Treatment of germ cell tumors in the pineal region

The authors retrospectively analyzed 107 patients with primary intracranial germ cell tumor (GCT), who were treated at the Department of Neurosurgery, Yonsei Medical Center between January 1986 and January 1996. The incidence of GCT was 2.8% in pediatric patients with intracranial tumor. Of the 107 tumors, 60 were located in the pineal region, 30 in the suprasellar region, 16 in basal ganglia or the thalamic region, and 1 in the posterior fossa. The 60 pineal GCT consisted of 39 germinomas (29 pure germinomas, 6 germinomas with STGC, 4 germinomas mixed with teratoma), 5 mature teratomas, and 16 nongerminomatous GCT. Thirty patients underwent surgery: their operations took the form of total resection in 14 cases, subtotal resection in 10, and biopsy in 6. Thirty patients (27 with germinomas, 3 with endodermal sinus tumors) were managed without surgery on the basis of radiological findings and tumor markers. The 5-year survival was 91% for 39 patients with germinomas, 80% for 5 with mature teratomas, and 49% for 16 with nongerminomatous GCT. Univariate analysis of prognostic factors with the Kaplan-Meier survival curve showed that histological tumor type, radiological findings, results of tumor marker studies, and response to trial radiation or chemotherapy were highly correlated with outcome. Chemotherapy was beneficial as the method of trial treatment in pineal GCT and treatment in recurrent tumors. The administration of trial chemotherapy or radiotherapy without tissue biopsy is well justified as a treatment modality in pineal GCT suspected on the basis of radiological findings and tumor marker studies. Aggressive multimodality approaches with surgery, radiotherapy, and chemotherapy are necessary to improve the outcome in these tumors. We propose new protocol for treatment of germ cell tumors in the pineal region, which is based on a minimally invasive approach.     

Early response to chemotherapy as an indicator for the management of germinoma-like tumors of the pineal and/or suprasellar regions

Recent advances in diagnostic imaging and experience with germinomas may allow for the differentiation of central nervous system germinomas from other tumors based on clinical information, without histological verification. We retrospectively analyzed clinically diagnosed germinoma-like tumors of the pineal and/or suprasellar regions. This was done to evaluate the efficacy of our strategy of defining germinoma-compatible tumors based on good responses to initial chemotherapy. The responses to chemotherapy and survival of 34 consecutive patients with germinoma-like tumors who underwent initial treatment from July 2001 to October 2010 were analyzed. The minimum apparent diffusion coefficient (minADC) value and proton magnetic resonance spectroscopy (MRS) were evaluated in recent patients. Twelve patients with histologically verified germinomas and 18 with germinoma-compatible tumors showed early logarithmic decreases in tumor volume in response to initial chemotherapy, typical low minADC values and typical MRS characteristics, including increased choline/creatine ratios, decreased N-acetylasparate/creatine ratios, and large lipid peaks. These patients had good progression-free survival. The other four patients, with histologically verified non-germinomas, showed no response to chemotherapy, and one patient with a pineoblastoma showed a similar minADC value and MRS characteristics to those of patients with germinomas. The response to initial chemotherapy can be used to distinguish germinoma-compatible tumors from non-germinoma in patients with germinoma-like tumors of the pineal and/or suprasellar regions. The evaluation of minADC and proton MRS are useful for distinguishing germinomas from other tumors. However, a subset of non-germinomas may show similar characteristics to germinomas. The benefit of bypassing unnecessary surgical intervention can be achieved, at least in Asian populations with a high incidence of germinomas.     

