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.     

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。     

原发性纵隔恶性生殖细胞肿瘤临床和预后分析

目的探讨原发性纵隔恶性生殖细胞肿瘤(PMGCT)的临床病理特点、治疗方法和预后因素。方法回顾性分析29例PMGCT患者的临床资料。结果29例患者均为男性,平均发病年龄26.1岁,肿瘤均来源于前纵隔,平均最大径16.0 cm。其中原发性纵隔精原细胞瘤(PMSGCT)5例(17.2%),原发性纵隔非精原细胞瘤(PMNSGCT)24例(82.8%)。PMGCT最常见症状是憋气、咳嗽与胸痛,其治疗采用化疗、手术、放疗相结合的综合治疗模式。PMNSGCT组中化生存期为19.0个月, 1年和2年生存率分别为65.3%和28.1%。PMSGCT组均长期生存,预后优于PMNSGCT组(P= 0.008)。多因素分析结果显示,病变局限于纵隔、以顺铂为基础的联合化疗是PMNSGCT患者预后的独立影响因素。结论PMGCT以PMNSGCT为主,主要治疗手段是以顺铂为基础的联合化疗。PMNSGCT预后明显差于PMSGCT,并与病变范围、化疗与否相关。     

原发于纵隔的生殖细胞肿瘤47例临床分析

目的探讨原发于纵隔的生殖细胞肿瘤的临床特点、治疗方法及预后的影响因素。方法回顾性分析47例原发于纵隔的生殖细胞肿瘤患者的临床资料。结果47例患者中,男性41例,女性6例,中位年龄26岁;8例（17．0％）精原细胞瘤,39例（83．0％）非精原细胞瘤。全组患者中位生存期为16个月,1、3、5年生存率分别为63．4％、37．5％和34．8％;非精原细胞瘤患者1、3、5年生存率分别为56．4％、30．0％和27．3％,8例精原细胞瘤患者中,7例生存满5年。多因素分析显示,病理类型是原发于纵隔的生殖细胞肿瘤患者预后的独立影响因素（P=0．045）。结论纵隔精原细胞瘤患者对放疗、化疗敏感,预后较好;纵隔非精原细胞瘤患者预后差,化疗是其主要治疗手段,以顺铂为基础的化疗明显提高了这类患者的生存率。     

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。     

46例原发纵隔恶性淋巴瘤临床分析

本文对４ｇ例原发纵隔恶性淋巴瘤的常见症状、体征及胸部Ｘ线表现作了临床分析。本组以儿童、青年及非何杰金氏杰淋巴瘤患者多见。合并上腔静脉综合征（２８．２６％），淋肉瘤白血病（２３．９１％），心包受侵，脑膜浸润分别为（６．５％），喉返神经麻痹（４．３％）。合并淋巴肉瘤白血病、脑膜侵犯后，病情发展快、预后差。     

原发纵隔恶性生殖细胞瘤32例临床分析

目的:探讨纵隔恶性生殖细胞瘤(malignant germ cell tumors,MGCT)的临床特点、治疗和预后。方法:32例纵隔MGCT患者,精原细胞瘤18例,非精原细胞瘤14例。所有患者均采用手术和(或)放疗和(或)化疗等多学科综合治疗的方法。结果:非精原细胞瘤患者中位生存期(OS)32.4个月,中位无进展生存期(PFS)18个月,5年无复发生存率和总生存率均为28.6%。精原细胞瘤患者5年无复发生存率和总生存率分别为83.3%和85.6%,中位OS和PFS均未到达。精原细胞瘤患者OS和PFS均明显好于非精原细胞瘤患者,P值分别为0.001 4和0.000 7。结论:纵隔精原细胞瘤采用多学科综合治疗方法能取得较好的治疗效果,本研究的结果与文献报道相符。纵隔非精原细胞瘤的治疗效果有待进一步提高。非精原细胞瘤是影响纵隔恶性生殖细胞瘤预后的重要因素。     

纵隔原发性恶性纤维组织细胞瘤

为了研究原发性纵隔恶性纤维组织细胞瘤的临床病理特点。采用常规石蜡包埋ＨＥ 染色及免疫组织化学方法研究３例ＭＦＨ。３ 例ＭＦＨ 均位于中纵隔,肿瘤界限清楚有包膜或假包膜,术后无局部复发及转移,最长无瘤生存者已达９ ａ。纵隔原发ＭＦＨ的病理特点与软组织ＭＦＨ 相同,但局部复发率及转移率低,提示其预后好于软组织ＭＦＨ。     

