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.     

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。     

纵隔恶性畸胎瘤的诊治(附6例报告)

目的提高临床对纵隔恶性畸胎瘤的认识.方法回顾性分析我院1972年～1999年经病理检查确诊的6例纵隔恶性畸胎瘤.男性5例,女性1例.术前均行胸片和/或胸部CT检查.1例单纯手术,4例手术加术后放疗,1例术后放疗及化疗.结果据术前影像学资料,仅1例患者能明确诊断为纵隔恶性畸胎瘤而非纵隔肿瘤.失访1例,无瘤生存3例(分别存活62、85和147月),死亡2例(分别无瘤存活1、9月).结论胸部CT/胸片检查很难明确畸胎瘤的诊断.手术彻底性明显影响预后.放疗对手术残留者可能有效.化疗效果有待进一步的经验.     

72例卵巢恶性生殖细胞肿瘤的治疗及预后

目的 总结72例卵巢恶性生殖细胞肿瘤的治疗及预后。方法 1978年至1991年我院共收治卵巢恶性生殖细胞肿瘤72例,占同期收治的卵巢恶性肿瘤总数的22.15％。Ⅰ期43例,Ⅱ期5例,Ⅲ期15例,Ⅳ期8例。实施以手术为主加化疗或/和放疗。结果 全组5年生存率54.2％,其中无性细胞瘤81.52％,未成熟畸胎瘤53.30％,混合性生殖细胞瘤33.33％,内胚窦瘤22.20％,胚胎癌0。结论 影响其愈后的诸因素中,除临床期别,病理类型外,恰当的手术切除范围,术后多疗程的联合化疗显得尤为重要。对年青需保留生育功能的无性细胞瘤患者,术后化疗取代传统的放疗较为妥当。     

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。     

原发性性腺外精原细胞瘤(附5例报告及文献复习)

目的：提高对原发性性腺外精原细胞瘤的认识，方法：5例原发性性腺外精原细胞瘤，均经手术切除及组织病理学证实，术后分别行化疗或放疗。结果：随访至今5例患者均健在，结合文献对原发性性腺外精原细胞瘤的组织发生，临床表现，治疗原则等进行讨论。结论：原发性性腺外精原细胞瘤少见，症状因部位而异，容易误诊，组织病理检查对该病诊断，治疗方案的选择有重要价值。治疗上尽量完整手术切除，但不必勉强，该肿瘤对放，化疗敏感。     

44例儿童青少年恶性生殖细胞肿瘤综合治疗结果分析

背景与目的:目前儿童青少年恶性生殖细胞瘤采用综合治疗,总生存率已达75%以上,然而,不同分期、病理类型和发病部位的患者有不同的预后。本文分析儿童青少年恶性生殖细胞瘤的临床特点、综合治疗的效果和影响预后的因素,并探讨其治疗策略。方法:对1997年1月～2005年12月中山大学肿瘤防治中心收治的儿童青少年恶性生殖系统肿瘤患者的临床表现、综合治疗疗效和不良预后因素进行分析;采用Kaplan-Meier法计算全组生存率。结果:44例患者中,25例行术后辅助化疗;1例单纯手术;18例行诱导化疗,其中7例患者化疗后肿瘤缩小行手术切除,2例原发纵隔绒癌伴多发转移患者化疗后行残留病灶放疗,1例术后腹腔转移和1例术后肺转移患者化疗后获得完全缓解,1例原发纵隔内胚窦瘤化疗后部分缓解,未做进一步治疗,6例患者化疗无效进展死亡。化疗的患者均采用含铂类化疗方案治疗2～7个疗程。中位随访时间32个月,全组3年总生存率为84.8%;Ⅰ Ⅱ期患者3年生存率为100%,Ⅲ期为83.3%,Ⅳ期为65.6%,复发患者为66.7%;初治生殖器内(睾丸和卵巢)肿瘤患者3年生存率为96.0%,生殖器以外肿瘤患者为61.0%。结论:手术联合含铂类药物化疗能明显改善儿童青少年生殖细胞瘤的疗效和生存率,但对Ⅳ期、复发转移和生殖器以外的生殖细胞瘤患者应探讨新的方案和增加剂量强度。     

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.     

