or = 5/50 HPF and those < or = 5 cm with mitoses >5/50 HPF had a high metastatic rate (>50%); tumors >5 cm < or = 10 cm with low mitotic rate had a 24% metastatic rate. The median survival times of patients with low mitotic rate tumors who died of disease decreased by increasing tumor size. KIT exon 11 mutations were detected in 90 cases, exon 9 mutation in 17 cases, and exon 17 mutation in 1 case; the presence of mutation or mutation type was not prognostically significant. There were no PDGFRA exon 12 or 8 mutations. Systematic data on prognosis of small intestinal GISTs of various size and mitotic activity categories can be helpful in management and surveillance of patients with these tumors.     

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??Clinical pathological features and prognosis analysis of 27 patients for gastrointestinal stromal tumor of pelvic floor ZHANG Shu-wei*??GAO Zhi-dong, DAI Rui-ning, et al. *Department of Gastrointestinal Surgery, Peking University People’s Hospital, Beijing 100044, China
Corresponding authors: YE Ying-jiang, E-mail: yeyingjiang@pkuph.edu.cn; GAO Zhi-dong, E-mail: gaozhidong@pkuph.edu.cn
ZHANG Shu-wei and GAO Zhi-dong are the first authors who contributed equally to the article
Abstract Objective To explore clinical features and prognostic factors of GIST of pelvic floor patients. Methods The clinicopathological data of 27 GIST of pelvic floor patients admitted from September 2005 to July 2017 in Peking University People’s Hospital were analyzed retrospectively. The prognosis was evaluated by univariate analysis. Results A total of 27 GIST patients were collected, including 16 males and 11 females. The median age of onset was 52,ranged from 27 to 81 years old. The median tumor size was 3.0 cm. The predominant site of tumor origin was from rectum (17 cases,68.0%), while the posterior regions of prostate and bladder, the posterior vaginal wall, the rectovaginal septum, the anterior sacral region, and the bladder were also seen. The 1-, 3- and 5-year survival rates of all patients were 100.0%,92.9%,73.1%, while 6 cases??25.0%?? of the patients developed recurrence or metastasis. Univariate analysis revealed that the factors impacting the prognosis were tumor size, surgical methods and preoperative infiltration or metastasis. Considering two factors of tumor diameter and mitotic count among 24 patients, 5-year survival rates of the cases with the maximum diameter of the tumor less than 5 cm and the mitotic image less than 5/50HPF were 100.0% vs. 54.7%, respectively,which compared with the other patients,the difference was statistically significant. Conclusion The symptoms of GIST of pelvic floor are dormant and difficult to diagnose. Preoperative imaging examination is helpful in preoperative diagnosis of tumor sources. Tumor size> 5cm or mitotic> 5/50HPF indicates poor prognosis.     

181例胃肠间质瘤的临床病理及预后分析

目的探讨胃肠间质瘤（GIST）的临床诊治经验,分析其病理特征和影响GIST预后的因素,方法回顾性分析1999年1月至2007年12月间经手术治疗的181例GIST患者的临床病理及随访资料,根据FJetcher推荐的生物学行为分级法进行分组,比较不同因素对生存率的影响并进行预后分析。结果本组GIST病变部位：胃107例（59．1％）,小肠51例（28．2％）,结直肠等其他部位23例（12．7％）;伴有肝转移7例。172例（95．0％）术前通过影像学检查明确病灶部位,其中胃镜53．5％（92／172）,CT 34,3％（59／172）,超声内镜或胶囊内镜17．4％（30／172）。瘤体长径0．5～30．0cm不等,平均7．0cm。CD117阳性171例（94．5％）,CD34阳性156例（86．2％）。外科手术完全切除176例,其中合并联合脏器切除26例;姑息性切除或活检术5例。全组患者1、3、5年生存率分别为95．2％、87．9％和78．5％。单因素分析显示,年龄、肿瘤大小、肿瘤原发部位、核分裂像数目、FIetcher分级和是否合并联合脏器切除对生存率有影响（P〈0．05）;多因素分析显示,Fletcher分级和肿瘤大小是影响预后的因素。术后有8例高危患者和3例复发转移患者服用伊马替尼后病情稳定。结论内镜和CT是GIST有效的诊断手段,用FIetcher分级法来判断GIST的生物学行为和预测预后是简单、有效的方法,外科手术仍是目前GIST的主要治疗方法,而结合靶向治疗将成为改善GIST预后的重要手段。     

