The clinic follow-up on chemotherapy and radiotherapy of children intracranial germinoma

Objective To study the clinical effects of chemotherapy and radiotherapy of children intracranial germinoma. Methods From December 1993 to December 2001, we counducted chemotherapy and radiotherapy to 63 children with intracranial germinomas. 34 patients have been followed up. Out of them, 20 cases have tumors in the pineal region, 6 cases have tumors in the pineal and suprasellar regions, 4 cases have tumors in the suprasellar region, 3 cases have tumors in the pineal and cerebral ventricle, 1 case has tumor in the hasal ganglia. The drugs we used are VCR, MTX, Bleomytin and Cisplatin (DDP). Four days are considered as one course of treatment. A repeat course was taken after 4 weeks. CT and/or MRI were repeated 1 month after the second course. Local radiotherapy was taken with low or middle dosage (25-35 Gy) 1-2 months after chemotherapy. Results 34 children have been followed up from 1 to 7 years. One child died within 4 years after chemotherapy. The other child has tumor relapsing 5 years after

Application of proton MR spectroscopy on evaluation of radiotherapy treated intracranial tumors

Objective： To evaluate the clinical application of proton magnetic resonance spectroscopy （1HMRS） in patients with radiotherapy treated intracranial tumors. Methods： Forty patients with intracranial tumors underwent multivoxel 1HMRS examination before and after radiotherapy. The concentrations of N-acetyl aspartate （NAA）, choline （Cho） and creatine （Cr） were obtained both in the tumors and the contralateral normal brain regions, The ratios of NANCr, Cho/Cr and Cho/NAA were calculated at the same time and follow-up one year. Results： （1） After radiotherapy, tumors inhibited by radiation had decreased Cho, NAA and Cr on proton MRS. Some cases showed necrotic wave. （2） During the one year follow-up, local tumor recurred in 8 cases and their Cho and Cho/NAA increased high again. Other cases without recurrence, HMRS showed no change. Conclusion： Multivoxel proton MR spectroscopy is available for study of tumor metabolites after radiotherapy and it is a valuable method in the evaluation of radiotherapy treated tumors,

Role of MR imaging in the diagnosis of intracranial germinoma

Objective: To investigate the role of MRI in the diagnosis of intracranial germinoma. Methods: MRI features of 19 cases of intracranial germinoma confirmed by operations and pathological findings were analyzed retrospectively. Results: Germinomas were found in the sellar region in 10 patients (including 5 males and 5 females), in the pineal region in 6 and in the thalamus and basal ganglia in 3, the 9 patients in the latter 2 groups all being males. The characteristic MRI findings of intracranial germinomas were as follows: (1) Lesions were isointense or slightly hypointense on T1WI while isointense or slightly hyperintense on T2WI. The germinomas in the sellar region and pineal region showed no edema, but lesions in the thalamus basal ganglia showed mild to moderate edema and space-occupying effects. (2) Homogeneous or inhomogeneous Gd-DTPA enhancement were seen in most of the tumors. Conclusion: Multiaxial imaging and Gd-DTPA enhancement in MRI are helpful in the diagnosis and differentiation of intracranial germinomas on the basis of the patient's gender, the location of the tumor and its imaging characteristics.

Primary intracranial extraskeletal myxoid chondrosarcoma:A case report and review of literature

BACKGROUND Primary intracranial extraskeletal myxoid chondrosarcoma(EMC)is an extremely rare low-to intermediate-grade malignant soft tissue sarcoma,and only 15 cases have been reported in the literature.Due to its rarity,clinical data and research on this tumor type are extremely limited,the pathogenesis and histological origin are still unclear,and the diagnostic and standard clinical treatment strategies for intracranial EMC remain controversial and undefined.CASE SUMMARY We reported a case of a 52-year-old male who was admitted to the hospital with headache and dizziness for 1 mo,and his health status deteriorated during the last week.CT of the head showed a well-defined low-density lesion situated in the left cavernous sinus.Brain magnetic resonance imaging(MRI)showed a 3.4 cm×3.0 cm sized,well-defined,round-shaped and heterogeneously enhanced lesion located in the left cavernous sinus.The entire lesion was removed via supratentorial craniotomy and microsurgery.Postoperative pathological diagnosis indicated primary intracranial EMC.Subsequently,the patient underwent 45 Gy/15 F stereotactic radiotherapy after discharge.At present,it is 12 mo after surgery,with regular postoperative follow-up and regular MRI examinations,that there are no clinical symptoms and radiographic evidence indicating the recurrence of the tumor,and the patient has returned to normal life.CONCLUSION Currently,the most beneficial treatment for primary intracranial EMC is gross total resection combined with postoperative radiotherapy.Long-term follow-up is also necessary for patients.

Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report

BACKGROUND Primary intracranial alveolar soft-part sarcoma(PIASPS)is a rare malignancy.We aimed to investigate the clinical profiles and outcomes for PIASPS.CASE SUMMARY We firstly reported five consecutive cases from our institute.Then,the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease.Our cohort included two males and three females.The median age was 21-years-old(range:8-54-years-old).All the patients received surgical treatment.Gross total resection(GTR),radiotherapy,and chemotherapy were administered in 3 patients,4 patients,and 1 patient,respectively.After a median follow-up of 36 mo,tumor progression was noticed in 4 patients;and 3 patients died of the disease.Pooled data(n=14)contained 5 males and 9 females with a median age of 19 years.The log-rank tests showed that GTR(P=0.011)could prolong progression-free survival,and radiotherapy(P<0.001)resulted in longer overall survival.CONCLUSION Patients with PIASPS suffer from poor outcomes.Surgical treatment is the first choice,and GTR should be achieved when the tumor is feasible.Patients with PIASPS benefit from radiotherapy,which should be considered as a part of treatment therapies.

Meningeal Hemangiopericytoma: A Rare Intracranial Tumor—About a Case

Introduction: Hemangiopericytoma is a very rare vascular tumor, difficult to diagnose, often confused with a meningioma. Imaging is not very specific and the diagnostic certainty remains histological. Observation: We report a case of meningeal hemangiopericytoma in a 48-year-old patient with a history of active smoking and whose sister died of glioblastoma. He was brought to the emergency room for a fall, confusion, and tonic-clonic seizures. The diagnosis was suspected on imaging (CT and MRI, in front of a syndrome of right temporal intraventricular mass measured at 37 mm in diameter, centered on the choroid plexus, with perilesional vasogenic edema and posterior white commissure. There was homogeneous enhancement of the lesion after gadolinium injection. The diagnosis was confirmed by immunohistochemical study, which showed diffuse expression of STAT6 and partial labeling for CD34, without significant expression of SSTR2. Expression of neurofilament protein was also found in the brain parenchyma. Conclusion: Hemangiopericytoma is a rare tumor, characterized by its malignant potential, its high rate of recurrence and distant metastasis. The management is based on a total microsurgical resection followed by radiotherapy.

Germinomas of the basal ganglia and thalamus: Four case reports

BACKGROUND The early diagnosis of basal ganglia and thalamus germinomas is often difficult due to the absence of elevated tumor markers,and atypical clinical symptoms and neuroimaging features.CASE SUMMARY Four male children aged 8 to 15 years were diagnosed with germinomas in the basal ganglia and thalamus by stereotactic biopsy from 2017 to 2019.All patients developed hemiplegia except patient 4 who also had cognitive decline,speech disturbance,nocturnal enuresis,polydipsia,polyuria,precocious puberty and abnormalities of thermoregulation.All four cases were alpha-fetoprotein and beta-human chorionic gonadotrophin(p-HCG)negative except patient 3 who had slightly elevated p-HCG in cerebrospinal fluid(CSF).No malignant cells were detected in the patients'CSF.Brain magnetic resonance imaging findings were diverse in these patients with the exception of the unique and common characteristics of ipsilateral hemisphere atrophy,especially in the cerebral peduncle.All patients were diagnosed with germinomas of the basal ganglia and thalamus by stereotactic brain biopsy.CONCLUSION Stereotactic brain biopsy is necessary to confirm the diagnosis of ectopic germinomas.Serial neuroimaging studies can not only differentiate disease but also determine the biopsy site.