卵巢恶性生殖细胞肿瘤（附83例临床分析）

卵巢恶性生殖细胞肿瘤起源于原始生殖细胞,多发于儿童及生育年龄妇女,包括无性细胞瘤,未成熟畸胎瘤及内胚窦瘤等。其生物行为和预后各不相同。无性细胞瘤对射线高度敏感,治疗方法得当,治愈率可高达90～95％;未成熟畸胎瘤经反复手术可发生恶性程度逆转~6;内胚窦瘤可产生AFP~4,借以指导治疗。现将我院的治的83例恶性生殖细胞肿瘤分析如下。材料和方法我院1975年至1985年共收治恶生殖细胞肿瘤83例,全部病历重新复查病理切片;临床分期采用FIGG分期法;生存率按寿命表法计算累计生存率,全部病历随访到86年12月未。治疗方法以手术为主术后辅以化疗或放疗。手术     

Primary Malignant Germ Cell Tumours of the Mediastinum: Results of Multimodality Treatment

Primary malignant mediastinal germ cell tumours are rare and considered to have poorer prognosis compared with those arising from gonads. Eighteen patients with primary mediastinal germ cell tumour were treated over an 11-year period; 9 had seminoma and 9 non-seminoma. Eight patients, 4 each with seminoma and non-seminoma underwent initial tumour excision and the rest had biopsy only. All patients received cisplatin-based chemotherapy. All patients with seminoma received consolidation radiotherapy to mediastinum. Three patients with non-seminoma received radiotherapy following partial response. All 9 patients with seminoma achieved complete response at the end of chemotherapy. Two patients with NSGCT had complete response to chemotherapy, 5 partial response and 2 no response. Two patients who underwent resection of the residual tumour mass are surviving free of disease. Addition of radiotherapy or second-line chemotherapy did not bring about any added response in partial and non-responders. Nine out of 9 patients with seminoma and 4/9 with non-seminoma are surviving disease-free at a median follow-up of 48 months (range 16-153 months). Mediastinal seminoma has excellent prognosis with cisplatin combination chemotherapy, whereas non-seminoma carries poor prognosis, and aggressive chemotherapy with resection of residual masses may improve the outcome. The role of additional radiotherapy and initial tumour debulking needs further evaluation.     

Primary malignant mediastinal germ cell tumours: improved prognosis with platinum-based chemotherapy and surgery.

A retrospective analysis was performed of 18 patients with primary malignant germ cell tumours of the mediastinum treated with platinum-based chemotherapy between 1977 and 1990. All seven patients with pure seminoma were treated initially with chemotherapy and four of these patients received additional mediastinal radiotherapy. Only one patient relapsed; his initial therapy had included radiotherapy and single-agent carboplatin and he was successfully salvaged with combination chemotherapy. With a follow-up of 11 to 117 months (median 41 months) all seven patients with seminoma remain alive and disease free giving an overall survival of 100%. Eleven patients had malignant non seminoma; following chemotherapy eight of these had elective surgical resection of residual mediastinal masses. Complete remission was achieved in nine (82%) patients, however, one of these patients died from bleomycin pneumonitis. With a follow-up of 12 to 113 months (median 55 months) eight of 11 (73%) patients with malignant mediastinal teratoma remain alive and disease free.     

Primary mediastinal germ cell tumors. Histologic patterns of treatment failures at autopsy

Twenty-five patients presented with primary mediastinal germ cell tumors at Roswell Park Memorial Institute between 1959 and 1984. All patients were treated by surgery and chemotherapy with or without radiotherapy. Four patients are still alive, and 21 patients died of mediastinal germ cell tumor and its sequelae. Two patients were found to have testicular scars and were dropped from the study. Nongerm cell malignant transformation of a teratoma occurred in five of the remaining 17 patients (29%), resulting in three adenocarcinomas and two sarcomas. Another patient developed leukemia. Metastatic disease most commonly involved the lungs, mediastinal lymph nodes, liver, bone, retroperitoneum, and heart. Respiratory failure was the cause of death in 12 patients. Of the possible mechanisms of germ cell transformation into malignant nongerm cell tumors discussed, this study suggests that chemotherapy alone is unlikely to induce stem cell differentiation. The presence of mature, differentiated teratoma within the primary lesion may be indicative of a poorer prognosis.     

