Malignant histiocytosis associated with mediastinal germ cell tumor:A case report

BACKGROUND Germ cell tumors(GCTs)account for 2%of human malignancies but are the most common malignant tumors among males aged 15-35.Since 1983,an association between mediastinal GCT(MGCT)and hematologic malignancies has been recognized.CASE SUMMARY We report a case in which malignant histiocytosis was associated with mediastinal GCTs.The clinical data of a male patient with MGCT admitted to Beijing Children's Hospital were collected retrospectively.The patient was first diagnosed according to imaging and pathological features as having MGCT,and was treated with surgery and chemotherapy.One year after stopping chemotherapy,imaging showed metastases in the right supraclavicular,mediastinum,hilar region and retroperitoneal lymph node,right pleura,right lung,and right para-cardiac margin.Pathological diagnosis of the liver nodular and hilar lymph nodes included systemic juvenile xanthogranuloma and Rosai-Dorfman lesions with malignant transformation(i.e.,morphological characteristics and immunophenotype of histiocytic sarcoma).Following diagnosis,the patient accepted chemotherapy with vindesine,cytarabine and dexamethasone.Positron emission tomography–computed tomography showed partial remission.The patient was followed-up for 10 mo after the diagnosis of malignant histiocytosis,and no sign of progression or relapse was observed.CONCLUSION Physicians should recognize the possibility of hematologic malignancies being associated with MGCT.Suitable sites should be selected for pathological examination.

The clinical characteristics and treatment outcomeof 57 children and adolescents with primary central nervoussystem germ cell tumors

Primary central nervous system germ cell tumors(CNS-GCTs) in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese children and adolescents with CNS-GCTs. Therefore, in this study we retrospectively analyzed the clinical features and treatment outcome of Chinese children and adolescents with primary CNS-GCTs. Between January 2002 and December 2012, 57 untreated patients from a single institution were enrolled. They were diagnosed with CNS-GCTs after pathologic or clinical assessment. Of the 57 patients, 41 were males and 16 were females, with a median age of 12.8 years(range, 2.7 to 18.0 years) at diagnosis; 43(75.4%) had non-germinomatous germ cell tumors(NGGCTs) and 14(24.6%) had germinomas; 44(77.2%) had localized disease and 13(22.8%) had extensive lesions. Fifty-three patients completed the prescribed treatment, of which 18 underwent monotherapy of surgery, radiotherapy, or chemotherapy, and 35 underwent multimodality therapies that included radiotherapy combined with chemotherapy or surgery combined with chemotherapy and/or radiotherapy. PEB(cisplatin, etoposide, and bleomycin) protocol was the major chemotherapy regimen. The median follow-up time was 32.3 months(range, 1.2 to 139 months). Fourteen patients died of relapse or disease progression. The 3-year event-free survival(EFS) and overall survival rates for all patients were 72.2% and 73.8%, respectively. The 3-year EFS was 92.9% for germinomas and 64.8% for NGGCTs(P = 0.064). The 3-year EFS rates for patients with NGGCTs who underwent monotherapy and multimodality therapies were 50.6% and 73.5%, respectively(P = 0.042). Our results indicate that multimodality therapies including chemotherapy plus radiotherapy were better treatment option for children and adolescents with CNS-GCTs.

Oncology and reproductive outcomes over 16 years of malignant ovarian germ cell tumors treated by fertility sparing surgery

BACKGROUND Malignant ovarian germ cell tumors(MOGCT)are rare and frequently occur in women of young and reproductive age and the oncologic and reproductive outcomes after fertility-sparing surgery(FSS)for this disease are still limited.AIM To evaluate the oncology and reproductive outcomes of MOGCT patients who underwent FSS.METHODS All MOGCT patients who underwent FSS defined as the operation with a preserved uterus and at least one side of the ovary at our institute between January 2005 and December 2020 were retrospectively reviewed.RESULTS Sixty-two patients were recruited for this study.The median age was 22 years old and over 77%were nulliparous.The three most common histology findings were immature teratoma(32.2%),dysgerminoma(24.2%),and yolk sac tumor(24.2%).The distribution of stage was as follows;Stage I,74.8%;stage II,9.7%;stage III,11.3%;and stage IV,4.8%.Forty-three(67.7%)patients received adjuvant chemotherapy.With a median follow-up time of 96.3 mo,the 10-year progressionfree survival and overall survival were 82.4%and 91%,respectively.For reproductive outcomes,of 43 patients who received adjuvant chemotherapy,18(41.9%)had normal menstruation,and 17(39.5%)resumed menstruation with a median time of 4 mo.Of about 14 patients who desired to conceive,four were pregnant and delivered good outcomes.Only one case was aborted.Therefore,the successful pregnancy rate was 28.6%CONCLUSION The oncology and reproductive outcomes of MOGCT treated by FSS are excellent.Many patients show a long survival time with normal menstruation.However,the obstetric outcome is not quite satisfactory.

