纵隔生殖细胞瘤临床分析

目的:分析生殖器外纵隔生殖细胞瘤诊断和影响预后的因素。方法:65例纵隔生殖细胞瘤均行开胸手术治疗。单纯完全摘除肿瘤59例;姑息性切除1例;肿瘤摘除联合肺叶切除或胸膜纤维板剥脱术3例;开胸探查2例。恶性生殖细胞瘤术后均行辅助放、化疗。结果:良性畸胎类肿瘤手术摘除或合并肺、胸膜切除后效果良好。恶性生殖细胞瘤,尤其是精原细胞瘤切除后,辅助放、化疗仍可获得较好的远期生存。3年生存率66.7%。结论:纵隔生殖细胞瘤是常见的纵隔肿瘤,诊断后积极手术治疗可获得较好的结果。     

影响儿童颅外恶性生殖细胞瘤生存率的临床因素分析

目的:对影响儿童颅外恶性生殖细胞瘤生存率的临床因素进行分析.方法:随访40例儿童颅外恶性生殖细胞瘤患者,对不同发病部位、Brodeur分期、血清AFP水平及不同化疗方案分别进行生存率和统计学分析.结果:40例中,中位生存期3年2个月,2年无瘤生存率55.00%;不同原发部位似对生存率无影响;未成熟畸胎瘤的生存率高于含内胚窦瘤成分肿瘤,Ⅰ、Ⅱ期生存率高于Ⅲ、Ⅳ期;诊断时血清AFP正常的病例2年无瘤生存率高于AFP升高者;铂类化疗方案较其它方案明显提高了2年无瘤生存率.结论:随着化疗的进展,儿童颅外生殖细胞瘤尤其是恶性生殖细胞瘤的生存率有很大的提高,组织学类型、临床分期、治疗前AFP水平和化疗方案的选择是影响生存率的重要因素.     

肺原发恶性纤维组织细胞瘤20例

背景与目的肺原发恶性纤维组织细胞瘤是一种罕见的肿瘤.本文研究肺原发恶性纤维组织细胞瘤的临床特点及预后影响因素.方法回顾性分析肺原发恶性纤维组织细胞瘤20例的临床特点、治疗方式及生存情况等资料,采用SPSS 16.0统计学软件,用Kaplan-Meier法及COX回归分析性别、肿瘤大小、肿瘤位置及化疗对术后生存期的影响.结果肺原发MFH的临床症状主要有咳嗽咳痰、痰中带血、胸痛、发热和胸闷.本组患者1年生存率为55.0%,2年生存率为25.096,死亡原因多为局部复发及远处转移.肿瘤的大小和位置是肺原发MFH总生存率的独立预后因素.结论肺恶性纤维细胞瘤恶性程度高,预后差.临床治疗以手术为主,术后辅助化疗疗效尚不确切,肿瘤大小及位置和预后相关.     

26例原发性纵隔恶性生殖细胞瘤的诊治

目的:探讨原发性纵隔恶性生殖细胞瘤的诊治及外科手术的作用.方法:对26例收治的原发性纵隔恶性生殖细胞瘤的临床资料进行回顾性分析.结果:22例手术治疗患者中,11例根治性切除,10例姑息性切除,1例探查,手术并发症发生率及死亡率分别为18.2%和9.1%,其中12例术后给予以顺铂为主的联合化疗,4例予以放疗.手术治疗患者术后病理为无性生殖细胞瘤12例,精原细胞瘤5例,未成熟畸胎瘤5例.3例未成熟畸胎瘤及1例胚胎癌患者明确诊断后未手术而给予放疗或放、化疗.本组26例患者中仅2例精原细胞瘤生存满5年,17例已证实死亡,除2例手术死亡外均死于肿瘤复发转移.结论:原发性纵隔恶性生殖细胞瘤的治疗应强调以化疗为主的综合治疗,外科切除只宜做为阶段性的辅助手段,手术时机把握应以具体患者情况而定.     

原发恶性纵隔生殖细胞瘤的治疗与预后

目的:探讨纵隔原发恶性生殖细胞肿瘤的临床特征、治疗及预后.方法:回顾性分析四川大学华西医院2008年至2013年收治的生殖细胞瘤患者,筛选纵隔原发恶性生殖细胞肿瘤,采集临床病理资料分析预后.结果:经病理确诊生殖细胞瘤患者共1 523例,原发于纵隔163例,其中恶性14例,包括精原细胞瘤6例,非精原细胞瘤8例.病人接受手术、放疗及化疗的综合治疗.精原细胞瘤患者生存时间优于非精原细胞瘤患者(P=0.015).结论:精原细胞瘤患者生存优于非精原细胞瘤患者.手术对纵隔原发恶性GCT的价值仍难以断定,需要仔细评估疾病的具体情况.放化疗的综合治疗应当是此类病人的主要治疗模式.     

