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.     

原发性松果体区胚胎癌的诊断与治疗(附二例报告及文献复习)

Objective Primary embryonal carcinoma in pineal region is extremely rare Here we report two such cases and combined literatures review to discuss its diagnosis, treatment and prognosis. Method Two primary embryonal carcinoma in pineal region verified histologically were presented. In one cases, the preoperative serum alpha - fetoprotein (AFP) level reached significantly high level, 6 810μg/L, but showed negative staining forβ-human chorionic gonadotropin (β- HCG) . In the other one, accompanied by elevation of β-HCG level, 5 260 mIU/ml, the serum AFP was negative. The tumors were microsurgically removed by transeccipito-tentorial approach. Endoscopic third ventriculostomy was used after tumor removal because of recurrent hydrocephalus. Both patients received adjunctive treatments included radiation therapy (whole central nervous system 30 Gy and tumor bed 50 Gy) and 4 course of chemotherapy. Results In both patients, the tumors were totally removed. Postoperative tumor immunohistochemistry indicators were present in case 1 with positive reaction for cytokeratin(CK) and AFP, in case 2 with positive CK and β - HCG. After adjunctive treatments, the serum AFP and β - HCG of two patiants were decreased to normal ranges. After post - operative follow - up of one year,two patients still survived and the serum markers were almost normal. Conclusions Measurement of serum β-HCG and AFP is extraordinarily significant for the diagnosis and post - treatment monitoring of pineal region embryonal carcinoma. Pathological differential diagnosis requires immunohistochemical indicators including cytokeratin (CK),AFP andβ- HCG. With radically surgical removal and combined adjunctive therapy including radiation and chemotherapy, good consequence could be obtained.     

原发性松果体区胚胎癌的诊断与治疗(附二例报告及文献复习)

Objective Primary embryonal carcinoma in pineal region is extremely rare Here we report two such cases and combined literatures review to discuss its diagnosis, treatment and prognosis. Method Two primary embryonal carcinoma in pineal region verified histologically were presented. In one cases, the preoperative serum alpha - fetoprotein (AFP) level reached significantly high level, 6 810μg/L, but showed negative staining forβ-human chorionic gonadotropin (β- HCG) . In the other one, accompanied by elevation of β-HCG level, 5 260 mIU/ml, the serum AFP was negative. The tumors were microsurgically removed by transeccipito-tentorial approach. Endoscopic third ventriculostomy was used after tumor removal because of recurrent hydrocephalus. Both patients received adjunctive treatments included radiation therapy (whole central nervous system 30 Gy and tumor bed 50 Gy) and 4 course of chemotherapy. Results In both patients, the tumors were totally removed. Postoperative tumor immunohistochemistry indicators were present in case 1 with positive reaction for cytokeratin(CK) and AFP, in case 2 with positive CK and β - HCG. After adjunctive treatments, the serum AFP and β - HCG of two patiants were decreased to normal ranges. After post - operative follow - up of one year,two patients still survived and the serum markers were almost normal. Conclusions Measurement of serum β-HCG and AFP is extraordinarily significant for the diagnosis and post - treatment monitoring of pineal region embryonal carcinoma. Pathological differential diagnosis requires immunohistochemical indicators including cytokeratin (CK),AFP andβ- HCG. With radically surgical removal and combined adjunctive therapy including radiation and chemotherapy, good consequence could be obtained.     

中枢神经细胞瘤94例临床分析

目的 总结中枢神经细胞瘤临床特点,探讨其治疗策略.方法 回顾性分析94例中枢神经细胞瘤患者临床资料.全部行手术治疗,肿瘤全切除66例,近全切除26例,部分切除2例;分析总结中枢神经细胞瘤的临床、病理、影像特点.结果 死亡2例,随访84例,术后放疗56例,肿瘤复发4例.多数患者生存良好.结论 中枢神经细胞瘤多发于室间孔附近侧脑室系统,手术全切除是最佳治疗手段,未全切患者术后放疗可减少复发率.