Diabetes insipidus with impaired vision caused by germinoma and perioptic meningeal seeding:A case report

BACKGROUND Germinoma is a type of germ cell tumor that most frequently arises in the midline axis of the brain.Impaired vision is a clinical manifestation of germinnoma.Although rare,intracranial germinoma seeding to the perioptic arachnoid space is one cause of visual acuity decrease.CASE SUMMARY An 11yearold girl who presented with polyuria and polydipsia and subsequently developed diminution of vision.Imaging showed bilateral heterogeneous enhancement of the optic nerve sheaths and atrophy of the optic nerve,and transsphenoidal biopsy revealed a germinoma.The patient experienced poor visual recovery following chemotherapy and radiotherapy.Germinomas are rare and they are mostly identified in children and adolescents.The manifestations include diabetes insipidus,pituitary dysfunction,visual complaints,etc.The mechanisms that lead to visual loss include intracranial hypertension,compression of optic chiasma,and tumor invasion.A literature review was performed to summarize the cases with a tumor infiltrating the optic nerve.Most of the reported patients were adolescents and presented with anterior pituitary hormone deficiency.Enhancement of optic nerve sheaths and optic disc pallor could be identified in most of the cases.The purpose of this report is to provide awareness that in cases where a germinoma is associated with visual loss,though rare,perioptic meningeal seeding should be taken into consideration.CONCLUSION The case report suggests that children with diabetes insipidus need a complete differential diagnosis.

Ocular manifestations of intracranial germinomas： three cases report and literature review

Intracranial germinomas are malignant neoplasms of gonadal origin, which have some features in terms of age, sex, and clinical manifestations. They mainly occur in children and adolescents. Patients with intracranial germinomas mainly manifest with hypothalamic pituitary axis dysfunction and/or compression syndromes. Visual disturbance is one of the most Significant clinical presentations, which is mainly caused by tumor cell infiltration into the optic pathway. In this article, we present three cases of patient with intracranial germinoma to analyze the ocular manifestations. All the three patients presented with endocrine symptoms in the early stage and with visual disturbances （including decreased visual acuity and visual field defects） later. In general, germinoma is diagnosed by its characteristic radiological appearance, supported by tumor markers and/or stereotactic biopsy. However, decisive diagnoses were established when ocular manifestations were presented. A suspicion for germinoma should be considered, when young patients manifest visual disturbances accompanied by endocrine symptoms.

Clinical outcomes for nasopharyngeal cancer with intracranial extension after taxanebased induction chemotherapy and concurrent chemo-radiotherapy in the modern era

Objective:To evaluate the survival outcomes for a cohort of nasopharyngeal cancer with intracranial extension(ICE)treated with induction chemotherapy(ICT)followed by chemo-intensity-modulated radiotherapy(CTRT)at a tertiary cancer center.Methods:We retrospectively analyzed 45 patients with histologically proven,non-metastatic NPC with ICE treated at our institute between October 2008 and October 2016.Patients were classified as minor ICE or major ICE,based on the extent of ICE.All the patients received 2-3 cycles of a taxane-based ICT regimen followed by CTRT.Radiotherapy was delivered with"riskadapted"intensity-modulated radiotherapy(IMRT)technique in all patients.Results:After a median follow up of 45 months(range:8-113 months),the estimated 5-year DFS,LRFS,DMFS,and OS of the entire cohort was 58%,82%,67%and 74%respectively.On multivariate analysis,histological subtype was an independent predictor of LRFS,and age was an independent predictor of DFS.The extent of ICE showed only a trend towards worse DFS(P=0.06).None of the factors significantly predicted for DMFS or OS.Gender,N-stage,and response to ICT did not significantly affect any of the outcomes.Grade 2 or worse subcutaneous fibrosis was seen in 22%of patients and grade 2 or worse xerostomia was seen in 24%of patients at last follow up.Thirty-three percent of the patients developed clinical hypothyroidism at last follow up.None of the patients experienced any neurological or vascular complications.Conclusions:Taxane-based induction chemotherapy followed by chemo-intensity modulated radiotherapy resulted in excellent locoregional control and survival with acceptable toxicities in patients of nasopharyngeal cancer with intracranial extension.Distant metastasis continues to be the predominant problem in these patients.