松果体区肿瘤诊治策略的探讨

目的 探讨松果体区肿瘤的诊治策略.方法 回顾性分析154例松果体区肿瘤的病例资料.结果 松果体区肿瘤多以颅高压、眼球运动障碍起病,诊断需进行CT、MRI及肿瘤标记物检查.基本确诊或高度怀疑生殖细胞瘤时直接放疗或活检后放疗23例,肿瘤均消失,5年生存率88.9%;其他肿瘤手术治疗131例,肿瘤全切除124例,次全切除7例,5年生存率84.4%.手术患者的病理结果:生殖细胞肿瘤60例,松果体实质肿瘤17例,神经上皮肿瘤27例,其他肿瘤27例.结论 松果体区肿瘤常合并脑积水,影像学表现有一定特征,联合肿瘤标记物检测能进一步鉴别肿瘤性质.基本确诊生殖细胞瘤直接选择放、化疗,高度怀疑生殖细胞瘤选择活检后放疗或实验性放疗,不能确定生殖细胞瘤时选择显微手术联合术后辅助放、化疗,在治疗过程中兼顾脑积水的处理是较理想的松果体区肿瘤的治疗策略.

Abstract：

Objective To define the optimal diagnosis and theraputic strategy of pineal region tumors.Method Clinical materials of 154 cases with pineal region tumors were analyzed retrospectively.Results The patients with pineal region tumors often complained with increased intracranial pressure and ocular movement deficiency.The procedure of diagnosis was imaging checking included CT and MRI,tumor mark testing.23 cases which were essential exact diagnosed or high doubt germinomas were choosed to direct radiotherapy or radiotherapy after biopsy.All the tumors disappeared during the follow up time.5 years survival rate was 88.9%.Other 131 cases were chosen for operation treatment.Total removal were achieved in 124 cases and subtotal were achieved in 7 cases.The 5 years survival rate was 84.4%.The operation pathological result included 60 germ cell tumors, 17 pineal parenchymal tumors, 27 neural epithelium tumors and 27 other tumors.Conclusions Pineal region tumors are often combined with hydrocephalus.There was some characteristic feature in the imaging appearance.The cases should be chosen to direct radiotherapy and chemotherapy if was diagnosed germinomas, and be choosed to radiotherapy after biopsy or experimental radiotherapy if was high doubt germinomas.The cases which unable determined germionmas should be choosed microsurgery and assist radiotherapy and chemotherapy postoperation.Hydrocephalus should be treated in consideration during the procedure of tumor treatment.     

化、放疗联合治疗儿童颅内生殖细胞瘤34例临床随诊观察

目的：观察颅内生殖细胞瘤经过化疗并辅以中低剂量放疗后的疗效及对儿童生长发育的影响。方法：自1993年12月至2001年12月共对63例颅内生殖细胞瘤的儿童进行静脉化疗并辅以中低剂量放疗，随访到34例，其中肿瘤位于松果体区20你，松果体区并鞍区6例，鞍区4例，松果体区并脑室内3例，底节区1例。使用药物为长春新碱、甲氨蝶呤、平阳霉素及顺铂。用药后每周化验2次血常规，1次血生化，以观察周围血象变化及心、肝、肾功能情况，并进行针对性治疗。4周后如血常规及血生化的各项指标均正常，再行第二疗程化疗。2个疗程结束后1个月，在肿瘤病灶局部补充中低剂量（25-35Gy）的放疗。每个疗程间进行CT或MRI检查，观察肿瘤消退的情况。：34例患者随访最短1年，最长7年。1例为手术加全剂量放疗后2年肿瘤复发，此次化疗进行2个疗程，但化疗后未再补充放疗，4年后肿瘤复发，家属放弃治疗，患者死亡。1例化、放疗后5年肿瘤复发，再行2疗程化疗，疗效较首次治疗差，其余32例（94％）化、放疗后1-7年，复查MRI显示肿瘤无复发，患者生存质量良好，儿童生长发育及智力均未受影响。结论：静脉化疗辅以中低剂量放疗应为治疗儿童颅内生殖细胞瘤的最佳方案。     

显微手术切除松果体区肿瘤(附34例报告)

目的 总结松果体区肿瘤显微手术切除的经验。方法 回顾34例松果体区肿瘤的临床资料和手术体会。结果 本组患者中生殖细胞瘤12例,畸胎瘤7例,胶质瘤5例,脑膜瘤4例,胆脂瘤、海绵状血管瘤各2例,松果体细胞瘤和异位垂体腺瘤各1例。所有患者均行手术切除肿瘤,采用枕部经小脑幕入路27例,幕下小脑上入路4例,侧脑室三角部入路2例,经胼胝体入路1例。肿瘤全切除28例,次全切除5例,部分切除1例。手术后无住院期内死亡者。结论 显微手术技术的提高显著降低了松果体区肿瘤直接手术切除的死亡率和并发症发生率,我们认为积极的显微外科手术治疗是松果体区肿瘤最佳的一线治疗手段。     

