儿童肺部原发性肿瘤56例临床分析

目的探讨儿童肺部原发性肿瘤的临床特征、治疗方案及预后。方法 2009—2019年间广州市妇女儿童医疗中心共收治儿童肺部原发性肿瘤患儿56例, 收集其一般资料、影像学资料、病理资料及手术记录等, 进行回顾性分析。结果 56例患儿中, 胸膜肺母细胞瘤28例, 炎性肌纤维母细胞瘤20例, 黏液表皮样癌6例, 血管瘤1例, 硬化性肺细胞瘤1例。临床表现以呼吸道症状为主, 咳嗽26例, 气促17例, 咳血3例。另外, 发热15例, 胸痛3例, 上腹部疼痛2例。肿瘤位于左肺下叶18例, 右肺下叶11例, 左肺上叶10例, 右肺上叶7例, 右肺中叶6例, 肺门4例。56例患儿均行手术治疗, 其中行开胸手术41例, 胸腔镜手术13例, 纤维支气管镜下手术2例。5例Ⅰ型胸膜肺母细胞瘤患儿术后未行化疗, 至随访结束仍存活。5例Ⅱ型胸膜肺母细胞瘤患儿中, 2例术后未化疗, 复发后死亡;3例术后化疗, 至随访结束仍存活。18例Ⅲ型胸膜肺母细胞瘤患儿术后均予IVADo方案化疗, 出现肿瘤复发6例, 远处转移3例, 肿瘤相关死亡8例。20例炎性肌纤维母细胞瘤患儿中, 行楔形切除术13例, 复发5例;行肺叶切除术6例, 复发1例;行纤维支气管镜手术1例, 术后复发。6例黏液表皮样癌患儿中, 行肺叶切除术5例, 行肺楔形切除术1例, 至随访结束均存活。1例血管瘤患儿行支气管镜下肿物切除术, 1例硬化性肺细胞瘤患儿行肺楔形切除术, 至随访结束均存活。结论儿童肺部原发性肿瘤缺乏特异性临床表现, 手术完整切除有利于取得良好预后。对于Ⅱ、Ⅲ型胸膜肺母细胞瘤, 化疗是必要的辅助治疗。     

成人型肺母细胞瘤五例报告及文献复习

背景与目的　肺母细胞瘤是一种少见的肺部原发恶性肿瘤,分为成人型肺母细胞瘤和儿童胸膜肺母细胞瘤。本文主要讨论成人型肺母细胞瘤的临床特点、诊断和治疗。方法　回顾我院从1964 年4 月至2004年3月收治的5例成人型肺母细胞瘤的临床资料,并复习有关国内外文献。结果　5 例中男3 例,女2例;发病年龄22~70岁;以咳嗽、咳血丝痰为主要症状,次为胸闷、胸痛;影像学主要表现为肺部单发实性肿块,纤维支气管镜检查及痰细胞学检查均没有发现肿瘤细胞。3 例行肺叶切除加肺门纵隔淋巴结清扫术,1例行左全肺切除加肺门纵隔淋巴结清扫术,1例行肺楔形切除术并行术后化疗。到随访结束,3 例死亡,2 例生存,生存期最短6个月,最长11年。结论　肺母细胞瘤术前难明确诊断。治疗以肺叶切除加纵隔淋巴结清扫术为主,对放疗及化疗不敏感,预后差别较大。     

全胸腔镜下手术治疗26例早期肺癌的体会

目的探讨全胸腔镜下手术治疗早期肺癌的安全性、可行性及手术适应症。方法所有患者均在3个微小切口非直视下行解剖性肺叶切除,同时行淋巴结清扫术。行左肺上叶切除术5例,左肺下叶切除术7例,右肺上叶切除术6例,右肺中叶切除术3例,右肺下叶切除术5例。结果术中无中转开胸、无二次手术、无输血、无严重并发症及围手术期死亡者。手术时间为（163.07±20.83）min,出血量（255.77±51.83）ml,术后引流量（348.04±39.13）ml,术后住院时间（9.96±0.82）天。术后随访6～12个月无复发。结论全胸腔镜下肺叶切除术是1种安全、有效、微创的术式,适用于早期肺癌患者。     