颅内原发无性细胞瘤伴继发性闭经1例

颅内原发性无性细胞瘤是一种罕见的肿瘤,主要发生在青少年,好发于松果体区和蝶鞍区,其临床表现主要与肿瘤位置、大小相关。大多数生殖细胞瘤对放疗和化疗非常敏感,早期治疗对减少并发症和病死率至关重要。由于其发病率较低,因此目前国内外针对颅内原发无性细胞瘤的诊治还没有一个统一的标准。本文报道一例颅内原发性无形细胞瘤,为20岁女性,因停经9个月入院,行神经内镜下肿瘤切除术,术后病理证实为无性细胞瘤。术后第6周开始“全脑+全脊髓”普通放射治疗,DT 30 Gy×15次(5次/周);随后,开始“全脑加量”普通放射治疗,DT 24 Gy×12次(5次/周)。病人入院诊断垂体功能减退症后,规律口服醋酸泼尼松及左甲状腺素钠替代治疗,放射治疗完成后3个月,MRI显示鞍区未见明显结节、肿块影,但月经尚未恢复。这提示颅内原发性无性细胞瘤病人早期正确诊治对后期激素紊乱的治疗非常重要。

TREATMENT OF INTRACRANIAL GERMINOMA

In 31 patients with intracranial germinoma who received radiotherapy, 15 were operated on, and 13 were treated with CSF shunt (7) and external ventricular drainage (6). After radiotherapy, clinical symptoms were improved in 93.5% of the patients. Follow-up of 16 patients for 2 to 14 years showed better results. We consider that initial treatment with radiotherapy may be appropriate for germinomas because of its high radiosensitivity. If hydrocephalus developed, external ventricular drainage combined with radiotherapy was performed, but shunting operation might be avoided because of potential peritoneal seedings of germinomas. In addition to chemotherapy, re-irradiation is an effective method for recurrent germinoma.

45例颅内生殖细胞瘤放射治疗的临床分析

目的:报告45例颅内生殖细胞瘤放射治疗的远期疗效并探讨其治疗方法。方法:分析1998年2月至2007年10月中南大学湘雅医院肿瘤科收治的45例颅内生殖细胞瘤患者,均行放射治疗,其中15例接受化疗。病理诊断23例,临床诊断22例。寿命表法计算5年,10年生存率。结果:40例获得随访,患者大部分症状明显减轻或完全消失,5年,10年总生存率分别为84%,74%。结论:放射治疗是颅内生殖细胞瘤主要的治疗手段,全中枢或全脑+局部放射治疗是颅内生殖细胞瘤的主要治疗方式,放射治疗联合化疗以减少放射治疗范围,降低放射治疗剂量是今后的发展趋势之一。

低剂量诊断性放疗联合化疗在诊治颅内生殖细胞肿瘤中的价值

背景与目的:颅内生殖细胞肿瘤(intracranial germ cell tumors)经积极放化疗常可获得令人满意的局部控制率和生存率,然而对部分无法取得病理诊断的颅内生殖细胞肿瘤的治疗,研究者们至今无法达成一致意见。该研究探讨低剂量诊断性放疗联合化疗在诊治颅内生殖细胞肿瘤的可行性及临床意义。方法:该研究分析了贵州省人民医院及北京天坛医院收治的28例(16例男性,12例女性,中位年龄14.5岁)高度怀疑为颅内生殖细胞肿瘤的患者。患者血浆和(或)脑脊液人绒毛膜促性腺激素(β-human chorionic gonadotropin,β-HCG)和甲胎蛋白(α-fetoprotein,AFP)均为阴性,头颅MRI诊断提示鞍区和(或)松果体区呈典型生殖细胞肿瘤影像特征,临床表现高度提示可能为生殖细胞瘤(germinoma)。这些患者因无法通过手术及立体定向活检明确病理诊断,或患者本身不同意手术或活检,因此我们建议实施诊断性低剂量放疗联合化疗方案,诊断性放疗剂量为3.4 Gy,1.7 Gy/2次。经低剂量诊断性放疗后行MRI复查。随后根据MRI复查结果采用以下治疗:⑴影像学为进展(progression disease,PD)或稳定(stable disease,SD),建议手术治疗。⑵影像学达完全缓解(complete response,CR)或部分缓解(partial release,PR),再予以2个周期化疗,顺铂+足叶乙甙(DDP+VP16)方案2个周期,化疗后再次行MRI复查,如果患者影像学检测结果显示达CR,随即按生殖细胞瘤进行调强放疗(intensity-modulated radiation therapy,IMRT)及三维适形放疗(three-dimensional conformal radiotherapy,3D-CRT)。放疗方案根据患者情况进行选择:(1)局部放疗;(2)全脑全脊髓+局部加量放疗;(3)全脑+局部加量放疗(松果体区病变总剂量小于等于50.4 Gy;鞍区病变总剂量小于等于41.0 Gy)。⑶经低剂量诊断性放疗联合化疗后行MRI复查,如果患者影像学仍未达CR,建议患者立即接受手术治疗。参考WHO实体瘤疗效评价标准判断放化疗效果。结果:经诊断性放疗后显示为SD的患者1例,被随后的手术证实为垂体瘤。经低剂量诊断性放化疗后,病灶影像学诊断结果显示26例达CR,考虑临床诊断为颅内生殖细胞肿瘤,随即予患者行IMRT及3D-CRT;治疗后影像学复查均为CR,以上患者随访时间1~8年,所有患者均无瘤生存,无复发,未见放射性坏死。经诊断性放疗及2个周期化疗后仍有1例患者影像学诊断结果显示未达到CR,后经手术证实为混合性生殖细胞肿瘤。结论:低剂量诊断性放疗联合化疗具备区分颅内生殖细胞瘤与非生殖细胞瘤的能力,对高度怀疑为颅内生殖细胞肿瘤的患者,在没有病理证实的情况下,可参考此方案进行诊断性治疗和临床处理。

