颅内生殖细胞瘤联合化疗的临床作用

采用长春新碱（ＶＣＲ）、甲氨喋呤（ＭＴＸ）、平阳霉素和顺铂（ＰＤＤ）四种药物静脉给药对８例颅内生殖细胞瘤进行联合化疗，皆取得满意的疗效，其中４例肿瘤完全消失，４例肿瘤缩小９０％￣９５％。化疗结束后１￣２月再辅以局部放疗，至今随访已２￣１５月，全部病人症状消失或明显好转，多数已恢复上学或工作。     

放、化疗对颅内生殖细胞瘤的效应

颅内生殖细胞瘤占颅内肿瘤的1．5％～2．4％，具有病程短、易播散、对放射线高度敏感等特点。我科自1992年以来采用放疗联合化疗治疗12例颅内生殖细胞瘤取得了满意的疗效，现将结果报告如下。     

颅内生殖细胞瘤联合化疗的临床应用

采用长春新碱（ＶＣＲ）、甲氨蝶呤（ＭＴＸ）、平阳霉素和顺铂（ＰＤＤ）四种药物静脉给药对８例颅内生殖细胞瘤进行联合化疗，皆取得满意的疗效，其中４例肿瘤完全消失，４例肿瘤缩小９０％～９５％。化疗结束后１～２月再辅以局部放疗，至今随访已２～１５月，全部病人症状消失或明显好转，多数已恢复上学或工作。     

颅内生殖细胞瘤的诊断及综合治疗

目的 探讨颅内生殖细胞瘤的诊断及综合治疗方法与疗效。方法 本组颅内生殖细胞瘤31例中，29例手术治疗(含1例行脑室—腹腔分流，3例行脑室外引流)，2例未手术。此外，29例进行了放疗，8例进行了化疗。结果 临床症状改善27例，无变化4例。随访27例，13例恢复正常工作；8例生活能自理；2例生活需要帮助；2例术后2年复发，经再次手术，症状缓解；死亡2例。结论 颅内生殖细胞瘤手术难以根治，术后正规放疗效果好，对多发性，复发性肿瘤可放疗加化疗。     

颅内生殖细胞瘤综合治疗

报告５０例颅内生殖细胞瘤，手术治疗１９例，放射治疗４３例。综合治疗后临床症状改善４１例，无效２例。随访３２例，其中恢复正常工作或学习者１４例，生活能自理１２例，生活部分需人帮助４例，死亡２例。５年生存率为５０％。推荐放疗为首选治疗手段，并对合并颅内压增高及多发性、复发性肿瘤的治疗作了讨论。     

颅内生殖细胞瘤诊疗的一些误区

我院统计原发于颅内的生殖细胞肿瘤占颅内肿瘤总数的1.9%,日本的发病率较高(4%～9%)。这是一组临床上很有特点的肿瘤。它多发生于儿童和青少年,男性明显多于女性,主要发生于松果体区和鞍上,第三位是基底节区。由于我国的不少医生对本病的认识尚不充分,漏诊和误诊现象     

颅内高度恶性非生殖细胞瘤性生殖细胞肿瘤

目的探讨颅内高度恶性非生殖细胞瘤性生殖细胞肿瘤（non-germinomatous malignant germ cell tumors,NGMGCTs）的临床特点、治疗和预后.方法回顾性分析了19例高度恶性NGMGCTs患者的临床特点、血清肿瘤标记物监测、治疗方法以及预后.其中胚胎癌2例,卵黄囊瘤4例,绒毛膜上皮癌7例,混合性生殖细胞肿瘤6例.有7例术前肿瘤标记物监测均异常,所有患者均行手术切除肿瘤,全切除7例,近全切除10例,大部分切除2例.所有患者均行放疗,有11例行术后化疗.结果共随访到17例患者,平均随访3.5年,1年生存率为29.4%,3年生存率为5.9%.其中卵黄囊瘤1年生存率为25.0%;混合性生殖细胞肿瘤3年生存率为20.0%,胚胎癌和绒毛膜上皮癌患者皆于1年内死亡.结论高度恶性NGMGCTs预后很差.根据临床表现、影像学资料和肿瘤标记物甲胎蛋白（AFP）和（或）β-绒毛膜促性腺激素（β-HCG）增高,多数患者在术前可确诊为高度恶性NGMGCTs.治疗应先采用化疗后放疗,对治疗后肿瘤标记物水平降至正常而影像学上肿瘤仍未完全消失者,可考虑手术切除,术后再行化疗和（或）放疗.治疗结束后注意随诊,如肿瘤标记物再次升高,经影像学证实肿瘤复发,可再行化疗和手术治疗.这样会延长高度恶性NGMGCTs患者的生存期.     