Abstract：

Objective To analyze the clinical characteristics of central neurocytomas,and discuss the therapeutic strategies.Methods 94 cases of central neurocytomas were studied retrospectively.All patients underwent operation with removal of the tumor through either transcallosal or transcortical approach.Total resection was achieved in 66 patients,subtotal resection in 26 patients and partial resection in 2 patients.The clinical,radiological,histologic and immunohistochemical features of these patients were reviewed and analyzed.Methods Amongthe 94 cases of central neurocytomas,two died after surgery.Among the 84 followed- up cases,56 cases underwent postoperative radiotherapy,4 cases had recurrence.Most patients have favorable prognosis.Conclusion Central neurocytomas occur mostly in the lateral ventricle near the Monro's foramen.Total resection is the best treatment.Postoperative radiotherapy for partially removed tumors may reduce the possibility of recurrence.     

原发性松果体区胚胎癌的诊断与治疗(附二例报告及文献复习)

目的 原发性松果体区胚胎癌非常罕见,本文报道2例,并结合文献探讨其诊断、治疗和预后.方法 2例经病理证实的原发性松果体区胚胎癌,术前例1患者血清甲胎蛋白(AFP)6 810μg/L,人绒毛膜促性腺激素(β-HCG)正常,例2患者血清AFP正常,β-HCG 5 260 mIU/ml.采用枕部经小脑幕入路显微手术切除肿瘤,术后联合放化疗.结果 2例患者肿瘤均达到镜下全切,术后肿瘤免疫组化示例1:CK、AFP阳性;例2:CK、β-HCG阳性.经放化疗后,血清AFP/β-HCG基本正常.术后1年随访2例患者仍然存活.结论 血清AFP、β-HCG对胚胎癌的诊断及预后判断有重要作用,免疫组化指标CK、AFP、β-HCG对病理诊断有鉴别意义,该病采用手术联合放化疗的综合疗法,可获得较好疗效.

Abstract：

Objective Primary embryonal carcinoma in pineal region is extremely rare Here we report two such cases and combined literatures review to discuss its diagnosis, treatment and prognosis. Method Two primary embryonal carcinoma in pineal region verified histologically were presented. In one cases, the preoperative serum alpha - fetoprotein (AFP) level reached significantly high level, 6 810μg/L, but showed negative staining forβ-human chorionic gonadotropin (β- HCG) . In the other one, accompanied by elevation of β-HCG level, 5 260 mIU/ml, the serum AFP was negative. The tumors were microsurgically removed by transeccipito-tentorial approach. Endoscopic third ventriculostomy was used after tumor removal because of recurrent hydrocephalus. Both patients received adjunctive treatments included radiation therapy (whole central nervous system 30 Gy and tumor bed 50 Gy) and 4 course of chemotherapy. Results In both patients, the tumors were totally removed. Postoperative tumor immunohistochemistry indicators were present in case 1 with positive reaction for cytokeratin(CK) and AFP, in case 2 with positive CK and β - HCG. After adjunctive treatments, the serum AFP and β - HCG of two patiants were decreased to normal ranges. After post - operative follow - up of one year,two patients still survived and the serum markers were almost normal. Conclusions Measurement of serum β-HCG and AFP is extraordinarily significant for the diagnosis and post - treatment monitoring of pineal region embryonal carcinoma. Pathological differential diagnosis requires immunohistochemical indicators including cytokeratin (CK),AFP andβ- HCG. With radically surgical removal and combined adjunctive therapy including radiation and chemotherapy, good consequence could be obtained.     