肺癌胸膜全肺切除手术技术探讨

[目的]探讨肺癌伴胸膜转移恶性胸水行胸膜全肺切除术手术技术和术中处理体会。[方法]1988年1月至2003年1月对21例肺癌伴胸膜转移恶性胸水患者行胸膜全肺切除术。肺部原发病灶位于左肺8例，右肺13例。其中鳞癌1例，腺癌12例，腺鳞癌2例，肺泡细胞癌6例。采用胸膜外径路整块切除病变，低能混切电刀分离壁层胸膜，尤其注重心包、纵隔大血管表面胸膜、膈胸膜的切除；对确无法彻底切除的少量残余癌灶，局部反复电刀烧灼；心包表面有转移病灶未行心包切除者常规行心包开窗；关胸前以大量蒸馏水反复冲洗胸腔及切口，氮芥留置胸腔。[结果]无围手术期死亡，无支气管胸膜瘘、脓胸、大出血等严重并发症。随访已死亡16例，生存期为5～34年月，其余5例患者随访24～29个月仍生存，全组中位生存时间18个月，死亡原因均为远处转移，无局部肿瘤复发。[结论]Ⅲb期肺癌病人有选择地行胸膜全肺切除术是安全可行的，改良手术技术可以减少手术并发症、降低肿瘤局部复发机会，采用该术式再结合综合治疗，能获得明显延长生存时间的良好效果。     

电视胸腔镜治疗胸部肿瘤11例报告

作者自1996年6月以来,采用电视胸腔镜手术治疗胸部肿瘤。共治疗11例,其中良性肿瘤4例,恶性肿瘤7例。行胸腔镜肺切除术2例,肺楔形切除术7例,胸膜活检及固定术2例,手术均获成功,效果良好,无严重并发症,文中重点介绍了胸腔镜肺叶切除术、胩楔形切除术、胸膜活检及固定术。胸膜粘连的手术处理方法。结果显示,电视胸腔镜治疗肿瘤具有手术创伤小、出血少,病人恢复快、效果好的特点。为胸部肿瘤的治疗开拓了广阔的前景。     

17例原发性肝癌自发性破裂的诊断与外科治疗

目的：探讨原发性肝癌自发性破裂出血的诊断和治疗。方法：回顾性分析我院7年来收治17例原发性肝癌自发性破裂出血行肝叶或非规则性肝叶切除术的临床资料。其中肝叶切除术加门静脉、肝动脉置化疗泵双化疗7例；单纯经周围静脉化疗6例；术后行一周期化疗3例。结果：1例于术后一周转回当地医院治疗。术后随访15例，生存期10个月～1年41．2％，1．2～2．3年53％，3．4年5．9％。结论：肝叶或非规则性肝叶切除术对原发性肝癌自发性破裂出血是有效的止血方法和达到肿瘤根治的治疗效果。而术中同时进行门静脉和肝动脉置化疗泵共术后定期化疗可以预防复发和延长患者的生存期。     

肺母细胞瘤2例报告及免疫组化观察

肺母细胞瘤是一种罕见的肺原发性恶性肿瘤 ,以上皮组织和间叶组织混合存在为特征 ,因组织学上类似假腺腔期的胎儿肺 ,故也有人称之为“肺胚胎瘤”。自Ｂａｒｒｅｔ和Ｂａｒｎａｒｄ首次报道以来 ,对肺母细胞瘤的组织学及超微结构有所了解 ,但其免疫组化特征的报道较少。作者对 2例肺母细胞瘤进行免疫组化观察并追踪随访 ,现报告如下。例 1,患者女性 ,2 2岁 ,因低烧伴右胸痛 10个月入院。胸片示右肺中叶 9ｃｍ× 5ｃｍ的团块状阴影 ,边缘清晰 ,右叶肺不张。支气管镜发现 :右中叶支气管开口狭窄。术中见右肺中叶 8ｃｍ× 6ｃｍ× 5ｃｍ的椭圆…     

腮腺肿瘤手术20例临床分析

目的探讨腮腺肿瘤的手术方式、手术范围及预后。方法对1998--2004年间行解剖面神经的腮腺肿瘤切除术20例临床随访资料进行分析。结果腮腺浅叶切除术13例中,并发腮瘘1例;全腺叶切除术7例中,并发暂时性面瘫1例,无Frey综合征。随访10个月至6年无复发。结论腮腺良性肿瘤需行解剖面神经的浅叶及肿瘤切除术或全腮腺切除术;恶性肿瘤在面神经未受累时行保留面神经的腮腺全切术,术后辅以放疗,可以减少肿瘤复发和面瘫等并发症。     