Clinico-biological behavior of germ-cell tumors

Fifty-five patients with intracranial germ-cell tumors were treated in Kobe University Hospital between 1972 and 1989. Thirty-four patients were male and 21 were female, and all patients were between 0 and 39 years of age. There was a peak incidence between 16 and 20 years in male patients and 11 and 15 years in female patients. The tumors occurred most frequently in the pineal region (25 cases), followed by the suprasellar (17), both pineal and suprasellar (6), basal ganglia (5), and frontal and temporal lobe (2) regions. There was a male predominance in the incidence of germ-cell tumors except for the patients with suprasellar tumors. Initial symptoms and neurological signs differed according to the location of the tumor. The symptoms of increased intracranial pressure and Parinaud's sign were most frequently seen in patients with a pineal tumor, while diabetes insipidus and visual disturbance were most common in patients with suprasellar tumors. Human chorionic gonadotropin were positive in the serum, cerebrospinal fluid and/or tumor cyst in 14/26 patients, and alpha-fetoprotein in 6/22 patients examined. There were 45 patients with pure germinoma both histologically verified and clinically diagnosed. The 5-year survival rate of the patients with germinoma was 69% in cases in which tumor marker was negative and unknown cases, and 86% in cases where the tumor marker was positive. The authors discuss management of the patients with intracranial germ-cell tumors.Presented at the XVIII Annual Meeting of the International Society for Pediatric Neurosurgery, Paris 1990     

松果体区生殖细胞肿瘤放射外科治疗的合理性探讨

目的 探讨立体定向放射外科作为一线方案治疗松果体区生殖细胞肿瘤的合理性.方法 回顾性分析14例经伽玛刀或X-刀治疗的松果体区生殖细胞肿瘤患者的临床资料,重点研究肿瘤放射外科治疗后变化及复发、转移情况.结果 14例患者于放射外科治疗后11 d至106个月再次入院.肿瘤放射外科治疗后缓慢增大1例,无明显变化2例,缩小后再增大1例,基本消失后原位复发2例,消失后周边复发4例,原位复发并种植转移2例,原位消失但出现种植转移2例.病理为:生殖细胞瘤5例、混合型生殖细胞肿瘤4例、成熟畸胎瘤3例、非成熟畸胎瘤和卵黄囊瘤各1例.结论 放射外科不能作为松果体区生殖细胞肿瘤的单一治疗方法.假使已采用放射外科治疗,则应依据肿瘤标记物结果和治疗后肿瘤的变化情况,及时手术或综合治疗.

Abstract：

Objective To discuss the rationality of the stereotaxic radiosurgery as the first therapy strategy on pineal region germ cell tumors.Methods To retrospectively analyze the clinical materials of 14cases which received the treatment of gamma knife or X- knife.The condition of tumor changing,recurring and metastasize post- radiosurgery were emphasized.Methods All the patients of 14 cases were admitted again from 11 days to 106 months after which had received radiosurgery.The tumor increasing slowing with 1cases,no marked change with 2 cases,repeated increasing after diminution with 1 case,situ recur after essential disappear with 2 cases,ambitus recur after disappear with 4 cases,situ recur and implantation metastasis 2 cases,situ disappear but implantation metastasis with 2 cases.The pathology results were 5germinomas,4 mixed germ cell tumors,3 mature teratomas,1 unmature teratoma and 1 yolo sac tumor.Conclusion Radiosurgery cant be regarded as the exclusive treatment for pineal region germ cell tumors.If the patients received the radiosurgery,they should be operated or combined therapy in time according the results of tumor marker and the condition of tumor changing.     