9例生殖细胞瘤的临床及影像学分析

颅内生殖细胞瘤约占颅内肿瘤的1.5%,主要发生在松果体区及鞍区,也有发生在基底节区及大脑半球的生殖细胞瘤,本组9例生殖细胞瘤均经病理证实,本文结合文献就其临床及影像学进行分析如下。     

颅内生殖细胞瘤的CT和MRI表现

目的探讨颅内生殖细胞瘤的CT和MRI影像特征。方法回顾性分析18例经病理或放射治疗证实的颅内生殖细胞瘤的CT和MRI表现。结果 18例肿瘤中,9例肿瘤位于松果体区,6例位于鞍区,3例位于基底节区及丘脑。松果体区肿瘤在MRI呈长T1、等或长T2信号,边缘光滑,出血少见;CT呈均匀等密度或稍高密度,松果体钙化常被肿瘤包埋,增强扫描时肿瘤均匀显著强化。鞍区肿瘤MRI表现为垂体柄增粗,垂体后叶正常短T1信号消失,肿瘤在T1WI像呈等信号,T2WI像上信号可呈等或高信号;CT图像见肿瘤实体部分呈高密度,增强扫描时明显强化。基底节区及丘脑肿瘤瘤体较大,信号及密度不均匀,边缘欠清晰,出血多见,增强扫捕呈不均匀强化。结论颅内生殖细胞瘤的影像表现具有一定特征,结合临床表现可进一步提高诊断正确率。     

立体定向放射外科针对颅内非生殖性生殖细胞瘤常规放疗后残留病灶的疗效分析

目的 探讨立体定向放射外科(SRS)治疗松果体区非生殖细胞瘤性生殖细胞肿瘤(NGGCTs)常规放疗后残留病灶的有效性和安全性。方法 回顾性分析2008年1月—2018年12月广东三九脑科医院肿瘤中心收治的颅内生殖细胞肿瘤患者临床资料,按纳入标准筛选患者：(1)病理或临床诊断为NGGCTs;(2)病灶位于松果体区;(3)临床分期为局限期;(4)松果体病灶经过常规放疗50～54 Gy/25～30 f后可见肿瘤残留(病灶最大径直径>10 mm)。分析入组患者SRS治疗后肿瘤局部控制率、无进展生存时间、总生存时间及治疗相关毒性。结果 符合标准并纳入分析患者共27例,均为男性患者,中位年龄16岁(8～31岁);随访至2019年12月30日,2例失访,中位随访时间34个月(8～142个月);SRS治疗后1个月客观反应率71.4%,疾病控制率为95.2%;3年无进展生存时间、3年总生存期分别为85.2%、88%;单因素分析显示年龄、同步化疗与预后无关(P=0.286、0.824);放化疗前肿瘤部分切除术、AFP>500 ng/mL、辅助化疗周期数≤4是不利预后因素(P=0.037、0.0...     

Radiotherapy and chemotherapy plus radiation in the treatment of patients with pure intracranial germinoma: A meta-analysis

ObjectiveTo evaluate the efficiency of radiotherapy (RT) only and chemotherapy plus radiotherapy (CRT) strategy in the treatment of pure intracranial germinoma.MethodsWe searched PUBMED, EMBASE, Medline and Cochrane library up to May 2016 for studies that enrolled patients with pure intracranial germinoma receiving either RT only or CRT treatment as their first-line treatment. The meta-analysis was conducted on the overall survival rate (OS) and disease free survival (DFS) at 3 years and 5 years. The outcomes were pooled using a random-effect model.ResultsThe final search included 15 studies with 310 patients. The pooled 3-year OS (97% vs. 94%, p = .000) and 3-year DFS (96% vs. 93%, p = 0.043) of CRT group was significantly higher than that of RT only group. However, at 5 years, the OS was 94% in RT only group and 92% in the combined group (p = 0.29) . For DFS, the RT only group was higher than the combined group (94% vs.89%, p = .000).ConclusionsBoth RT and CRT for intracranial pure germinoma gain satisfying outcomes, and the CRT strategy has a higher overall survival rate and disease free survival rate at 3 years than RT regimen. At 5 years in the postoperative period, the advantage of survival rates for CRT is eliminated or even reversed. For patients with pure intracranial germinoma, especially those with acute and severer condition and poorer prognosis, CRT strategy would be a better choice.     