Surgery and Prognostic Factors for Gastric Stromal Tumor

Morphologic studies of gastric stromal tumors (GSTs) indicate that mitotic counts (MCs) and tumor size are major discriminants predictive of biologic behavior. The purpose of this study is to improve the understanding of GST behavior, including the prognostic factors and surgical treatment of GSTs. A retrospective analysis (1990–1997) of the clinical course for 116 patients with GSTs was completed, with a median follow-up of 43 months. Tumors were categorized as malignant GSTs (n= 17) when the MC was > 5/50 high-power fields (HPF) and the size > 5 cm or as benign GSTs (n= 99) when the MC was ≤ 5/50 HPF and the size ≤ 5 cm, MC ≤ 5/50 HPF and size > 5 cm, or MC > 5/50 HPF and size ≤ 5 cm. None of 99 benign tumors recurred or metastasized, whereas 7 of 17 malignant tumors recurred. MCs had a close correlation with tumor size. Immunohistochemical studies using CD34, smooth muscle actin, S-100 protein, and synaptophysin have shown positive rates of 61%, 33%, 14%, and 3%, respectively. Smooth muscle actin reactivity was more common in the benign tumors (p= 0.046) and synaptophysin reactivity in the malignant tumors (p= 0.010). Univariate analysis showed that the following clinicopathologic factors were potentially related to poor survival of patients: (1) MC > 5/50 HPF (p= 0.0001); (2) severe pleomorphism (p= 0.0062); (3) necrosis (p= 0.0173); (4) marked cellularity (p= 0.0112); (5) presence of ulceration of overlying gastric mucosa (p= 0.0091); (6) tumor size > 5 cm (p= 0.0195); and (7) exogastric growth pattern (p= 0.0344). Tumors with MC > 5/50 HPF and size > 5 cm were found to be strong indicators of an unfavorable prognosis. The type of surgery and tumor site did not affect the prognosis of the patients.     

胃肠间质瘤术后247例预后分析

目的探讨胃肠间质瘤（GIST）手术患者的预后影响因素。方法回顾性分析2003年1月至2012年11月青岛大学医学院附属医院收治的247例GIST术后患者的临床病理资料,采用Log．rank检验和Cox比例风险模型分别对其预后影响因素进行单因素和多因素分析。结果随访1～113（中位26）个月,26例出现复发或（和）转移,18例死亡,1、3、5年生存率分别为94％、91％和83％。单因素预后分析显示,年龄、肿瘤部位、肿瘤大小、核分裂像及肿瘤是否破裂与患者预后有关（均P〈0．01）。对于中、高风险患者,术后服用伊马替尼者5年总体生存率明显高于未服用者（85．7％比81．0％。P〈0．05）。多因素预后分析显示,肿瘤大小（P-0．030,RR=2．248,95％CI：1．081-4．677）、核分裂像（P=0．041,RR=2．220,95％C1：1．032—4．776）和肿瘤是否破裂（P=0．004,RR=5．183,95％CI：1．677—16．017）是影响术后患者预后的独立因素。结论肿瘤大小、核分裂像及肿瘤是否破裂是G1sT术后患者预后的独立影响因素：伊马替尼可改善GIST术后中、高风险患者总体生存。     

腹内胃肠外间质瘤30例临床病理分析

目的研究腹内胃肠外间质瘤(EGIST)临床病理特点及预后。方法复阅1986年7月至2003年6月47例经病理诊断为腹腔或腹膜后平滑肌瘤、平滑肌肉瘤、平滑肌母细胞瘤、许旺细胞瘤和间质瘤患者的组织切片,重新诊断,免疫组织化学染色检测CD117、CD34、SMA、Desmin及S-100 5种蛋白表达,分析其临床病理变量与预后的关系。结果30例患者最终确诊为EGIST。肿瘤位于肠系膜12例,腹膜后8例,小网膜囊6例,其余4例肿瘤病例记载为腹腔来源。肿瘤中位直径12.5(4～30)cm,其中梭形细胞为主型23例,上皮为主型4例,混合型3例。随访中位时间44个月。随访率90%。全组患者1、3、5年生存率分别为79.7%、59.5%和45.4%。单因素分析结果显示.肿瘤位于腹膜后和肠系膜及腹腔、肿瘤直径超过10cm、肿瘤有坏死、核分裂像数目超过5个/ 50HPF、肿瘤细胞异型性和中、低分化的肿瘤,其预后不佳。结论EGIST有其特有行为谱,预后评价除参照GIST的指标外;肿瘤直径超过10cm和肿瘤的生长部位有助于对EGIST预后的判断。     

Exophytic gastrointestinal stromal tumor of the stomach. Report of two cases

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. Most GISTs occur in the stomach (60-70%). Their diagnosis is established immunohistochemically and reveals that the tumor cells are immunoreactive for the antigens CD117 and CD34. The infiltration of the adjacent organs, the large tumor size (>5cm) and the mitosis count of the cells (>5/50 CHPFs) are typical characteristics of GIST malignancy. GISTs rarely infiltrate adjacent organs and usually push them back. The clinical behaviour of GISTs is highly variable and tumor size mitotic rate and location are prognostic determinants.The biologic behaviour of GIST is variable. The majority were previously thought to be benign due to their characteristically bland histopathologic features. However, it is becoming increasingly clear that with long follow-up, virtually all GISTs have the potential for malignant behaviour, even those 2 cm or less with bland histologic features. Thus, it is not appropriate to define any GIST as "benign" per se.We present two cases of exophytic gastric GIST which were recently treated successfully in our Clinic with “wedge” gastric resection. The margins were negative and there was neither rupture of the tumor, nor spillage.     