30例原发颅内生殖细胞瘤治疗的远期疗效

目的 报告30例原发颅内生殖细胞瘤治疗的远期疗效和探讨其治疗方法。方法 30例先经不同程度肿瘤切除手术后给予放射治疗,放射治疗采用常规分割外照射方法。28例全脑预防照射后缩小照射野到肿瘤区追加放射至肿瘤足量;2例仅做肿瘤区放射治疗。19例做了全脊髓预防照射。未做全脑预防和低剂量脑预防照射的2例出现脑转移（1例合并脊髓转移）做再程放射治疗。结果 全部病例5、10、15、19年生存率分别为93．1％、87．6％、87．6％、68．2％。临床症状缓解率为95％。再程放射治疗的2例中1例生存超过17年,1例出现也超过10年。结论 颅内生殖细胞瘤争取手术治疗的贡献在于减小肿瘤体积并获得病理诊断和分流脑积水,放射治疗是治愈的有效方法。全脑预防照射作为必要治疗措施,脑预防剂量为26－30Gy和肿瘤灶治疗量在50－55Gy为宜。脊髓预防照射可因病情需要选择采用,不可作为常规方法。14岁以下幼年在脑预防照射时要保护垂体,不做脊髓预防照射为好,对原发蝶鞍区肿的放射治疗总量降低到45Gy。     

原发鞍上生殖细胞瘤的放射治疗

原发于鞍上的生殖细胞瘤较少见，我科从1987～2001年治疗7例经组织学证实的原发鞍上生殖细胞瘤，报告如下。     

原发性纵隔恶性生殖细胞肿瘤

原发性纵隔恶性生殖细胞肿瘤临床少见,与相应原发性性腺生殖细胞肿瘤的临床表现、诊断、治疗及预后均不相同。以顺铂为基础联合化疗是其主要治疗手段,近年通过综合治疗患者预后明显好转,原发性纵隔精原细胞瘤治愈率高,但非精原细胞瘤预后仍不理想。     

Chemotherapy trials in recurrent primary intracranial germ cell tumors

Summary Gonadal germ cell tumors respond favorably to chemotherapy either at diagnosis or when they recur. Histologically similar tumors may arise in the CNS usually in the pineal or suprasellar regions. Although radiation therapy may produce a 5 year disease-free survival in excess of 60% in localized pure germinoma, gern cell tumors of other histology tend to recur. We have conducted 14 chemotherapy trials in 8 patients with recurrent CNS germ cell tumors using 3 different single agent and 2 multi-agent chemotherapy regimens. The histologic diagnoses of the patients were germinoma (4), endodermal sinus tumor (2), embryonal carcinoma (1), and mixed tumor — germinoma plus choriocarcinoma (1). There were 7 males and 1 female with a median age of 13 years. The primary tumor arose in the pineal region in 6 and was multicentric in 2. Seven patients had local recurrences and one developed an initial recurrence in the spinal canal. Three patients had CNS metastases at relapse and 2 had systemic metastases. Objective responses were documented in 7 of 14 trials (50%). Responses were observed with cyclophosphamide (80 mg/kg) in 3 of 4 patients for 2⁺, 3, and 5 mos, cisplatin (120 mg/m²) in 1 of 2 patients for 2⁺ mos, and the VAB 6 protocol (vinblastine, bleomycin, cyclophosphamide, actinomycin-d, cisplatin) in 3 of 5 patients for 5, 8, and 18 mos. The median duration of response was 5 mos. (2¹-18). High doses of single chemotherapy agents such as cyclophosphamide and cisplatin as well the VAB6 regimen have definite activity in recurrent CNS germ cell tumors, especially germinoma. Good palliation may be achieved with chemotherapy alone with acceptable morbidity. Adjuvant chemotherapy should be considered in patients with newly diagnosed primary intracranial germ cell tumors whose tumors are considered unlikely to be permanently controlled with radiation alone.     

Second-line high-dose chemotherapy in patients with mediastinal and retroperitoneal primary non-seminomatous germ cell tumors: the EBMT experience.