Craniopharyngiomas in children: surgical experience at Children’s Memorial Hospital

Objectives Craniopharyngioma during childhood poses difficulty in management because of the high incidence of surgical complications and treatment failure. In order to identify less detrimental and more effective treatment, a personal series of craniopharyngioma was reviewed in regard to various clinical factors, patient factors (age and sex), tumor factors (location and extension, relationship with chiasm, and hydrocephalus), and therapeutic modes [extent of resection and radiation therapy (RT)].Materials and methods Fifty-four childhood craniopharyngiomas treated from 1984 to 2003 were reviewed. Preoperative neuroimaging studies were classified depending upon tumor location and extension. In this series of 54 patients, 43 had total tumor resection and 11 had subtotal resection. Of the total resection group, ten showed evidence of residual tumor on postoperative neuroimaging studies. Following the initial resection, 46 did not have RT whereas 8 with subtotal resection received RT.Results There were no surgical deaths. Postoperative complications included pseudoaneurysm in 1, hemiparesis in 3, severe obesity in 5, panhypopituitarism in 50, and worsening of visual function in 7. During follow-up ranging from 12 months to 21 years, 24 patients had recurrence. Of the 33 patients with radiographic total resection, 9 (27.3%) had recurrence. Among the patients with total resection but radiographic residual and those with subtotal resection, the craniopharyngioma recurred in 90% and 100%, respectively. Three (37.5%) of eight patients with subtotal resection with RT had recurrence. Overall recurrence-free survival was 62% at 5 years and 49% at 10 years. The sex and age, location and extension of the tumor, nature of the optic chiasm, and hydrocephalus did not influence survival with statistical significance. However, the extent of surgical resection and use of RT showed significant differences for survival. Patients with total resection had a recurrence-free survival rate of 83% and 70% at 5 and 10 years, respectively. Patients with subtotal resection with RT had 71% at 5 years and 36% at 10 years. Patients who had subtotal resection or radiographically residual tumor without RT had a recurrence-free survival rate of only 9%. Among 22 patients whose recurrent tumor was treated with RT, a second recurrence-free survival rate was 90% at 5 years.Conclusion Total resection provided the best outcome. However, recurrence rates and surgical complications remained high following radical tumor resection. RT was effective for recurrent tumors and should be considered being the primary treatment for recurrences or difficult tumors, which are not amenable to total resections.     

Intracranial germ cell tumors: a retrospective study of 44 children

This 16-year retrospective study review sought to determine the factors influencing prognosis and treatment results of all patients with primary intracranial germ cell tumors treated at our hospital who were younger than 17 years of age at the time of diagnosis. A total of 44 patients were treated during the study period, including 32 males and 12 females with a male:female ratio of 2.67:1. The median age at diagnosis was 12 years and 5 months of age (range = 2-16 years). The 44 intracranial germ cell tumors consisted of 27 pure germinomas (61.4%) and 17 nongerminomatous germ cell tumors, including 10 mixed germ cell tumors (22.7%), three yolk sac tumors (7.8%), two immature teratomas (4.5%), and two choriocarcinomas (4.5%). Univariate analysis of prognostic factors using Kaplan-Meier survival estimates revealed that only histologic tumor type was correlated with outcome (P < 0.005). The projected 5-year overall survival and event-free survival rate of patients with germinomas vs those with intracranial germ cell tumors were 92.6%, 92.6% vs 47.3%, and 42.1%, respectively. Our analysis suggests that radiation involving the spinal axis has limited usefulness in patients with intracranial germ cell tumor, although better results have been obtained for germinomas using radiotherapy in this study.     

松果体区肿瘤的手术治疗

目的:旨在进一步提高松果体区肿瘤的手术疗效。方法与结果:回顾性研究23例松果体区肿瘤的治疗情况,本组2例根据影像学特征考虑为生殖细胞瘤,给予脑室-腹腔分流术-放射治疗,结果肿瘤消失,余21例均经枕切开天幕入路显微手术治疗。其小4例同时行脑脊液(CSF)分流术,无手术死亡。术后计算机体层摄影(CT)复查证实肿瘤全切除5例,次全切除9例,大部或部分切除7例。结论:松果体区肿瘤手术难度及危险性大,手术前应参照CT或磁共振成像(MRI)等影像资料。针对肿瘤的具体部位及毗邻等情况,选用适当的治疗方法和合理的手术入路,强调经枕切开天幕入路的优越性、指出显微技术、麻醉方法的改进以及必要的放射治疗可以提高此病的治疗效果。     