Primary Malignant Giant Cell Tumor of Bone:A Case Report

Primary malignant giant cell tumor of bone is clinically rare,lack of specificity,and often misdiagnosed.Currently,related literature about this tumor remains scarce.One case of primary malignant giant cell tumor of bone was diagnosed and treated in our hospital,and the treatment effect was satisfactory.There was no recurrence or metastasis in 2 years of followup.The report is as follows.

Nuclear Protein in Testis Midline Carcinoma Simulating Germ Cell Tumor of the Mediastinum

Nuclear protein in testis (NUT) midline carcinoma (NMC) is a very rare and aggressive human cancer characterized by overexpression of the nuclear protein in testis (NUT) most commonly due to a chromosomal translocation that fuses the NUT gene on chromosome 15 with the BRD4 gene on chromosome19. It has been described mainly in younger individuals in the mediastinum and head and neck regions and known to be highly aggressive with poor outcomes. We report the case of 23 years old male, diagnosed with locally advanced mediastinal malignancy metastatic to the lung with elevated serum alpha-fetoprotein (AFP) suggestive of germ cell tumor. However, pathology with immunohistochemistry excluded the dignosis of germ cell tumor and confirmed the diagnosis of poorly differentiated carcinoma. Despite aggressive treatment, evolution was marked by rapid clinical deterioration leading to death within 1 month of initial diagnosis. We report this case to underline the rarity of this disease, clinico-radiological and pathologic features, especially misleading presentation with germ cell tumors, treatment management and prognosis.

Clinical characteristics of malignant germ cell tumors in adolescents:A multicenter 10-year retrospective study in Beijing

Background:The aim of this study was to review clinical features of adolescent malignant germ cell tumors(MGCTs)in Beijing and analyze the peculiar characteristics of this age group.Methods:Clinical characteristics,pathological presentations,and survival outcomes of 34 patients were analyzed retrospectively.Results:Of 34 patients,12 girls and 22 boys,18(52.9%)had an extra-cranial tumor,including one testicular tumor,five ovarian tumors,one sacrococcygeal tumor,and 11 mediastinal tumors.Histologically,we found immature teratomas(n=6),yolk sac tumors(n=5),mixed malignant tumors(n=5),an embryonic carcinoma(n=1),and seminoma(n=1).Three-year event-free survival(EFS)and overall survival(OS)were 48.8%and 62.9%,respectively.Another 16(47.1%)patients had an intracranial tumor,including nine in the pineal region,five in the suprasellar region,one in basal ganglia,and one in cerebellopontine.All patients had localized disease and an excellent outcome with 3-year EFS and OS of 93.7%and 100%,respectively.Conclusions:Adolescent MGCTs are rare with a strong dependence on gender,and the mediastina and pineal region are the most common tumor locations.The prognosis is promising compared with that of other adolescent tumors and MGCTs in other age groups.MGCTs in mediastina have a tendency to companion with other hematological malignancies,and the prognosis is extremely poor in these patients.

A single institution, retrospective study of treatment experience in primary mediastinal germ cell tumors： elucidating the significance of systemic chemotherapy

Background Primary malignant germ cell tumors （GCTs） of mediastinum are rare neoplasms.We introduce our institutional experience in managing patients with primary malignant GCTs of the mediastinum,focusing on the analysis of therapeutic modalities.Methods A retrospective review was done in 39 consecutive patients with mediastinal malignant GCTs treated in our institution between 1991 and 2007.Results A total of 39 patients were enrolled in this study with a median age of 27 years.The 5-year overall survival （OS） and progression-free survival （PFS） rates of the whole population were 60.2% and 57.7%,respectively.Stratified by the histology,18 patients （46.2%） had seminoma and 21 patients （53.8%） had nonseminomatous germ cell tumors （NSGCTs）.The 5-year OS rate of patients with seminoma was 87.4% as compared with 36.7% in patients with NSGCTs （P=-0.0004）.The 5-year PFS rate was also significantly higher in seminoma patients （87.4% vs.31.6%,P=-0.003）.For 19 patients with NSGCTs managed with multi-modality treatment,chemotherapy exposure appeared to impact the prognosis.The 5-year OS rate was 44.9% in patients with chemotherapy exposure as compared with 20.0% in patients without it （P=0.43）.Conclusion Our study confirmed the significance of systemic chemotherapy in the treatment of primary mediastinal GCTs.