10例肺原发恶性纤维组织细胞瘤临床分析

目的研究肺原发恶性纤维组织细胞瘤的治疗方法及其预后。方法回顾性分析经手术病理证实的肺原发恶性纤维组织细胞瘤10例,其中5例单纯手术切除,3例术后放射治疗,2例术后化疗。结果10例患者中,6例生存1年以上,其中4例生存均超过2年,且3例超过3年。1年生存率为60%,2年生存率为40%。局部复发1例,远地转移1例,局部复发加远地转移3例,复发时间1～18个月。结论肺原发恶性纤维组织细胞瘤的预后较差,主要治疗手段为外科手术,术后放射治疗和化疗的疗效尚不肯定。     

原发于睾丸和纵隔生殖细胞恶性肿瘤预后因素分析

[目的]探讨原发于睾丸和纵隔生殖细胞恶性肿瘤的临床特征、生存率及预后影响因素。[方法]回顾性分析1990至2000年收治的22例原发于睾丸和19例原发于纵隔的生殖细胞肿瘤患者的临床资料，以无疾病进展时间和总生存率为主要观察目标，进行预后评估。[结果]睾丸和纵隔生殖细胞肿瘤患者无疾病进展时间分别为62个月和19个月，10年总生存率分别为60．0％和13．3％（P=0．019）。Cox比例风险模型多因素分析结果显示：病理类型和临床分期是影响预后的主要因素。[结论]睾丸生殖细胞瘤患者的预后明显高于纵隔生殖细胞瘤患者，需要进一步探讨如何提高纵隔生殖细胞瘤的治疗效果。     

100例神经母细胞瘤临床预后分析

目的:探讨神经母细胞瘤(Neuroblastoma,NB)的临床特征、治疗方法及预后.方法:回顾性分析1995年4月至2009年8月天津医科大学附属肿瘤医院收治的并且随访资料完整的100例神经母细胞瘤患者的临床资料,比较不同因素对预后的影响.结果:中位随访期26.6个月(2～161个月);末次随访时CR 33例(33%),中位CR时间为14.4个月;带瘤生存病情稳定20例,总生存率(SR)53%.全组1、2及5年总生存率分别为93.5%、81.5%及33.6%.单因素分析发现:肿瘤是否原发纵隔、INSSⅢ+Ⅳ期、NSE>100μg/L、LDH>700U/L、骨髓以外转移、手术方式、病理类型为影响预后的因素.多因素分析显示:纵隔以外原发、是否完全切除肿瘤及LDH>700U/L为独立的预后影响因素.结论:原发于纵隔的神经母细胞瘤患者预后较其他部位好;神经母细胞瘤临床应采取手术、放疗及化疗为主的综合治疗,手术完整切除肿瘤可明显改善其预后;检测血清LDH对评估NB患者预后具有重要的意义.     

神经母细胞瘤37例临床分析

背景与目的:神经母细胞瘤是儿童的常见肿瘤,预后差。本研究探讨神经母细胞瘤(neuroblastoma,NB)的临床特征、治疗方法及预后。方法:对1998年6月至2007年11月中山大学肿瘤防治中心收治的37例神经母细胞瘤患者的临床表现、治疗方法、治疗疗效及不良预后因素进行分析;采用Kaplan-Meier法计算全组生存率,组间比较用log-rank检验,并采用Cox模型进行多因素分析。结果:37例患者中男女比例为2.7∶1,中位年龄4岁;常见原发部位为腹膜后及肾上腺(78.4%)、纵隔(16.2%)、颈部(2.7%)、盆腔(2.7%);常见首发症状为发热(54.0%)、发现肿块(48.6%)、局部疼痛(45.9%);临床分期:Ⅱa期(5.4%)、Ⅱb期(2.7%)、Ⅲ期(24.3%)、Ⅳ期(67.6%);全组患者中19例行综合治疗,其中8例行手术+化疗+放疗,10例行手术+化疗,1例行化疗+放疗;全组1年、2年及5年总生存率分别为66%、40%及11%。单因素分析显示,综合治疗、手术方式、放疗、化疗疗程数(≥7疗程)及血清LDH(>800U/L)为影响预后的因素。Cox回归模型多因素分析显示化疗疗程数(≥7疗程)及血清LDH(>800U/L)为独立的预后因素。结论:NB临床上应采取以手术、化疗和放疗为主的综合治疗,增加化疗疗程数可以提高NB患者总的生存率,并改善其预后;检测血清LDH对估计NB患者预后进而指导治疗具有重要意义。     