Primary pontomedullary germinoma in a 12 year old boy

Primary intracranial germinomas are rare tumors, accounting for approximately 1–4% of all intracranial tumors. Intracranial germinomas are more commonly found in the suprasellar and pineal midline structures of the brain. Brainstem and posterior fossa germinomas are rarer still, with few reported cases in the literature, and little discussion of their presentation, management and clinical outcome. A unique case of pontomedullary germinoma, diagnosed in a 12-year-old boy, is described. Only six previous cases of lower brainstem germinoma have been reported, with varying modes of presentation and a lack of definitive management guidelines. Of these, all occurred in either females or a patient with Klinefelter’s syndrome. We report the first case of a lower brainstem germinoma in a male without known genetic abnormality. Tumor remission was achieved with partial surgical resection, chemotherapy and radiotherapy.     

小儿松果体区肿瘤的治疗

目的探讨小儿松果体区肿瘤的治疗策略。方法对43例患松果体区肿瘤病例施以立体定向放射外科、全脑放疗为主,化疗、Ⅴ-P术、手术切除为辅助的治疗方略,以探讨疗效。结果随访3～44个月,平均22.6个月,肿瘤消失25例,缩小11例,不变5例,增大2例,无死亡病例。肿瘤控制率95.3%。结论采取以立体定向放射外科、全脑放疗为主,化疗、手术切除为辅助的治疗策略,可以极大限度地降低手术造成的损伤,提高小儿松果体区肿瘤患者生存质量,延长生存期。     

Central nervous system germinomas. A review

The germinoma represents a less malignant form of germ cell tumor. Depending on the individual's age, this neoplasm constitutes approximately 0.1% to 3.4% of all intracranial tumors. The embryologic origin remains a mystery; however, current theories implicate an aberration in primordial germ cell migration. Clinical presentation depends on tumor location and may involve endocrine, hypothalamic, visual, and cognitive dysfunction. In evaluating midline intracerebral masses, it is imperative that one be aware of the various radiologic appearances, endocrinologic changes, and chemical markers that help to distinguish germinomas from other neoplasms that appear in the pineal, suprasellar, and periventricular regions. Only through the careful evaluation of all available studies can the physician institute appropriate therapies such as biopsy, radiation, and chemotherapy. This article focuses on the epidemiology, embryology, clinical presentation, means of diagnosis, treatment, and outcome of this rare neoplasm.     

松果体区非生殖细胞瘤性恶性生殖细胞肿瘤的治疗

目的 探讨松果体区非生殖细胞瘤性恶性生殖细胞肿瘤(NGMGCTs)的临床特点、治疗和预后.方法 回顾性分析了2000年1月至2010年1月经病理证实的34例高度恶性NGMGCTs 患者的临床特点、血清肿瘤标记物检测、治疗方法及预后.所有患者均行枕部经小脑幕(Poopen)入路显微手术切除肿瘤,并行辅助放化疗.结果 全切除32例,近全切除2例,术后病理示未成熟畸胎瘤11例,畸胎瘤恶性变2例,胚胎癌2例,卵黄囊瘤l例,绒毛膜上皮癌6例,混合性生殖细胞肿瘤12例.共随访31例患者,随访时间6个月至10年,1年生存率为97％,3年生存率为62％,5年生存率为44％.结论 多数松果体区NGMGCTs根据临床表现、影像学资料和肿瘤标记物可在术前定性,以手术为主术后辅以化疗和放疗的综合治疗可以获得良好疗效.     