颅内多灶性生殖细胞肿瘤的临床特点及诊治

目的：总结颅内多灶性生殖细胞肿瘤的临床特性,并探索合理的治疗方案。方法回顾性分析27例颅内多灶性生殖细胞肿瘤病人的临床资料,颅内共有病灶69个,以同时位于松果体区和鞍上区最常见。最大肿瘤灶位于松果体区15例,鞍上区11例,额叶1例。单纯血浆β-人绒毛膜促性腺激素（β-HCG）增高7例,单纯甲胎蛋白（AFP）增高1例,β-HCG 和 AFP 同时增高1例。接受放疗24例,其中8例联合化疗;放弃治疗3例。结果确诊后接受治疗的24例中,3例完成放疗,正在化疗;检查显示肿瘤病灶均已消失。余19例随访6个月~6.25年,平均3.75年;死亡1例,复发1例,其余17例无复发征象,均能够正常生活。失访2例。结论颅内多灶性生殖细胞肿瘤主要为生殖细胞瘤,考虑系种植转移所致,如能强化治疗,包括全脊髓放疗,预后多良好。     

鞍区生殖细胞瘤的早期诊断及综合治疗

目的 总结颅内鞍区生殖细胞瘤的早期诊断及综合治疗的经验.方法 回顾性分析23例高度怀疑鞍区生殖细胞瘤病人的临床资料.9例病人经手术证实为生殖细胞瘤后进一步行放化疗;14例通过临床特点、血清学和影像学检查、诊断性放疗后明确为生殖细胞瘤,直接进行放化疗.结果 随访23例,时间11～57个月,平均23.8个月.肿瘤复发1例,化疗后肿瘤消失.8例血HCG升高病人均恢复正常.所有病人头痛、视力减退等症状显著缓解,多饮多尿等内分泌异常症状仍存在.结论 鞍区生殖细胞瘤通过早期诊断和放化疗等综合治疗达到满意疗效.     

Delayed resolution of intracranial germinoma after radiotherapy: A preliminary study of the correlation between histology and magnetic resonance imaging

A central nervous system germinoma is curable in most cases by radiotherapy, and most of the tumor mass disappears promptly after 20–30 doses. However, some germinomas take a longer period to vanish completely from magnetic resonance imaging (MRI) or computed tomography (CT) scans. In such cases, the tumor may contain components such as teratoma. The aim of this study was to clarify the nature of the persistence of some germinomas. Five cases of histologically verified germinoma in which radiotherapy was performed to treat residual tumor were selected. The doses of focal radiotherapy and whole brain radiotherapy were 10–20 Gy and 20–34 Gy, respectively. In these cases, correlation was made between the degree of persistence of the tumor when assessed by MRI and the amount of interstitial content, as determined by histology. The histological evaluation, using hematoxylin–eosin stain, silver impregnation and Azan staining was carried out independently of clinical information. The tumor vanished soon after radiotherapy in three cases, but 3–15 months passed before the tumor completely vanished from the MRI scans in the other two cases. The histology of the cases in which the tumor disappeared rapidly was predominantly of large tumor cells and only small amounts of reticulin. However, in the persistent tumors, large amounts of reticulin and vascular components were present. Thus, it is proposed that tumors with a large parenchymal component disappear soon after radiotherapy, whereas tumors composed mainly of interstitial component persist. Long‐standing enhancement seen on MRI or CT scans of patients with an intracranial germinoma is indicative of a large amount of interstitial component in the tumor.     

Pineal germinoma: Diagnosis, treatment and tumor response

We report the case of pineal germinoma in a 25 year old man. The neurological signs and symptoms were insidious in onset; Parinaud syndrome allowed a topical diagnosis, confirmed by CT scan. Computerized Tomography and CSF cytological examination were of the utmost importance to diagnosis and treatment and it confirmed the success of radiotherapy.

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Sommario Viene riportato un caso di un giovane paziente affetto da germinoma della pineale. La TAC si rivelò di enorme importanza nel definire la sede anatomica della lesione. L'esame citologico liquorale permise di definire la natura della lesione e diede così la possibilità di stabilire un corretto approccio terapeutico e di confermare il successo del trattamento radioterapico.