软组织恶性纤维组织细胞瘤化疗的临床观察

目的 观察软组织恶性纤维组织细胞瘤动脉灌注化疗的疗效.方法 44例软组织恶性纤维组织细胞瘤,采用皮下埋植式动脉介入化疗系统,用阿霉素和顺铂联合咖啡因、去甲斑蝥素治疗,治疗后随访32个月.结果 本组病变影像改变PR 18例(56.3%).化疗后手术病理中重度以上化疗反应30例(71.4%).平均随访32个月,术后无肿瘤局部复发.肺转移3例,2例死亡.结论 软组织恶性纤维组织细胞瘤的治疗可以选择动脉灌注化疗,以化疗后手术切除的肿瘤病理改变作为重要的预后评价标准.     

改良BFM-90方案治疗20例儿童青少年淋巴母细胞型淋巴瘤

背景与目的：儿童青少年淋巴母细胞型淋巴瘤属于高度恶性淋巴瘤．进展快,死亡牢高。德国BFM-90淋巴母方案是目前治疗儿童青少年淋巴母细胞型淋巴瘤疗效最好的方案之一。本研究采用改良BFM-90淋巴母方案治疗中国儿童青少年淋巴母细胞型淋巴瘤,观察其疗效、毒性和可行性。方法：20例3～18岁初治的T淋巴母细胞淋巴瘤患者,Ⅲ期7例,Ⅳ期13例。18例(90％)患者有纵隔肿块伴上腔静脉阻塞综合征,10例(50％)有骨髓侵犯。所有患者均接受改良BFM-90方案化疗．方案包括诱导缓解、巩固治疗和中枢神经系统预防、再诱导缓解和维持治疗。总疗程2年。用Kaplan-Meier法统计全组生存率。结果：诱导缓解结束后18例(90％)患者获得完全缓解(complete response,CR),1例(5％)部分缓解(partial response,PR),1例(5％)肿瘤进展(progressive disease,PD),总有效率95％。PR和PD的2例患者最后肿瘤进展死亡。CR的18例患者中有2例在再诱导结束后CR1时行外周血自体造血干细胞移植。移植后有1例复发,经再次化疗后CR存活;另1例一直存活。其他CR的患者中有5例复发,其中1例行异基因移植后存活,1例自体造血干细胞移植后存活,3例复发后单纯化疗的患者肿瘤进展死亡。全组患者3年总生存率74％。所有患者在诱导和再诱导阶段均发生Ⅲ／Ⅳ骨髓抑制,积极对症处理后可恢复。结论：改良德国BFM-90淋巴母方案适用于中国儿童青少年淋巴母细胞型淋巴瘤患者,可明显改善生存率;主要不良反应是血液毒性,应在有经验的血液／肿瘤中心中应用。     

Conservative surgery for malignant ovarian tumor in women of childbearing age

Background. The traditional operative procedures for the treatment of ovarian cancer have been simple total hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. However, young patients who want to preserve fertility are occasionally encountered. This study was designed to assess the results of conservative surgical management for young women with stage I epithelial ovarian carcinoma or malignant germ cell tumor and to explore the indications for such surgical treatment. Methods. Fifty-eight patients aged under 35 years were treated for malignant ovarian tumors (germ cell tumor, n = 20; epithelial tumors, n = 38) between 1971 and 1996. We studied all 20 patients with germ cell tumors and 22 pa-tients with stage I epithelial tumors; we excluded the 12 patients with stage II and more advanced disease and the 4 patients with stage I clear cell carcinoma, because these patients had poor prognoses. Eleven of the 22 patients with stage I epithelial tumors and 8 of the 20 patients with germ cell tumors received conservative surgery (conservative surgery group); the remaining 23 patients underwent radical surgery, including hysterectomy and bilateral salpingo-oophorectomy (radical surgery group). The overall survival rates of the two groups were compared, and ovarian function and pregnancy outcome were evaluated in the conservative surgery group. Results. Of the patients with malignant epithelial tumor who were treated with conservative surgery, 3 patients with stage Ic mucinous adenocarcinoma died 1 year and 8 months, 7 years and 6 months, and 8 years, respectively, after the initial surgery. The 10-year survival rate was 65% for the conservative surgery group and 91% for the radical surgery group. All patients with stage Ia epithelial tumors in both groups survived, and there were no differences in survival curves between the two groups among the patients with stage Ic epithelial tumors. All patients with germ cell tumors in the conservative surgery group survived. Their survival rate, however, was not significantly better than that in the radical surgery group. All patients with germ cell tumors who received platinum-based chemotherapy survived and had a significantly better survival rate than those who received non-platinum-based regimens (P < 0.05). All the patients in the conservative surgery group received postoperative chemotherapy; 10 of these patients had transient ovulation failure after the completion of chemotherapy, although a normal menstrual cycle was restored within 1 year. Of the 10 patients who wished to have babies, 6 became pregnant, and they have had a total of 11 children. Conclusion. Conservative surgery is feasible in patients with stage Ia epithelial carcinoma and germ cell tumor. Postoperative chemotherapy suppressed ovarian function in these patients, but only for a brief period. Received: January 5, 1999 / Accepted: October 27, 1999     