217例胃肠间质瘤临床分析

目的探讨胃肠间质瘤（GIST）的临床特征、诊治及影响预后的因素。方法回顾性分析2005年1月至2010年9月华中科技大学同济医学院附属协和医院经手术治疗的217例GIST患者的临床病理资料。比较不同因素对患者预后的影响。结果217例患者中男性103例,女性114例,中位年龄55岁。除4例患者因广泛浸润未完整切除外,其余213例均行完整切除,其中35例行腹腔镜手术;48例术后EI服伊马替尼。178例（82．0％）患者获得术后随访,随访时间3。74个月。随访期间有16例（9．0％）发生术后复发和（或）转移,Logistic回归分析显示．肿瘤部位（OR=2．547,95％CI：1．466～4．424）和核分裂像（OR=6．556,95％CI：2．974。14．449）是影响根治术后复发和（或）转移的独立危险因素。随访患者中带瘤生存者5例,11例死于GIST,其中小肠GIST7例,肠道外GIST4例。Cox回归分析显示,核分裂像（RR=2．654,95％CI：1．094～6．438）与复发和（或）转移（RR=32．988,95％CI：3．879～280．529）是GIST患者的独立预后因素。结论肿瘤部位与核分裂像是影响GIST根治术后复发和（或）转移的独立因素,核分裂像与术后复发和（或）转移是影响GIST预后的独立因素。外科手术完整切除联合靶向治疗可使GIST患者获得满意疗效。     

Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors

Occasional glomus tumors display unusual features, such as large size, deep location, infiltrative growth, mitotic activity, nuclear pleomorphism, and necrosis. Although a small number of purportedly malignant glomus tumors have been described, histologic criteria for malignancy in glomus tumors have never been elaborated. The authors studied 52 unusual glomus tumors (retrieved from their consultation files) previously diagnosed as "atypical" or "malignant" by virtue of nuclear atypia, infiltrative growth, or mitotic activity. They evaluated size, depth, growth pattern, cellularity, nuclear grade, number of mitotic figures per 50 high-power fields (HPF), atypical mitotic figures, vascular space involvement, and necrosis to define criteria for malignancy in glomus tumors. Estimated relative risk was calculated and the Fisher exact test was used for statistical analysis. The 27 female patients and the 25 male patients ranged in age from 8 to 83 years (median age, 43 years). The tumors measured from 0.2 to 12 cm (median size, 2 cm) and occurred predominantly in the extremities, in both the superficial (n = 35) and deep (n = 17) soft tissues. Atypical features were usually observed centrally with a rim of benign-appearing glomus tumor. Follow-up information (n = 35; range, 5 months-23 years; mean 5.5 years) showed seven recurrences, eight metastases, and seven deaths from disease. Five-year cumulative metastatic risk increased significantly for tumors with a deep location (p = 0.005), with a size of more than 2 cm (p = 0.004), and with atypical mitotic figures (p = 0.004). Mitotic activity of more than 5 mitoses/50 HPF, high cellularity, the presence of necrosis, and moderate to high nuclear grade approached but did not reach significance. High nuclear grade alone, infiltrative growth, and vascular space involvement were not associated with metastasis. The authors propose the following classification scheme and criteria. Malignant glomus tumor: Tumors with a deep location and a size of more than 2 cm, or atypical mitotic figures, or moderate to high nuclear grade and > or =5 mitotic figures/50 HPF. Symplastic glomus tumor: Tumors with high nuclear grade in the absence of any other malignant feature. Glomus tumor of uncertain malignant potential: Tumors that lack criteria for malignant glomus tumor or symplastic glomus tumor but have high mitotic activity and superficial location only, or large size only, or deep location only. Glomangiomatosis: Tumors with histologic features of diffuse angiomatosis and excess glomus cells. Using this classification scheme, metastasis was observed in 38% of tumors fulfilling the criteria for malignancy. In contrast, metastatic disease was not seen in any specimen classified as symplastic glomus tumor, glomus tumor of uncertain malignant potential, or glomangiomatosis.