儿童原发性恶性颅外生殖细胞肿瘤复发的风险因素分析

目的探讨儿童原发性恶性颅外生殖细胞肿瘤(MGCT)复发的危险因素。方法回顾性分析2010年1月—2019年12月郑州大学第一附属医院诊断并治疗MGCT患儿的临床资料,使用Logistic回归法分析其复发的高危因素;使用Kaplan-Meier法统计其总生存率(OS)、无事件生存率(EFS)。结果共109例MGCT患儿,5年的OS率、EFS率分别为(91.3±2.8)%、(86.5±3.4)%;复发率为12.8%,主要的复发部位为骶尾部(9/14)。复发组与未复发组相比,在发病部位(χ^(2)=18.45,P<0.01)、COG分期(χ^(2)=11.32,P<0.01)、是否转移(χ^(2)=12.80,P<0.01)、肝转移(χ^(2)=15.29,P<0.01)和肺转移(χ^(2)=9.57,P<0.001)等方面存在差异。Logistic回归分析示骶尾部为其复发的独立危险因素;复发组的OS(59.1±1.4)%低于未复发组(95.7±2.1)%;未完整切除肿瘤组的OS(65.8±1.4)%低于完整切除组(94.5±2.4)%,差异均有统计学意义。结论复发、未完整切除肿瘤的MGCT患儿预后差,骶尾部为MGCT患儿复发的独立危险因素。

直肠肛管区特殊类型肿瘤的诊治进展

直肠肛管区存在诸如直肠神经内分泌肿瘤、胃肠间质瘤、肛管鳞癌、直肠肛管黑色素瘤、原发性直肠淋巴瘤等特殊类型肿瘤,其临床特征与直肠癌存在差异,且发病率较低,导致临床医师对其认识不足。本文对直肠肛管区特殊类型肿瘤的临床诊疗进展进行综述。

肺癌恶性胸腔积液来源肿瘤细胞的小鼠PDX模型构建及实验验证

目的·构建肺癌患者恶性胸腔积液(malignant pleural effusion,MPE)肿瘤细胞来源的肿瘤异种移植(patientderived tumor xenograft,PDX)模型,并进行实验验证。方法·从基因表达综合数据集(Gene Expression Omnibus,GEO)下载人肺癌伴MPE单细胞转录组测序公共数据GSE131907和人肺癌实体瘤单细胞转录组测序公共数据GSE203360,对数据进行聚类、差异基因本体功能富集分析,明确应用MPE建模的可行性。同时收集肺癌患者的MPE样本,经离心、裂解红细胞等富集细胞操作后,将其植入非肥胖型糖尿病重症联合免疫缺陷(non-obese diabetic/severe combined immunodeficient,NOD/SCID)小鼠皮下,待移植瘤生长至1000 mm³时进行瘤体传代及保存。对稳定传代移植瘤进行组织病理学检测,通过苏木精-伊红染色(hematoxylin-eosin staining,H-E染色)观察细胞组织形态,免疫组织化学法(immunohistochemistry,IHC)检测肺癌标志物表达情况。结果·经单细胞数据分析发现MPE中肿瘤细胞的增殖功能更强,提示MPE中肿瘤细胞PDX建模或具备更佳成瘤效果;共收集35例肺癌MPE样本,成功构建13例PDX模型,成功率达37.14%;在组织病理学检测中,H-E染色可见移植瘤组织细胞异型性明显,IHC检测显示细胞角蛋白7(cytokeratin 7,CK7)、甲状腺转录因子1(thyroid transcription factor-1,TTF1)和天冬氨酸蛋白酶A(Napsin A)等肺癌标志物均呈阳性表达。结论·通过富集肺癌患者MPE中的肿瘤细胞,成功构建了更为简便高效、可实时动态建模的PDX模型。该模型保留了肺癌患者肿瘤细胞的恶性特征及蛋白表达特性,为肺癌伴MPE患者的基础研究和临床用药指导提供了重要的实验模型工具。

Primary Embryonal Lung Carcinoma and Testicular Seminoma in the Same Patient Ten-Years Later: Case-Report and Literature Review

Primary germ cell tumors of lung are extremely rare. The prognosis is usually poor, with various symptoms seriously affecting quality of life. In this paper we describe the unique case of a patient affected by an embryonal carcinoma of lung and a testicular seminoma after ten years. We also report literature about pulmonary extragonadal germ cell tumors.