Results of treatment after relapse from high-dose chemotherapy in germ cell tumors.

PURPOSE: To identify therapy-related or patient-related characteristics that predict response and long-term survival after failure of high-dose chemotherapy (HDCT) for germ cell tumors (GCT). PATIENTS AND METHODS: Between 1986 and 1997, 101 GCT patients relapsed after high-dose carboplatin and etoposide (VP-16) at Indiana University (Indianapolis, IN). Median time to relapse was 10 months (range, 1 to 17 months). HDCT was the first salvage treatment in 29 patients and second or later salvage treatment in 72 patients. RESULTS: Fifty-four of 101 patients received post-HDCT treatment. Of these, 47 received chemotherapy, alone (n = 35) or in combination with surgery (n = 12). Seven patients underwent surgery alone. There were only 12 objective responses (three complete and nine partial responses) for 66 chemotherapy regimens given to 47 patients, for an overall response rate of 18.2%. Fifteen patients received platinum-based chemotherapy, with only one objective response. Chemotherapy was discontinued in 17% of cases because of toxicity. A longer interval between HDCT and post-HDCT treatment was the only variable that was associated with response. Five patients (4.9%) are disease-free at 30, 53, 57, 85, and 93 months after relapse. Of these, three responded to oral VP-16 and underwent resection of residual mediastinal, retroperitoneal, and inguinal cancer, respectively. One had resection of residual mediastinal yolk sac tumor, followed by oral VP-16. One relapsed with teratoma and received thoracoabdominal resection without chemotherapy. CONCLUSION: Patients who experience disease progression after HDCT often receive further chemotherapy and/or surgery. Chemotherapy resulted in a response rate of less than 20%, with only three complete responses. All of the long-term survivors (4.9%) had surgery as a component of their post-HDCT regimen.     

顺铂联合化疗治疗卵巢恶性生殖细胞肿瘤

目的：探讨卵巢恶性生殖细胞肿瘤的治疗疗效及复发患者二线治疗。方法：对我院1958年11月－1998年12月312例卵巢恶性肿瘤中的154例接受顺铂方案化疗的卵巢恶性生殖细胞肿瘤结果进行分析。结果：154例卵巢恶性生殖细胞肿瘤的总生存率为74．7％，初治患者（I－Ⅲ期）92例，5年生存率为92．2％，其中66例（71．7％）保留生育功能，19例已正常生育20次。复发转移62例，5年生存率51．1％，8例保留生育功能。结论：为了提高卵巢恶性生殖细胞肿瘤生存率，首次手术时应正确分期，术后及时采用当前标准BEP方案作为一线化疗，连续3－6疗程。对复发患者，根据以往治疗情况尽早选用有效的二线方案化疗，有利于提高生存率。     

Primary mediastinal germ cell tumors in children and adolescents: results of the German cooperative protocols MAKEI 83/86, 89, and 96.