Management of germ cell tumors in children: approaches to cure

The introduction of cisplatinum chemotherapy and current advances in the surgical treatment have resulted in a dramatic improvement of the prognosis of children with malignant germ cell tumors (GCT). Cisplatinum chemotherapy generally results in sufficient systemic tumor control, but local relapses may still occur in patients who did not receive adequate local treatment. Therefore, the therapeutic consideration must take into account age, primary site of the tumor, and its histology. In gonadal tumors, there is a high chance of primary complete resection since these tumors tend to be encapsulated, and particularly testicular GCT are often detected at a low tumor stage. In contrast, a primary complete resection may be impossible in large nongonadal tumors such as sacrococcygeal or mediastinal GCT. In these tumors, a neoadjuvant or preoperative chemotherapy after clinical diagnosis by imaging and evaluation of tumor markers significantly facilitates complete resection on delayed surgery. In addition, the impact of chemotherapy on local tumor control may be enhanced by locoregional hyperthermia. In most intracranial GCT complete resection is impossible and may be associated with significant morbidity. Nevertheless, biopsy is essential for diagnosis in nonsecreting tumors. In intracranial GCT, radiotherapy significantly contributes to local tumor control, and doses are stratified according to histology. These general considerations have been integrated into national and international cooperative treatment protocols. In most current protocols, treatment is stratified according to an initial risk assessment that includes the parameters age, site, histology, stage, completeness of resection and the tumor markers alpha(1)-fetoprotein (AFP) and human choriogonadotropin (beta-HCG). With such modern protocols overall cure rates above 80% can be achieved. Moreover, the previously high-risk groups may now expect a favorable prognosis with this risk-adapted treatment, whereas an increasing number of low-risk patients are treated expectantly or with significantly reduced chemotherapy. As current biologic studies reveal distinct genetic patterns in childhood GCT, it can be expected that further combined clinical and genetic studies will be valuable for risk assessment of childhood GCT.     

Role of postchemotherapy adjunctive surgery in the management of patients with nonseminoma arising from the mediastinum.

PURPOSE: To evaluate the role of postchemotherapy surgery in patients with nonseminomatous germ cell tumors arising from the anterior mediastinum. PATIENTS AND METHODS: Thirty-two patients with nonseminoma arising from a mediastinal primary site were treated on a clinical trial at our center, and they underwent postchemotherapy surgery. The results of postchemotherapy surgical resection, frequency of viable tumor found during postchemotherapy surgery, and prognostic factors for survival were assessed. RESULTS: Complete resection of all gross residual disease was achieved in 27 patients (84%). Histologic analysis of resected residua postchemotherapy revealed viable tumor in 66%, teratoma in 22%, and necrosis in 12% of the specimens. Viable tumor included embryonal carcinoma, choriocarcinoma, yolk sac carcinoma, seminoma, and teratoma with malignant transformation to nongerm cell histology (eg, sarcoma). Clinical characteristics associated with a shorter survival after surgery included the presence of viable tumor in a resected specimen (P =.003) and more than one site resected during surgery (P =.06). There were no statistically significant differences in survival for patients who underwent surgical resection with normal markers compared with patients with elevated serum tumor markers (P =.33). A trend toward shorter survival was found in patients with increasing tumor markers before surgery compared with patients with normal and declining serum tumor markers (P =.09). CONCLUSION: Surgical resection of residual mass after chemotherapy plays an integral role in the management of patients with primary mediastinal nonseminoma. Teratoma and viable tumor were found in the majority of resected residua after chemotherapy. Because patients who undergo conventional salvage chemotherapy programs rarely achieve long-term disease-free status, selected patients with elevated markers after chemotherapy are considered candidates for surgical resection.     

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.