Acute myeloid leukemia following a primary mediastinal germ cell tumor

Introduction:There is a known association between primary mediastinal germ cell tumor(PMGCT)and hematologic malignancy that is not linked to treatment.They are exceptionally rare entities with a low morbidity and a poor prognosis.Case presentation:An 11-year-old boy presented with an anterior mediastinal mass diagnosed as a malignant germ cell tumor on the basis of an excisional biopsy.He was found to have acute myeloid leukemia(AML)two years after the chemotherapy for his germ cell tumor.The clinical course was very aggressive with a survival time of only 1 week after diagnosis of AML associated with PMGCT.Conclusion:AML associated with PMGCT needs to be diagnosed correctly.Relevant examinations should be carried out in patients with PMGCTs during and after chemotherapy,and long-term follow-up is still necessary to reduce the risk of morbidity and mortality.

DOK3基因表达下调对原代结肠癌细胞生物学行为的影响及其可能机制

目的 分析对接蛋白3(DOK3)基因表达下调对原代结肠癌细胞增殖、侵袭/迁移能力的影响,并基于DOK3与G蛋白偶联受体84(GPR84)的相互作用探讨可能的作用机制。方法 培养原代结肠癌细胞HCT16,将其分为对照组、小干扰RNA(siRNA)-NC组、siRNA-DOK3组进行实验。分别将siRNA-NC、siRNA-DOK3转染至siRNA-NC组、siRNA-DOK3组,而仅将培养液加入对照组。培养24 h后通过实时荧光定量PCR评估转染效果,再使用CCK-8法、Transwell实验分别检测各组细胞的增殖情况、侵袭/迁移能力,采用蛋白免疫共沉淀实验检测HCT16细胞中DOK3与GPR84之间的相互作用关系。结果 与对照组比较,siRNA-DOK3组HCT16细胞中DOK3 mRNA表达量降低(P<0.05),而siRNA-NC组HCT16细胞中DOK3mRNA表达量差异无统计学意义(P>0.05)。培养48 h、72 h后,siRNA-DOK3组HCT16细胞的增殖活力较对照组增加(P<0.05)。与对照组相比,siRNA-DOK3组HCT16细胞的迁移和侵袭数量增加(P<0.05),而siRNA-NC组HCT16细胞的迁移和侵袭数量差异无统计学意义(P>0.05)。蛋白免疫共沉淀实验结果显示,在HCT16细胞中GPR84与DOK3相互结合。结论 DOK3基因可能作为抑癌基因,参与了结肠癌的发生与发展,且其可能通过与GPR84结合发挥抑癌作用。

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≥10000 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。

儿童原发颅内生殖细胞肿瘤延迟诊断的危险因素分析

背景目前对儿童颅内生殖细胞肿瘤(ICGCTs)的及时诊断仍面临困难。目的 分析ICGCTs患儿延迟诊断的影响因素。设计病例对照研究。方法 纳入2011年10月至2021年4月在首都医科大学附属北京世纪坛医院就诊的ICGCTs患儿,发病至诊断时间≥6个月为延迟诊断组,反之为无延迟诊断组。根据导致延迟诊断的原因将延迟诊断组进一步分为疾病原因亚组、家庭原因亚组和诊断原因亚组。截取患儿的一般信息、临床表现、辅助检查等临床资料,在两组间单因素有序Logistic回归分析的基础上行多元Logistic回归分析。主要结局指标ICGCTs延迟诊断的影响因素。结果 158例ICGCTs患儿纳入分析,男114例,女44例。无延迟诊断组88例,延迟诊断组70例,其中疾病原因亚组15例,家庭原因亚组23例,诊断原因亚组32例。起病症状:无延迟诊断组以高颅压三主征多见,疾病原因亚组和家庭原因亚组以内分泌症状多见,诊断原因亚组以神经精神症状和内分泌症状多见。诊断原因亚组以肿瘤多病灶为主,其余各组以单病灶为主。无延迟诊断组病理分型为非生殖细胞性的生殖细胞瘤(NGGCTs)、肿瘤最大直径≥3 cm、发生脑积水、需行脑室腹腔分流术/三脑室底造瘘术的患儿较多,β-HCG≥5 mIU·mL^(-1)在发病≥6个月出现的患儿较少。进一步分析导致延迟诊断的危险因素后发现,内分泌症状是家庭原因亚组患儿延迟诊断的危险因素;诊断原因亚组中高颅压三主征、肿瘤最大直径≥3 cm是患儿延迟诊断的保护因素,β-HCG≥5 mIU·mL^(-1)在发病≥6个月出现是延迟诊断的危险因素。结论 ICGCTs的延迟诊断与疾病自身特征、患儿家庭认知和医院诊疗水平相关,需根据患儿具体情况制定个体化诊疗方案。