PURPOSE: To evaluate children and adolescents with primary mediastinal teratoma and malignant germ cell tumors (GCTs). PATIENTS AND METHODS: Forty-seven patients from the German nontesticular GCT studies were analyzed (median age, 2.5 years; range, neonate to 17 years). Teratoma (n = 21) were resected, and no adjuvant treatment was given. Malignant GCTs (n = 26) were treated with cisplatin-based chemotherapy and resection. Three of 26 patients underwent radiotherapy. RESULTS: In all patients with teratoma, tumor markers were normal. Surgery of teratoma was complete in 17 of 21 patients and microscopically incomplete in four of 21 patients, and we observed no relapse after a median follow-up of 29 months. In 23 of 26 patients with malignant GCTs, alpha-fetoprotein and/or beta-human chorionic gonadotropin were elevated. Twelve of 26 patients received adjuvant chemotherapy after initial resection, which was complete in six of 12 patients, whereas delayed resection after preoperative chemotherapy was complete in 10 of 11 patients (P =.03). Four of six patients underwent second-look thoracotomy after incomplete primary surgery. Three of 26 patients did not undergo tumor resection. The final completeness of resection was the strongest prognostic indicator (event-free survival ?EFS, 0.94 +/- 0.06 v 0.42 +/- 0.33; P <.002). Local stage and distant metastases were not prognostically significant at the.05 level. For all malignant GCTs, the 5-year survival rate was 0.87 +/- 0.05 (median follow-up, 51 months), with an EFS of 0.83 +/- 0.05. CONCLUSION: The prognosis of mediastinal teratoma is excellent after complete or microscopically incomplete resection. In children with malignant GCT, the prognosis is favorable with a therapeutic strategy of delayed resection after preoperative chemotherapy. In most children, the diagnosis can be based on elevated tumor markers and imaging. Biopsy is indicated in nonsecreting GCT.     

卵巢恶性生殖细胞肿瘤的治疗

探讨改进卵巢恶性生殖细胞肿瘤的治疗方法。方法 回顾分析我院收治的２３３例卵巢恶性生殖细胞肿瘤,Ｉ期９４例,Ⅱ－Ⅳ期４３例,复发转移９６例。单附件或全宫双附件加大网膜阑尾切除分别为７８例和１５１例,活检４例,单纯手术１７例,手术加放疗和化疗６５例,手术加化疗１５１例。结果全组存活１２７例,Ⅰ期存活７８例,Ⅱ－Ⅳ期１７例,复发转移３２例。     

A clinico-statistical study of testicular tumors--a reevaluation of 90 cases at the Nagasaki University Hospital and affiliated hospitals

We have treated 90 cases of testicular tumors at our institutes from April, 1962 to March, 1986. Of 75 which were germ cell type tumors, 35 were a seminoma, for which the 5-year survival rate was 100% for patients in stage I, and 50% for those in stage III, respectively. Forty cases were non-seminomas, and all cases determined as being in stage I survived for 5 years, whereas 47.6% cases of those in stage III survived for 4.5 years. Thirty-six percent of those with a stage I germ cell tumor were treated with a orchiectomy alone, while other cases also combined radiotherapy and/or chemotherapy. Almost all cases of an advanced tumor received combined retroperitoneal lymph node resection, radiotherapy and/or chemotherapy. Patients with an advanced nonseminomatous tumor showed better a survival rate if they were given CDDP chemotherapy.     

Pediatric germ cell tumors

Pediatric germ cell tumors are a diverse group of neoplasms with variable clinical behaviors, depending upon the age and site of presentation. Most result from sporadic mutations, although environmental exposures and other genetic aberrations may play a role. Platinum-based chemotherapy has dramatically improved the event-free and overall survival outcomes of pediatric patients with malignant germ cell tumors over the past two decades. Prognosis is dependent on tumor stage and location. Patients with gonadal germ cell tumors have at least a 95% 5-year survival for early stage disease and at least a 85% 5-year survival for advanced stages. In general, extragonadal germ cell tumors carry a poorer prognosis with mediastinal location having the worst outcomes (70% 4-year survival). Current trials are focused on maintaining similar excellent outcomes while reducing morbidity by reducing the dose and duration of chemotherapy. Cytogenetic research studies have found chromosomal aberrations specific to some of these tumors that may serve as prognosticators and even direct therapy.     

Poor prognosis of mediastinal germ cell cancers containing sarcomatous components.

Fifteen patients with biopsy-proven mediastinal germ cell tumors treated with platinum-based chemotherapy were reviewed. They had a period of 4 to 6 weeks between the onset of symptoms and diagnosis. Four patients had sarcomatous elements in their tumor in association with common germ cell histologies. The sarcomatous components consisted of one angiosarcoma, one rhabdomyosarcoma, and two cases with mixed angiosarcoma and rhabdomyosarcoma. All patients with sarcomatous elements died; the median survival for these patients was 9 months. In contrast, six (54%) of the patients who did not have sarcomatous elements in their tumor are long-term disease-free survivors 5 to 8 years after diagnosis. The occurrence of sarcomatous elements in a mediastinal germ cell tumor is a poor prognostic sign, and therapy should be oriented to include drugs and regimens that may be effective against sarcoma.