原发性肺淋巴上皮瘤样癌21例手术患者临床分析

背景与目的 原发性肺淋巴上皮瘤样癌(lymphoepithelioma-like carcinoma,LELC)是一种少见的肺部肿瘤,为肺大细胞癌的一个亚型.本研究回顾性分析LELC患者的临床特征、手术方法 、实验室指标、术后放化疗及预后.方法 回顾性分析胸科医院2004年-2008年间原发性肺淋巴上皮瘤样癌手术患者21例,对其临床资料及预后进行临床分析.结果 21例LELC术后患者中行肺叶切除术15例,楔形切除术4例,全肺切除术1例,袖形切除术1例.术后接受化疗12例,接受放疗3例.电话随访至2009年4月31日为止,共4例患者死亡,中位生存时间(median survival time,MST)为49个月.结论 LELC是一种罕见、独特类型的肺癌,经手术及术后辅助放化疗后有较好的预后.     

经自制硬直镜行肺肿瘤切除术--附22例报告

目的 :介绍一种新的微创技术———经自制硬直镜行肺肿瘤切除术。方法 :总结分析 1993年 3月～ 1999年 3月采用本法完成的肺楔型切除术 18例 ,左肺下叶切除术 1例 ,右肺中下叶切除术 1例 ,右肺下叶背段切除术 1例 ,纵隔肿瘤切除术 1例的临床资料。结果 :手术切口长 4～ 8cm ,手术时间 15～30min ,术中出血 2 0～ 50ml ,均未输血 ,无术后并发症 ,术后平均住院时间 8.5天 (不包括肺叶切除者 )。结论 :与标准剖胸手术相比 ,本术式具有创伤小 ,手术时间短 ,术中出血少 ,无需输血 ,无术后并发症 ,术后切口疼痛轻 ,下床活动早 ,术后住院时间短等优点 ,可作为肺良性肿瘤手术治疗的常规术式 ,也可应用于肺恶性肿瘤减瘤术     

Bronchoscopically-guided conformal radiation therapy for radiographically occult lung carcinoma.

Radiographically occult lung carcinoma has a very good prognosis after complete surgical resection. In medically inoperable patients three-dimensional conformal radiation therapy cannot be performed, as computed tomography scan images fail to localize the disease. Presented here is an original technique of marking radiographically occult tumors by fiberoptic bronchoscopy, applied on four patients. No short-term complications were recorded. All the patients are alive, with no evidence of disease, after a mean follow-up of 15 months.     

Primary Pleuropulmonary Neoplasms in Childhood: Fourteen Cases from a Single Center

Background: We aimed to review clinical characteristics, treatment results and outcome of pediatric patientswith primary pleuropulmonary neoplasms. Methods: Medical records of 14 cases diagnosed between 1972-2009were reviewed retrospectively. Results: The male/female ratio was 5/9 and the mean age at diagnosis was 9.1years (2-16). All but one were symptomatic, presenting with fever, coughing, dyspnea, or weight loss. One patientpresented with hemoptysis, and another with digital clubbing. One mesothelioma was diagnosed incidentally.Some 8/14 patients were initially diagnosed as having pneumonia (median delay in diagnosis of 2.5 months).Diagnoses included pleuropulmonary blastoma (PPB, n=5), inflammatory pseudotumor (n=3), mesothelioma(n=2), mucoepidermoid carcinoma (MEC, n=2), and carcinoid tumor (n=2). Patients with PPB underwent surgeryand received chemotherapy ± radiotherapy. Two carcinoid tumor cases underwent surgery, one further receivedchemotherapy. Patients with mesothelioma were treated with chemotherapy. Inflammatory pseudotumors wereall resected. Two cases with MEC received chemotherapy, one after surgery. 2/5 PPB patients survived withoutrecurrence, 3 died; all carcinoid tumors and inflammatory pseudotumors were alive; 1/2 MEC patients wasalive after 252 months, the other one was lost without disease; 1/2 mesothelioma patients was alive withoutdisease, the other was died. For all cases, median follow-up was 30.5 months (0.6-252). Conclusions: Primarypleuropulmonary tumors are rare but clinical presentation can be varied and delay in diagnosis is common.Children with persistent coughs, recurrent pneumonia or hemoptysis should be considered as indicators forearly diagnosis, very important because the prognosis of these tumors varies with histology and stage.     