Re-evaluation of the significance of cerebrospinal fluid human chorionic gonadotropin in detecting intracranial ectopic germinomas

This study was conducted to establish a reference value for cerebrospinal fluid (CSF) human chorionic gonadotropin (hCG) levels. We also evaluated the sensitivity of CSF hCG as a biomarker to detect intracranial ectopic germinomas that arise in rare sites other than the pineal and suprasellar regions. CSF hCG was measured in 201 male patients who had various types of neurological disease (not tumours of germ cell origin or other malignant tumours). A reference value of 1.009 U/L was established, and the CSF hCG level among different age groups was not significantly different. CSF and serum hCG were measured before and after radiotherapy in 14 consecutive patients with intracranial ectopic germinomas. The CSF hCG levels were all above 1.009 U/L before radiotherapy. In male patients, a CSF hCG value above 1.009 U/L suggests abnormal intrathecal hCG secretion. These results demonstrate that the CSF hCG assay is a sensitive method for diagnosing intracranial ectopic germinoma.     

Controversy pertaining to therapeutic modalities for tumors of the pineal region: a worldwide survey of different patient populations

The management of tumors of the pineal region differs between Western countries and Japan. This paper reports on a worldwide survey of individual experience and regimens for treating pineal region tumors in different patient populations. Fifteen pediatric neurosurgeons from nine different countries participated in the survey, and a total of 408 pineal region tumors were evaluated. Determination of tumor histology as an initial procedure was strongly supported by the majority of neurosurgeons in North and Central America and Europe (group A), whereas all but one from Asia and Egypt (group B) emphasized initial application of the radiation test. The analysis of patient populations clearly revealed racial differences in tumor type which explain this discrepancy. Germinoma, the most radiosensitive tumor, constituted 43–70% (mean: 53.7%) of tumors in group B, followed by teratoma, pineoplastoma, and others, whereas in group A the incidence of germinoma was only 21–44% (mean: 34.7%), followed by a variety of tumors, such as astrocytoma, pineoblastoma, etc. The age distribution among intracranial germ cell tumors (GCT) obtained from data from the Brain Tumor Registry in Japan also demonstrated clear differences in the incidence of tumor types in different age groups in Japan: while germinoma constituted 70–84% of GCT in patients between the ages of 15 and 35 years, the incidence was much lower before 15 yeras and after 35 years, being 24% of tumors under 4 years and 34% of tumors after 40 years of age. The therapeutic regimen for pineal region tumors should depend on the patient population concerned, because of the differences relating to race and age distribution.     

松果体区肿瘤的MRI表现与其病理学对照研究

目的探讨不同病理性质的松果体区肿瘤MRI表现,评价其临床诊断价值。方法回顾性分析1999年1月至2009年1月经开颅手术治疗的131例松果体区肿瘤患者的MRI及病理学资料,并进行对比分析。结果这131例松果体区肿瘤中,纯生殖细胞瘤21例,畸胎瘤18例,混合性生殖细胞肿瘤12例,绒毛膜癌6例,胚胎性癌2例,内胚窦瘤1例,神经上皮肿瘤27例,松果体实质肿瘤17例,脑膜瘤14例和其它肿瘤13例。本组生殖细胞肿瘤60例,MRI增强后均有不同程度的强化,含有小囊状改变39例;其中生殖细胞瘤21例,5例MRI有较典型蝶形征;畸胎瘤18例,13例MRI有较典型的峰窝状改变;绒毛膜癌6例,5例MRI示出血。脑膜瘤14例,MRI示边界清楚,增强扫描均明显均匀强化,局部硬膜增厚6例;松果体实质肿瘤17例、神经上皮肿瘤27例及其他肿瘤13例,MRI表现各式各样。结论松果体区肿瘤的MRI表现与病理类型有关,部分MRI征象对一些肿瘤的诊断有较大特异性,但敏感度不高,依据MRI仍不能完全鉴别出松果体区肿瘤的病理性质。     