BACKGROUND: Results of second-line chemotherapy in patients with extragonadal non-seminomatous germ cell tumor (NSGCT) appear inferior to results in testicular NSGCT. Patients with retroperitoneal NSGCT achieve a comparable long-term survival rate of 30%, but the salvage rates of patients with mediastinal primary are less than 10%. We conducted a retrospective analysis on patients with mediastinal and retroperitoneal NSGCT treated with second-line high-dose chemotherapy (HDCT) registered with the European Group for Blood and Marrow Transplantation (EBMT). PATIENTS AND METHODS: Between 1987 and 1999, 59 registered patients with retroperitoneal (n=37) and mediastinal (n=22) primary NSGCT, median age 28 years (range 18-60), were treated with second-line HDCT. All had received cisplatin-containing chemotherapy as first-line treatment. RESULTS: Toxic death occurred in three cases (5%). With a median follow-up of 58 months (range 14-114), 18/59 patients (30%) continue to be disease-free. Of three patients who had a disease recurrence after HDCT, one patient achieved a disease-free status with further chemotherapy and surgery. In total, 19 patients (32%) are currently disease-free. Sixteen of 37 patients (43%) with retroperitoneal NSGCT, and three of 22 patients (14%) with mediastinal NSGCT are currently alive and disease-free. CONCLUSIONS: Second-line HDCT might represent a possible option for patients with retroperitoneal primary NSGCT. New salvage strategies are needed for patients with mediastinal NSGCT.     

Correlation of endoscopic biopsy with tumor marker status in primary intracranial germ cell tumors

Summary We retrospectively analyzed the results of eight patients who underwent endoscopic biopsy of a newly diagnosed primary intracranial germ cell tumor (GCT), and correlated tumor pathology with serum and cerebrospinal fluid (CSF) tumor markers and treatment outcome in order to determine the reliability of GCT sampling by this method. A biopsy diagnosis was made in each patient, and the tumor histology correlated with tumor marker measurements for all six patients diagnosed with germinoma and for one with a yolk sac tumor. One biopsy revealed only mature teratoma, an inconclusive result since the patient’s serum and CSF tumor markers were elevated. No morbidity was experienced as a result of the operative procedure. Five of six patients diagnosed with germinoma responded completely to radiation therapy and are without evidence of disease, while one suffered a likely germinoma recurrence and was subsequently successfully retreated. We conclude that endoscopic biopsy of marker-negative germ cell tumors is a safe, reliable method of establishing a diagnosis of germinoma. However, endoscopic biopsy may fail to yield an accurate diagnosis in cases of malignant non-germinomatous tumor. We would thus conclude that when primary germ cell tumor is considered, endoscopic tumor biopsy is recommended in patients with a negative biochemical analysis, but not suggested for patients presenting with elevated tumor markers.     

儿童颅外恶性生殖细胞肿瘤的临床特点及预后分析

目的:总结儿童颅外恶性生殖细胞肿瘤的临床特点及影响预后的因素。方法:回顾性分析2015年01月至2019年12月我院收治的49例颅外恶性生殖细胞肿瘤的临床资料。结果:共入组49例颅外恶性生殖细胞肿瘤患者,男性29例,女性20例,男女比例1.45∶1;中位发病年龄20月（4~189月）;<4岁42例,4~11岁4例,>11岁3例;原发瘤灶位于性腺者30例（睾丸22例,卵巢8例）,性腺外者19例（纵隔2例,腹腔1例,盆腔4例,阴道4例,骶尾部8例）;I期6例,II期16例,III期14例,IV期13例;低危组6例,中危组24例,高危组19例;病例类型:卵黄囊瘤31例,恶性畸胎瘤12例,混合型恶性生殖细胞肿瘤6例。所有患儿均行手术、化学治疗,部分患者行放疗。中位随访时间49月,经治疗全组CR 44例,PR 1例,复发或进展4例,其中死亡2例。2年总生存率（OS）和无事件生存率（EFS）分别为（98.0±2.0）%和（93.9±3.4）%。5年OS和EFS为（95.6±3.1）%和（91.7±4.0）%。结论:以铂类为主的化疗对于儿童颅外恶性生殖细胞肿瘤治疗效果肯定,初诊患者AFP≥10 000 ng/mL及年龄>11岁与预后不良相关,复发/难治患者可行常规二线化疗、大剂量化疗、自体干细胞移植、放疗等综合治疗。     

Mediastinal non-seminomatous germ cell tumours (MNSGCT) treated with cisplatin-based combination chemotherapy