同时支气管肺动脉联合成形术治疗中央型肺癌

背景与目的：近年来支气管肺动脉联合成形术愈来愈广泛的应用于肺功能不佳的肺癌患者中，该术式符合最大限度地切除肺癌和最大限度地保留肺功能的肺癌手术原则，该项手术技术有着很好的前景。我们总结近年来采用支气管肺动脉联合成形术治疗中央型肺癌的体会来探讨该项手术的应用指征和方法。方法：1993年12月-2003年12月，对63例中央型肺癌施行支气管肺动脉联合成形肺叶切除，包括支气管肺动脉双袖状切除术43例，其中右肺上叶袖切＋肺动脉袖切20例；左肺上叶袖切＋肺动脉袖切23例；切除肺动脉的长度为1．5～4．5cm，平均2．5cm．支气管肺动脉双禊形切除术3例；其中右肺上叶禊切＋肺动脉楔切2例；左肺上叶楔切＋肺动脉楔切1例。支气管袖切并肺动脉禊切17例，其中右肺上叶袖切＋肺动脉楔切5例；左肺上叶袖切＋肺动脉禊切12例。结果：本组无死亡及吻合门瘘发生：结论：支气管肺动脉联合成形术使肺癌手术指征扩大，符合最大限度地切除肿瘤及最大限度保留肺功能的肺癌手术基本原则，是一种安全、有效、可行的术式。     

Endobronchial metastases secondary to solid tumors: report of eight cases and review of the literature

Endobronchial metastases (EBM) secondaries to extrapulmonary solid malignant tumors are rare. Breast, colon and renal adenocarcinomas are the most frequent tumors associated with EBM. Since 1990 we have treated eight patients with EBM secondary to renal adenocarcinoma (three cases), colon adenocarcinoma (two cases), gastric adenocarcinoma (one case), bladder carcinoma (one case) and basal cell carcinoma (one case). Endobronchial lesions were detected by bronchoscopy and their metastatic nature was confirmed histopathologically in all eight cases. We also conducted a review of EBM reporting studies published in English language. The median interval from the diagnosis of the primary tumour was 41 months. Symptoms and radiological findings were indistinguishable from those of primary lung cancer. Five patients were treated with external radiotherapy with symptomatic improvement while two patients had chemotherapy and one patient underwent surgical resection of the metastasis. Systemic treatment was used in six cases with no significant effect on EBM. Median survival after EBM diagnosis was 9 months with one patient surviving 3.5 years and two patients still alive at 1 year. In conclusion, EBM usually represent a late manifestation requiring differential diagnosis from a primary lung cancer. Local treatment may result in symptomatic improvement but prognosis is generally poor averaging 1-2 years in most series.     

Gastrointestinal stromal tumors of the stomach. A ten-year surgical experience

Gastrointestinal stromal tumors show an increasing incidence. Immunohistochemistry is mandatory to make differential diagnosis with other mesenchimal tumors. We retrospectively reviewed 15 primary stomach GISTs operated during the last decade. Gastroscopy, Ultrasonography and CT scan were employed to obtain the diagnosis. Tumor size ranged from 1.5 to 30 cm in diameter. Treatment consisted of curative surgical resection without sistematic lymph node dissection. A wedge resection was sufficient in 8 cases. In 2 patients a distal subtotal gastrectomy was required and in 1 a total enlarged gastrectomy with pancreaticosplenectomy was performed. 4 GISTs were incidentally discovered and removed during surgical procedures for other gastrointestinal malignancies. In 4 cases a laparoscopic wedge resection was possible. In all cases postoperative course was uneventful. No adjuvant treatment was administered. Concerning the follow-up, two patients died for local and distant relapse while 13 are still alive (most of them operated during the last three years). GISTs show a very unpredictable clinical course and curative surgery is the only potential effective curative treatment.     