Treatment of malignant glioma using hyperthermia

Thirty pathologically diagnosed patients with grade III–IV primary or recurrent malignant glioma(tumor diameter 3–7 cm)were randomly divided into two groups.The control group underwent conventional radiotherapy and chemotherapy.In the hyperthermia group,primary cases received hyperthermia treatment,and patients with recurrent tumors were treated with hyperthermia in combination with radiotherapy and chemotherapy.Hyperthermia treatment was administered using a13.56-MHz radio frequency hyperthermia device.Electrodes were inserted into the tumor with the aid of a CT-guided stereotactic apparatus and heat was applied for 1 hour.During 3 months after hyperthermia,patients were evaluated with head CT or MRI every month.Gliomas in the hyperthermia group exhibited growth retardation or growth termination.Necrosis was evident in 80%of the heated tumor tissue and there was a decrease in tumor diameter.Our findings indicate that radio frequency hyperthermia has a beneficial effect in the treatment of malignant glioma.     

脑脊液细胞学在脑生殖细胞瘤诊断中的应用及八例报告

目的 通过总结8例脑生殖细胞瘤患者的脑脊液细胞学结果,探讨脑脊液细胞学在脑生殖细胞瘤诊断中的价值.方法 总结2006年1月至2009年6月我院脑脊液细胞学发现肿瘤细胞的8例脑生殖细胞瘤患者,分析其临床特点、影像学和脑脊液细胞学结果.结果 8例患者中男性7例,女性1例.年龄13～25岁,分别以多饮、多尿和少汗等内分泌症状或神经科症状起病.神经科症状包括:头晕、头痛、智能减退、精神行为异常、复视、双下肢无力、尿便障碍等.神经影像学可见鞍上区和(或)松果体区占位、脑室扩张、室管膜和软脑膜强化、神经根增粗强化等.脑脊液绒毛膜促性腺激素3.2～1087.0 mIU/ml.脑脊液细胞学8例均见肿瘤细胞,呈中等大小的圆形,核大,核仁明显,胞质丰富,胞质内可见较多空泡.背景间有小淋巴细胞为主的炎性反应.过碘酸Schiff染色肿瘤细胞胞质中见阳性颗粒.4例行免疫细胞化学染色,其中2例抗胎盘碱性磷酸酶阳性;2例行Ki-67染色,阳性细胞分别占12%和20%.细胞角蛋白和癌胚抗原染色等阴性.结论 脑生殖细胞瘤患者的脑脊液细胞学结果特征明显,结合细胞免疫化学可以明确其类型及脑脊液播散,在诊断脑生殖细胞瘤过程中起着重要作用.

Abstract：

Objective To investigate the value of the cerebrospinal fluid ( CSF ) cytology in diagnosis of intracranial germinomas by reviewing the outcomes of CSF cytology of 8 patients with intracranial germinomas. Methods Eight patients with positive CSF cytology at our clinic from January 2006 to June 2009 were reviewed. Conventional cytology and immunocytochemistry of CSF were performed. The relevant literature on the subject was reviewed. Results The patients, including 7 male and 1 female, developed endocrinological or neurological symptoms at the age of 13 to 25, and the typical neurological presentation included vertigo, headache, mental and behavior disorders, double vision and weakness of legs. The CSF cell count ranged from 0 to 300 leukocytes per cubic and elevated in 7 cases, typically lymphocytic inflammation. CSF level of human chorionic gonadotropin was 3.2-1087.0 mIU/ml, higher than the individual serum level. On CSF cytology studies, typical tumor cells of germinima were found, which had positive particles in cytoplasm on periodic acid Schiff stain. All presents had lymphocyte inflammation ( small lymphocyte predominant ). On immunocytochemical studies of CSF, the tumor cells were positive on placental alkaline phosphatase and Ki-67 stains. Conclusions CSF cytology is clinically useful for diagnosis of primary intracranial germinoma. Further clinical and cytological studies will be necessary for a better understanding of the biology of these tumors.     