     

基底节区生殖细胞肿瘤一例并文献复习

目的探讨基底节区生殖细胞肿瘤(basal ganglia germinoma,BGG)临床和影像学特点,以提高对早期BGG的认识。方法分析作者医院收治的1例BGG患者的临床资料及诊治、转归情况并复习相关文献。结果患者男性,17岁,病程28个月。发病时表现为智能减退、左侧肢体痉挛性瘫痪。发病7个月时头颅MRI未见明确占位征象;发病28个月时头颅MRI发现右侧基底节区巨大囊实性占位病变,左侧基底节区小病灶。双侧基底节区MRS分析发现,右侧胆碱(Cho)峰显著升高,肌酐(Cr)和N-乙酰天冬氨酸(NAA)峰明显降低,Cho/Cr增加;左侧Cho峰明显升高,Cr和NAA峰无明显变化,Cho/Cr>1。血人绒毛膜促性腺激素(β-HCG)和甲胎蛋白(AFP)水平明显增高,分别为3481.4mIU/mL和32.1ng/mL。临床诊断为生殖细胞肿瘤,行开颅手术将右侧病变大部切除。病理证实为混合性生殖细胞肿瘤。结论 (1)患者可能有双侧BGG;(2)BGG早期诊断困难,根据临床和MRI表现,动态观察血和脑脊液中β-HCG和AFP的变化,必要时行进一步的影像学检查(MRS或11 C-蛋氨酸PET/CT)及病灶活检,以提高早期诊断率。     

A simple method of predicting hormonal outcome in children with intracranial germinoma

Even a small intracranial germinoma (GE) frequently provokes pituitary hypofunction. We evaluated the relationships between preoperative hormonal status and hormonal outcome and between size of suprasellar mass and hormonal outcome in 22 children with intracranial pure GE to determine whether or not these can reflect the degree of hypothalamo-pituitary axis destruction. Preoperative hormonal status was graded from I to IV according to the serum prolactin level (s-PRL) and thyroid function (TF). The hormonal outcome was estimated by the type and the number of hormonal replacement medications taken on the basis of the triple load test and endocrinological examinations at the time of the last follow-up (median period: 43 months). Fifteen of the 22 patients had suprasellar lesions. All 13 patients who had diabetes insipidus (DI) at presentation needed desmopressin acetate (DDAVP) during the follow-up period. The correlation between increment of preoperative grade and the increment in the number of hormones to be replaced was statistically significant (P<0.05). This preoperative grading was a more reliable predictor than the size of suprasellar tumor. In conclusion, preoperative grading by s-PRL and TF test is useful for the prediction of posttreatment hormonal replacement in children with intracranial pure GE. Received: 10 July 1998 Revised: 30 August 1998     

Primary intracranial germinoma involving the midbrain

To our knowledge, this is the first reported case of a germinoma involving the midbrain without the demonstrable coexistence of any common midline tumors. A 27-year-old man was referred to our institution for evaluation and treatment of diplopia persisting for 5 years. Magnetic resonance imaging (MRI) showed the mass in the midbrain to be of iso intensity on T1-weighted images, and of high intensity on T2-weighted images with homogeneous enhancement. MRI-guided stereotactic biopsy was performed, and the histologic diagnosis was germinoma. Following biopsy, external beam radiotherapy of 50 Gy (whole brain 30 Gy; local 20 Gy) was performed. At the time of discharge, the patient's neurological symptoms had resolved. Follow-up MRI revealed disappearance of the tumor. These findings suggest the diagnostic value of magnetic resonance image-guided stereotactic biopsy in the differential diagnosis of adult brainstem lesions, which should now include germinoma.     

Recurrent intracranial germinoma with dissemination along the ventricular catheter: a case report

Most recurrences of intracranial pure germinoma occur at the primary site, ventricular wall or subarachnoid space. We report a rare case of intracranial germinoma that recurred along the shunt tube 17 years after prior ventriculoperitoneal shunt and radiotherapy. The recurrent tumor, verified histologically as a pure germinoma, involved the right frontal lobe along the ventricular catheter. In spite of subsequent surgery, radiation and chemotherapy, the tumor recurred repeatedly. Recurrence after such a long period highlights the necessity of long-term follow up for patients with germinoma. We also discuss possible causes of tumor dissemination along the ventricular catheter.     

X-刀治疗颅内疾病128例报告

应用X-刀治疗颅内巨大肿瘤及脑血管畸形128例,以中、小病灶为主,亦可作为颅内巨大肿瘤综合治疗的一部分.强调了最大剂量与覆盖剂量比及治疗体积与靶体积比两项比值的重要性.治疗效果的观察,早期主要靠CT扫描检查,一般表现为肿瘤部分失增强效应.