CT仿真支气管内窥镜在中央型肺癌诊断中的应用

目的：评价螺旋CT仿真支气管内窥镜（CTVB）在中央型肺癌诊断中的作用。方法对45例经纤维支气管检查和病理证实的气管、支气管肿瘤患者进行CT仿真内窥镜检查，包括中央型肺癌33例、肺癌术后8例，肺癌放疗后2例及气恶性肿瘤2例。用新型螺旋CT机对患者进行扫描后，将数据建成CTVB图像进行分析，并与纤维支气管镜进行对照，24例与病理标本进行对照。结果CTV显示了100％的段以上支气管和80％以上的亚段支气管，肿瘤呈块状或结节状，引起管腔狭窄或闭塞。肺癌术后正常的支气管残端呈光滑的盲端，肿瘤复发呈结节状突出，与纤维支气管镜所见基本一致，肺癌放疗后支气管腔轻度凹凸不平及管腔狭窄，结论CTVB是一种新的无创性观察气管、支气管腔的方法、酷似纤维支气管镜所见，结合其他三维重建功能，能同时显示管壁的厚度和肿瘤向管腔外侵犯的范围，但与纤维支气管镜相比，不易观察支气管黏膜的表浅病变且不能活检。     

肺黏液表皮样癌1O例临床分析

目的回顾病理证实的肺黏液表皮样癌（pulmonary mucoepidemoid carcinoma,PMEC）临床资料,结合文献分析,为PMEC的诊治提供参考。方法全部病例均行胸部X线、CT和纤维支气管镜检查,近期2例行符合线路显像（SPECT）检查。肿瘤分化程度的确定参照WHO关于涎腺肿瘤的分类,分为高分化（4例）、中分化（2例）、低分化（4例）。结果10例中男6例,女4例;年龄18—62岁,平均43岁。其中7例有临床症状。9例行手术治疗,无手术相关死亡。4例低分化者中2例肺门淋巴结有转移,细胞异型性明显;4例高分化者中1例有肺门淋巴结转移。高、中分化者共6例,术后随访3-15年均存活,未发现复发与转移。低分化4例中1例确诊时广泛转移,未经治疗,3个月后死亡;另3例手术治疗,其中1例确诊时已出现肾上腺转移,行左。肾上腺转移瘤切除,左肺下叶切除＋纵隔淋巴结清扫,术后化疗6个周期,随访8个月,无复发、转移;另2例肺叶切除术后分别随访25和37个月,均存活。结论PMEC起病隐匿,易误诊、漏诊,诊断依赖病理检查。PMEC可见远处转移。建议常规行肺门淋巴结清扫,并至少对纵隔淋巴结进行采样。     

Treatment and prognosis of patients with intracranial nongerminomatous malignant germ cell tumors: a multiinstitutional retrospective analysis of 41 patients

BACKGROUND: The relative roles of surgical resection, radiotherapy, and chemotherapy in the management of patients with intracranial nongerminomatous malignant germ cell tumors have been controversial. The authors retrospectively investigated the results of different treatment regimens in patients with these tumors. METHODS: The records of 41 patients who were treated between 1981 and 2001 were reviewed. They were grouped into patients with a good prognosis (n=3), an intermediate prognosis (n=24), and a poor prognosis (n=14) based on the histology of their tumors. Fifteen patients (37%) underwent surgical resection and received radiotherapy, and 26 patients (63%) also received chemotherapy. The median follow-up of 18 patients who remained alive was 61 months (range, 14-194 months). RESULTS: The 5-year actuarial overall survival rates for patients in the good prognosis, intermediate prognosis, and poor prognosis groups were 100%, 68%, and 8%, respectively. In the analysis, histology alone had a statistically significant impact on overall survival (P<0.0001). All 3 patients in the good prognosis group were treated successfully with surgical resection and radiotherapy. In the intermediate prognosis group, the 5-year actuarial overall survival rate was 44% for patients who underwent surgical resection and received radiotherapy (n=9) and 84% for patients who also received chemotherapy (n=15; P=0.01). Patients in the poor prognosis group who underwent surgical resection and received radiotherapy (n=3) or who underwent incomplete resection and received both radiotherapy and chemotherapy (n=8) all died of disease, whereas 2 of 3 patients who underwent macroscopic total resection and received both radiotherapy and chemotherapy survived free of disease. CONCLUSIONS: The treatment of patients with intracranial nongerminomatous malignant germ cell tumors should be based on tumor histology. For patients who had a good prognosis (mature teratoma with germinoma), surgical resection and radiotherapy were sufficient; however, for patients in the intermediate prognosis group, multimodal treatment, including surgical resection, radiotherapy, and chemotherapy, was effective. Conversely, for patients in the poor prognosis group, more intensive multimodal treatment, including macroscopic total resection, may improve the survival rate.