原发纵隔生殖细胞瘤相关血液肿瘤1例并文献复习

目的:探讨原发纵隔生殖细胞瘤(primary mediastinal germ cell tumors,PMGCTs)相关血液肿瘤(hematologic malignancies,HMs)的临床特点,以期提高对该病的认识。方法:回顾性分析我院1例PMGCT相关HM患者的临床资料,并结合文献总结该类疾病的临床特点。结果:患者男性,12岁,以乏力并胸部不适为首发症状,行胸部CT增强扫描提示右前上纵隔包块,接受纵隔肿块切除术,病理报告提示混合性生殖细胞瘤。患者外周血染色体核型提示:47,XXY,诊断为PMGCT合并克氏综合征(Klinefelter’s syndrome,KS)。手术切除联合化疗治疗结束后2个月出现难治性血细胞减少,经骨穿等检查诊断为骨髓增生异常综合征(myelodysplastic syndromes,MDS),给予地西他滨治疗获得部分缓解,后因经济原因放弃治疗死亡。对患者纵隔生殖细胞肿瘤(mediastinal germ cell tumor,MGCT)组织和MDS骨髓样本分别行基因检测发现二者均有KRAS基因体细胞突变,突变频率相似。文献复习PMGCTs相关HMs是一种独特的临床综合征,年轻男性易患,PMGCTs和相关的HMs存在共同克隆起源。该病进展迅速,目前无标准治疗,预后极差。结论:PMGCTs相关HMs发病率低,表现为侵袭性病程,对于PMGCTs需密切随访监测HMs,以早期诊断及治疗。

基于SEER数据库构建原发性纵隔生殖细胞瘤患者生存预测的列线图

背景与目的原发性纵隔生殖细胞瘤(primary mediastinal germ cell tumor,PMGCT)是一种相对罕见且偶尔会具有高度侵袭性的纵隔肿瘤。目前对PMGCT的疾病特异性生存期(disease special survival,DSS)的相关研究报道较少,大数据分析亦相对较少,DSS预后模型也较为少见。本研究旨在探讨影响PMGCT DSS的预后相关因素,并构建简便、有效、可对PMGCT患者DSS预后情况进行预测的列线图。方法回顾性分析从监测、流行病学和最终结果(Surveillance,Epidemiology,and End Results,SEER)数据库提取的1975年-2019年共347例PMGCT患者的临床病理资料。采用Kaplan-Meier法及Log-rank检验估计DSS。执行Cox比例风险回归模型筛选影响预后的独立危险因素,构建个体化列线图预测PMGCT患者的3年、5年、8年DSS。通过受试者工作特征(receiver operating characteristic,ROC)曲线、校正曲线及决策曲线分析(decision curve analysis,DCA)评估模型的预测精度。结果PMGCT患者的3年、5年、8年生存率分别为84.6%、83.6%、83.3%。单因素Cox回归分析显示组织学分型、手术与否、年龄、肿瘤大小、肿瘤转移情况及肿瘤分期6项因素可影响PMGCT的预后(P<0.05),多因素Cox回归分析显示组织学分型、手术与否、年龄、肿瘤大小是PMGCT患者预后的独立危险因素(P<0.05),利用这些独立危险因素构建了列线图模型。ROC的曲线下面积(area under the curve,AUC)为0.824,3年、5年、8年生存时间的校正曲线以及DCA曲线,三者结果提示本研究的列线图评估预测结果与真实结果之间有良好的一致性。结论PMGCT中组织学分型为精原细胞瘤的患者比非精原细胞瘤患者预后更佳,年龄>40岁、肿瘤大小≥15 cm且未进行过手术治疗的患者预后不佳。列线图模型可以对PMGCT患者的DSS进行准确直观的预测。