Background: Primary mediastinal non-seminomatous germ cell tumours (MNSGCT)constitute a rare malignancy. This study was performed to review ourexperience with cispatin-based chemotherapy in patients with MNSGCT.Patients and methods: Patients with MNSGCT treated with cisplatin-basedcombination chemotherapy between 1978–1995 in three university hospitalsin Spain were retrospectively studied.Results: There were 25 males and two females with a median age of 26 years(range 4–71). Fifteen patients had disease confined to the mediastinumand 12 had metastatic disease. All patients were treated with cisplatinchemotherapy regimens (PVB: 7, BEP: 6, and other regimens 12) and consideredfor residual mass surgery (RMS) when indicated. Eleven patients (40.7%)were rendered disease-free with initial treatment: four with chemotherapyalone, one with surgery plus adjuvant chemotherapy and six with chemotherapyplus RMS. Three of these patients relapsed at two, six and seven months. Theremaining 16 had unfavourable reponses (five partial response, three nochange, seven progressive disease and one toxic death) . Eleven patientsreceived salvage treatment but none of them achieved a durable response. Aftera median follow-up of 77 months (range 1–168), 10 patients remain alive.Actuarial survival at five years is 31.7%. No patients in this seriesdeveloped a haematological malignancy. Chromosomal analysis showed that 2 outof 10 patients (20%) had a 47XXY karyotype.Conclusions: Only patients who achieved disease-free status are likely tobe cured. Therefore, new up-front strategies are needed for the treatment ofMNSGCT.     

Cisplatin-based chemotherapy in primary central nervous system germ cell tumors

We report a retrospective review of our experience with cisplatin-based chemotherapy in eight patients (ages 9–44 years) with histologically confirmed primary central nervous system germ cell tumors. Five patients received chemotherapy as the primary treatment, radiation therapy being administered either at completion of chemotherapy or between chemotherapy courses. Three patients received cisplatin-based chemotherapy for recurrent disease after prior radiation therapy and/or surgery. Four of five patients treated with chemotherapy at diagnosis are in complete remission at 11–14 months from diagnosis. The remaining patient twice achieved complete remission prior to dying of progressive disease 16 months after diagnosis. Two of three patients treated with chemotherapy for recurrent disease are in complete remission at 20 and 26 months; the remaining patient deteriorated after the first cycle of chemotherapy and expired six months thereafter. Overall, of seven patients evaluable for response, five achieved complete remission with chemotherapy alone, and two with chemotherapy and radiation therapy. Our results confirm previous reports of high complete remission rates utilizing cisplatin-based chemotherapy in conjunction with radiation therapy. Prospective evaluation of cisplatin-based chemotherapy followed by radiation therapy is warranted.     

Primary malignant mediastinal germ cell tumours: A literature review and a study of 18 cases

From 1957 to 1992, 18 cases of primary mediastinal germ cell tumours were referred to the Peter MacCallum Cancer Institute (PMCI). Six were seminomas, six were mixed germ cell tumours, two were embryonal cell carcinomas, three were teratocarcinomas and one was labelled an ‘anaplastic germ cell tumour’. Two of the 18 patients were female. For seminomas, surgical (and in one case chemotherapeutic) debulking, followed by radiotherapy produced the best results. Mediastinal doses ranged from 30 to 40 Gy. Local control was achieved in those patients receiving mediastinal radiotherapy. Four patients currently survive disease-free. The non-seminomatous germ cell tumours showed a significantly poorer survival, and only two of 12 patients remain alive in remission at 110 and 130 months after diagnosis. Survival has been updated as of November 1997. Attention is focused on the anterior position of the primary germ cell tumours in the mediastinum. A review of the literature up to and including 1997 is presented.     

生殖细胞肿瘤治疗的研究进展--2004年ASCO文献综述

生殖细胞肿瘤是15～35岁男性最常见的恶性肿瘤.目前的多学科综合治疗使大多数患者得到了治愈,人们正试图如何进一步提高疗效,减少治疗带来的远期毒性.2004年ASCO年会收录的关于男性生殖细胞肿瘤的研究报告主要集中在以下几个方面:术后辅助化疗的地位、基于预后因素的个体化治疗、新药和新的化疗方案、自体造血干细胞移植的治疗策略和长期生存者生活质量的影响因素.本文就这些方面的研究进展作一综述.