颅内生殖细胞瘤诊断性放疗剂量的初步探讨

目的探索颅内生殖细胞瘤诊断性放疗最低有效剂量,减少患者的无谓照射。方法回顾性分析了经放疗证实的生殖细胞瘤患者75例共104个病灶(其中单发病灶者51例,2个病灶者19例,3个病灶者5例),观察应用10Gy和20Gy的照射剂量时,病灶反应数目的变化。结果2种实验性放射疗法剂量对肿瘤病灶数目的影响未见显著性差异。结论在颅内生殖细胞瘤的诊断性放射疗法中,10Gy可作为排除部分非生殖细胞瘤的初步剂量。

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。

全脑全脊髓放疗不同照射方式的疗效

目的:回顾性分析不同照射方式下全脑全脊髓放疗对生殖细胞瘤及髓母细胞瘤的疗效,并探讨最佳放疗方法。方法:1999年8月至2010年2月本院收治的42例中枢神经系统肿瘤患者行术后全脑全脊髓放疗,放疗方式分为标准全脑全脊髓照射(连续照射)和分段照射(先全脑后全脊髓)两种类型,观察不同照射方式的近期疗效、不良反应、血液学毒性、远期疗效以及复发情况。结果:42例患者经全中枢照射治疗达完全缓解(complete response,CR)者32例。不同照射方式有一定差异,分段照射延长了疗程且不良反应较多,随访显示复发率高于连续照射组,分段照射并未使患者得到明显的生存获益。结论:术后全脑全脊髓放疗对生殖细胞瘤及髓母细胞瘤疗效较好,与以往文献资料相比,可明显提高治愈率及生存期。从最佳照射方式角度出发,分段照射延长疗程,且复发率高,仍以连续照射为佳。

手术辅以中低剂量放疗对儿童颅内生殖细胞瘤疗效观察

目的探讨儿童颅内生殖细胞瘤手术后辅以中低剂量放射治疗后的临床效果。方法对1999年至2008年收治的54例儿童(≤16岁)颅内生殖细胞瘤患者进行手术联合γ-刀中低剂量放射治疗。其中肿瘤位于松果体区34例,松果体并鞍区10例,鞍区4例,松果体区并侵及邻近脑室3例,基底节区3例。所有患者经术后病理证实均为生殖细胞瘤,手术后辅以中低剂量(25～35Gy)的放疗,并获得随访的46例。结果 46例随访者中40例(87%)复查MRI显示肿瘤无复发,患者生存质量明显改善,6例患者经治疗后复发,经再次手术或放疗后效果差。结论手术后辅以中低剂量的放射治疗应为治疗儿童颅内生殖细胞瘤的较好方案。

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。

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。