恶性肿瘤经治后继发纵隔淋巴结肿大临床分析

目的分析总结恶性肿瘤经治后继发纵隔淋巴结肿大原因,探讨病理在其诊断中的价值。方法回顾性分析2017年6月至2022年8月北京市垂杨柳医院胸外科收治的44例恶性肿瘤经治后继发纵隔淋巴结肿大行经支气管内超声引导纵隔淋巴结针吸活检术患者的临床资料并进行随访。结果患者中男26例,女18例;年龄38~77岁,平均(61.57±8.76)岁。44例患者初治恶性肿瘤为肺癌21例,食管癌11例,乳腺癌8例,鼻咽癌1例,结肠癌1例,肝癌1例,肾癌1例。所有患者均成功行支气管内超声引导纵隔淋巴结针吸活检。病理结果为恶性35例,其中与初治肿瘤病理相同32例,与初治肿瘤病理不同3例,良性9例。根据穿刺病理结果,将患者分为良性病例组和恶性病例组。采用logistic回归模型进行多因素分析,结果显示,症状(β=2.606,OR=13.538,95%CI:1.517~120.849)是发生恶性的独立危险因素(P=0.020)。恶性组的症状构成比高于良性组,差异有统计学意义(P<0.05);两组患者CT影像学表现中淋巴结的数目比较,差异均无统计学意义(P>0.05)。结论恶性肿瘤经治后出现继发纵隔淋巴结肿大临床上容易误诊,病理是纵隔淋巴结肿大确诊的重要手段。

42例卵巢恶性生殖细胞肿瘤临床特征及预后分析

目的:探讨卵巢恶性生殖细胞肿瘤的临床特征与预后的关系。方法:收集我院2006年8月至2012年2月收治的42例卵巢恶性生殖细胞肿瘤的临床资料,分析其肿瘤类型、分期、治疗方案等预后相关因素,研究其临床特征与不良预后的关系。结果:42例卵巢恶性生殖细胞肿瘤包括混合性生殖细胞肿瘤16例(38.1%)、未成熟畸胎瘤12例(28.6%)、无性细胞瘤6例(14.3%),卵黄囊瘤4例(9.5%)、绒毛膜癌1例(2.4%)及胚胎癌1例(2.4%)等。42例患者全部进行手术治疗,其中30例在初次手术中保留了生育功能,34例在术后给予BEP方案(博来霉素+依托泊苷+顺铂)或BVP方案(博来霉素+长春新碱+顺铂)化疗。2年生存率97.5%(39/40),死亡1例(Ⅳ期混合性生殖细胞肿瘤),复发率37.5%(15/40),混合性生殖细胞肿瘤复发占66.7%(10/15);5年生存率88.2%(15/17),死亡2例(均为恶性混合性生殖细胞肿瘤)。不同病理分期、肿瘤直径、肿瘤类型和手术方式的5年生存率比较,差异均无统计学意义(P>0.05)。结论:保留生育功能手术及规范化的BEP或BVP方案化疗,5年总体生存率较高,但混合性生殖细胞肿瘤预后相对较差。卵巢恶性生殖细胞肿瘤的病理分期、肿瘤直径及肿瘤类型与生存率的关系还需进一步研究。

原发性纵隔恶性生殖细胞瘤2例并文献复习

目的提高对原发性纵隔恶性生殖细胞瘤(primary malignant mediastinal germ cell tumor,PMMGCT)的认识。方法回顾分析2例PMMGCT患者的临床资料,并检索复习文献。结果病例1,男性,22岁,咳嗽、呼吸困难5个月,胸部CT显示前纵隔巨大肿物,血β-人绒毛膜促性腺激素(β-human chorionic gonadotropin,β-HCG)升高,超声引导穿刺病理为精原细胞瘤。病例2,男性,20岁,胸痛10 d,胸部CT显示前纵隔巨大肿物,血甲胎蛋白(α-fetoprotein,AFP)明显升高,CT引导穿刺病理为纵隔卵黄囊瘤。回顾文献,PMMGCT多见于青年男性,症状缺乏特异性,胸部CT可见巨大肿物多位于前纵隔,内部可见低密度囊性改变和钙化。精原细胞瘤血β-HCG升高,卵黄囊瘤血AFP明显升高。PMMGCT对化学药物治疗(以下简称化疗)敏感,首选含铂类的联合化疗,后可辅以手术或放射治疗清除残留病灶。结论 PMMGCT临床罕见,呼吸科和放射科医师应提高认识,为患者争取较好的预后。