Pineal tumors: experience with 48 cases over 10 years

The authors retrospectively reviewed 48 patients treated at Seoul National University Hospital (SNUH) between 1986 and 1995. There were 35 children and 13 adults, accounting for 10.1% of 345 pediatric and 0.68% of 1914 adult brain tumors in SNUH during the same period. The 48 cases consisted of 33 cases of germ cell tumor (69%, GCT); 6 of pineoblastoma (PB, 12.5%); 3 of pineocytoma (PC, 6.3%); 3 of anaplastic astrocytoma (6.3%); 1 of astrocytoma; 1 of glioblastoma; and 1 of ependymoma. The median age was 13 years (range 1–59) and the male-to-female ratio was 3.36:1. The most frequent presenting symptom was due to increased intracranial pressure (90%), followed by Parinaud syndrome or diplopia (50%). Patients with a benign tumor, such as teratoma (TE), astrocytoma, or ependymoma, underwent surgery by the occipital transtentorial approach (OTT) for attempted radical resection without adjuvant therapy, while patients with immature teratoma (imTE), PC, and anaplastic astrocytoma underwent regional radiotherapy (RT) after debulking via OTT. Seven patients with nongerminomatous malignant GCT (NG-MGCT) and 3 with germinoma (GE) underwent craniospinal radiation only, 6 with GE, a NG-MGCT, and 2 with GE+TE received craniospinal radiotherapy (CSRT) after debulking via OTT. Three patients with GE, 4 with NG-MGCT, and 3 with PB underwent radiochemotherapy after debulking via OTT. Forty-four patients were followed up after treatment. The median follow-up period was 36 months. All patients with GE were alive after RT at 36 months (median) of follow-up (range 7–70 months). All with GE+TE and TE were alive. Three patients with PC or astrocytoma were also alive with stable or no evidence of disease. In 1 of the 3 cases of imTE there was a recurrence. However, 4 patients with NG-MGCT died, all of whom had undergone CSRT only; 2 PB patients were alive (12, 19 months), 1 in a moribund status (36 months), and 2 were dead (6, 60 months). The overall mean survival time with pineal tumors was 66 months and the 3-year survival rate was 84% with minimal posttreatment complications. It is concluded that pineal region tumors have male and childhood predominances, and the most common tumor is GCT. The majority of pineal region tumors are malignant. Pineal region tumors can be approached safely and effectively and the surgical complications are mostly transient. Their prognosis is dependent on the pathologies and treatment modalities.     

Pineal and nonpineal supratentorial primitive neuroectodermal tumors

Pineal region supratentorial primitive neuroectodermal tumors (SPNETs; pineoblastomas) and nonpineal SPNETs are rare tumors that historically have carried a very poor prognosis. With multimodality therapy, including maximal surgical resection, craniospinal radiation therapy and chemotherapy, the survival for patients with pineal PNETs has significantly improved. Chemotherapy alone, at least in conventional doses, appears to be insufficient treatment for younger children with pineoblastomas, in whom there is almost universal rapid tumor progression and death. Survival of patients with nonpineal SPNETs remains in the order of 30–35% despite multimodality therapy. Unlike those with pineal SPNETs, a significant percentage of infants with nonpineal SPNETs who undergo gross total surgical resection followed by chemotherapy will be long-term survivors. This article gives an overview of the natural history, prognostic factors and treatment of both pineal and nonpineal SPNETs. Received: 12 April 1999     

Pineal region tumours in childhood A 30-year experience

Patients aged under 16 years presenting to the Royal Children's Hospital between 1967 and 1997 with pineal region tumours were retrospectively reviewed. Thirty-seven patients were identified, with 13 germinomas, 7 nongerminomatous germ cell tumours, 6 pineoblastomas, 2 pineocytomas, and 3 astrocytomas, while in 6 patients no histopathological diagnosis was obtained. The most common presentation was with symptoms of raised intracranial pressure due to hydrocephalus. Thirty-two of the 37 patients required a shunt. Thirteen had a biopsy as a separate procedure, 3 of which were stereotactic. Tumour excision was performed in 21 patients and was complete in 4 and subtotal in 17. There were 2 perioperative deaths and 6 patients who were neurologically worse after surgery. Twenty-six patients had radiotherapy and 16 chemotherapy, with significant complications of radiotherapy in half of the patients who received it. The 5-year survival of patients with benign tumours was 75%, 5-year survival with germinomas 62% and with other malignant tumours 14%. This series demonstrates significant improvements in management of pineal region tumours in the last 30 years and highlights some of the current controversies. A collaborative research approach is necessary to determine optimal management of the varied tumour types occurring in the pineal region in childhood. Received: 29 October 1998