Cllnical analysis on 80 cases with hypertensive cerebral hemorrhage compllcating upper digestive tract hemorrhage

Objective: To irrvestigate the treatment and prognosis of the hypertensive cerebral hemorrhage (HCH)With upper dgest tract hemorrhage (UDDIH). Scence:I a surey of 760 cases randomely with HCH in nerve department betweem Jaruarry 1995 and October 1999, including 80 cases wuth UDTH (9.5%). Of the 80 cases 55 were males and25 were fenales, mean age of 60.2 years andrange of 45 to 80 years. Methods:To analyze the clinical materials of 80 cases with HCH complicating UDTH and HCH without UDTH for the same time. Results: Of the 80 cases 52 were cled during the treatment, death rate was 65%, but the death rate of cases without UDTH was 30.2% at the sane time. The death rate of HCH complicating UDTH was significantly ligher than that without UDTH Discussion The nosogeny of HCH complicating UDTH was more likely related to thalanic and brain-stem Lew is thought that thalemric and brain-stem were stimulated to secret CRF and made pituitany gland release ACTH, futhet secret advencortirn These factors gave rise to UDTH by incriesed to secretion of gastic acid Therefore, original ciseases wre Primarily be predcted and treated Patients with intracranial lypenrtension should be inmediately treated by using manniol or glyceric co, and antiacid, anticholinergic drugs. If all kinds of methods shouldn′t control UDTH, thrombin and noradrenaline bitarlrate with cold Natrii Chlori de would be giwn by nostril There have bettertherapeutic efficiency. Conclusion: HCH with UDTH belongs to severe cases, death rates are higher, original diseases and complication should be actively treated     

颅咽管瘤侵袭第三脑室的方式对手术切除的影响

Objective To assess the impact of the positional relationship between the large craniopharyngiomas and the third ventricle on the outcome of surgical resection of the tumors. Methods In 72 cases of craniopharyngiomas, the tumors were classified into two types according to the radiographic and surgical findings defining the relationship between the tumors and the third ventricle. For each type of tumors, 3 grades were classified based on the tumor size, texture, and infiltration pattern of the third ventricle. The extend of tumor removal was confirmed by both intraoperative evaluation and postoperative CT or magnetic resonance imaging. The correlation between the degree of hypothalamus invasion and the extend of tumor removal was analyzed in all the cases. Results The craniopharyngiomas were classified into two types, namely those residing in the third ventricle (7 cases) and those invading the third ventricle(65 cases). The degree of tumor resection was significantly different between the 3 grades: total tumor removal was achieved in 95.7% of grade Ⅰ cases, in 80.0% of grade Ⅱ cases, and in only 47.4% of grade Ⅲ I cases (P<0.05). conclusion Understanding of the anatomical relationship of the tumor to the third ventricular floor can be great value for improving the outcome of surgical resection of large craniopharyngiomas.     

颅咽管瘤侵袭第三脑室的方式对手术切除的影响

Objective To assess the impact of the positional relationship between the large craniopharyngiomas and the third ventricle on the outcome of surgical resection of the tumors. Methods In 72 cases of craniopharyngiomas, the tumors were classified into two types according to the radiographic and surgical findings defining the relationship between the tumors and the third ventricle. For each type of tumors, 3 grades were classified based on the tumor size, texture, and infiltration pattern of the third ventricle. The extend of tumor removal was confirmed by both intraoperative evaluation and postoperative CT or magnetic resonance imaging. The correlation between the degree of hypothalamus invasion and the extend of tumor removal was analyzed in all the cases. Results The craniopharyngiomas were classified into two types, namely those residing in the third ventricle (7 cases) and those invading the third ventricle(65 cases). The degree of tumor resection was significantly different between the 3 grades: total tumor removal was achieved in 95.7% of grade Ⅰ cases, in 80.0% of grade Ⅱ cases, and in only 47.4% of grade Ⅲ I cases (P<0.05). conclusion Understanding of the anatomical relationship of the tumor to the third ventricular floor can be great value for improving the outcome of surgical resection of large craniopharyngiomas.     

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.     

Experience with pineal region tumors

The results are reported of a retrospective review of the presentation and outcome of 43 pineal region tumors treated from 1982 to 1996, including 20 identified tumors: 5 germinomas, 8 teratomas, 2 embryonal carcinomas, 1 endodermal sinus tumor, 2 pineocytomas and 2 pineoblastomas. Of the 43 tumors reviewed, 36 were located in the pineal region, 5 in the suprasellar, and 2 in both the pineal and suprasellar regions. Twenty patients underwent surgical resection: total in 6 and partial in 10, while only a biopsy was taken in 4 cases. Fifteen patients were managed on the basis of serum CSF tumor markers and radiation response. Twenty-three patients with germinomas received radiotherapy (RT) and had a 5-year survival rate of 87%. Fifteen patients with non-germinomatous germ cell tumors received RT and chemotherapy following direct surgery, and 5 died (mortality rate of 33.3%). The overall survival rate of the 43 patients with pineal tumors was 79.1% (34/43) and the death rate was 20.9% (9/43). It is now recognized that the wide variety of tumor types found in the pineal region necessitates different modes of treatment, and improved microsurgical and stereotactic surgical techniques have made mortality and morbidity rates acceptably low. Because the radiation response and CSF cytology are not enough to determine optimum treatment, a tissue diagnosis should be obtained in all patients.     

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。     

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.     

Therapeutic strategies and surgical results for pineal region tumours.

Until recently, surgery for pineal region tumours has met with poor results. Although experience remains limited, developments in imaging, surgical approaches, and microsurgery have improved outcomes. Over 26 years we treated 36 patients including: 24 with germinoma; four, teratoma; three, pineal cyst; and one each, embryonal carcinoma, choriocarcinoma, pineocytoma, pineoblastoma and metastasis. All 24 germinomas responded to radiotherapy, as did one case each for teratoma, pineoblastoma, and choriocarcinoma. Eight patients underwent resection, and one patient with germinoma had biopsy via endoscopy. Occipital transtentorial and parieto-occipital transcallosal approaches were used in three each, and infratentorial supracerebellar and a combined infratentorial supracerebellar and occipital transtentorial approach in one each. Total removal was achieved in four patients (50%), and subtotal removal in two. Postoperative complications resolved within 2 weeks. Two patients had recurrence and one had dissemination. All tumours except germinomas should be explored and diagnosed histologically. Current operative techniques allow safe, effective removal of pineal region tumours.     

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     

Isolated pineal region metastasis of small cell lung cancer.

The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. Rarely the symptoms of metastatic involvement of the pineal gland precede those of the primary tumor or other metastatic sites. An 83 year-old man presented with gait disturbance and limitation of upward gaze. Brain MRI showed homogeneous enhancement of a solitary mass in the pineal region with obstructive hydrocephalus. A stereotactic biopsy was performed, and small cell carcinoma was diagnosed. A systemic investigation for the primary lesion subsequently revealed small cell carcinoma of the lung. The patient was referred for radiotherapy and chemotherapy. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors, particularly in elderly patients.     

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.     

Intracranial germinomas in a father and his son

Background

Primary intracranial germ cell tumors (IGCTs) represent an uncommon category of neoplasms, and familial occurrence is rare. We present the first report of parent-child patients with pathologically confirmed pure germinomas.

Case report

A 36-year-old Japanese man presented with diabetes insipidus and hypopituitarism. Magnetic resonance imaging (MRI) revealed a mass lesion in the pituitary stalk, which was diagnosed as a pure germinoma by open craniotomy tumor biopsy. Seven years later, his 13-year-old son also presented with diabetes insipidus. MRI revealed mass lesions in the pituitary stalk and the pineal region. He underwent endoscopic tumor biopsy for the pineal lesion, which was diagnosed as a pure germinoma. Both the father and his son were treated with combined radiochemotherapeutic regimens and achieved complete remission after one to two cycles of chemotherapy.

Conclusion

Although there have been three previous case reports of familial germinoma, all of these involved sibling pairs. The present report represents the first parent-child cases. This type of familial occurrence suggests the possibility that germline mutations may also be involved in the development of IGCTs.     

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.