第四脑室肿瘤的诊断和显微手术治疗

目的分析第四脑室肿瘤的诊断,探讨显微手术切除颅脑肿瘤的效果。方法86例颅脑肿瘤患者,显微镜下手术切除第四脑室肿瘤,其中全切62例,次全切19例,大部切除5例。术后有39例患者行放射治疗。结果86例患者中,3例术后10 d内死亡;83例生存,且症状均有缓解,平均生存期>3年。术后病理诊断髓母细胞瘤32例,室管膜瘤23例,星型胶质细胞瘤15例,血管母细胞瘤10例,脉络丛乳头状瘤2例,表皮样囊肿4例。结论对髓母细胞瘤、星型胶质细胞瘤、血管母细胞瘤应尽可能做到全切,室管膜瘤与脑干粘连紧密者,可考虑次全切除。颅脑恶性肿瘤患者,应结合放疗等综合治疗。     

16例骶尾部脊索瘤放疗随访5年疗效观察

脊索瘤是一种少见的低度恶性肿瘤。手术治疗为主要手段，但是由于肿瘤所处部位和肿瘤常侵犯椎旁间隙、椎间隙、椎管、硬脑膜等部位而不能彻底切除，术后易复发需做放疗。脊索瘤曾被认为对放射线不敏感。术后常规放疗量为７０～８０Gy。本文追踪５年观察了１６例骶尾部脊索瘤的术后放疗或单纯性放疗疗效，以及采用大剂量分段放疗后放射性损伤的发生情况，探讨放疗在脊索瘤临床治疗中的作用。１材料与方法１．１临床资料１６例患者中男１０例，女６例。其中年龄３０～４９岁者１２例。１３例手术切除后放疗，３例因手术困难行单纯性放疗。１…     

颅内生殖细胞瘤的放射治疗

颅内生殖细胞瘤的放射治疗张峰副主任医师姜翠琴空军总医院放射治疗科（１０００３６）我院自１９８６年９月至１９９３年３月放射治疗颅内生殖细胞瘤９例，报道如下：资料与方法本组９例，全部男性，年龄６－２８岁，平均年龄１８．８岁，肿瘤位于松果体区７例，松果体区...     

松果体瘤的放疗及CT观察

松果体瘤较少见,其中真性松果体瘤更罕见,多为生殖细胞瘤。因肿瘤位于颅内中线深部,手术难度大,不易彻底切除,活检亦很困难,然而对放射线敏感,疗效颇好。我院采用CT检查,观察肿瘤位置,形态,既可精确定位设野,亦可观察肿瘤消退情况,并依之调整剂量判定疗效。现简介我们治疗的10例患者。 10例中男9例、女1例。年龄7～37岁。4例经手术病理证实为生殖细胞瘤,余6例依CT检查为松果体区肿瘤。 使用远距离~(60)CO治疗机,依CT片的肿瘤形态     

X线多平面旋转和固定野放射治疗鞍区肿瘤27例

我院利用美国瓦里安２３００Ｃ／Ｄ直线加速器,采用多平面旋转和固定野的照射技术,对鞍区肿瘤进行根治剂量照射。现将该技术报告如下。方法　１９９７年６月至１９９８年５月,我们共收治了２７例鞍区肿瘤患者,男性１７例,女性１０例,年龄１７岁～７０岁,平均３８岁。其中,垂体腺瘤１６例,颅咽管瘤５例,松果体瘤２例,其它肿瘤４例。根据症状体征、ＣＴ或ＭＲＩ诊断：视力减退１８例、头痛１０例、头晕７例、月经紊乱５例、肢端肥大症３例,其中２３例经手术切除,术后６个月内查ＣＴ或ＭＲＩ证实复发４例、肿瘤残留１２例、出现临…     

松果体区肿瘤的显微手术治疗：21例经验

背景与目的：松果体区肿瘤治疗目前仍存在争议,为此我们进一步探讨松果体区肿瘤的临床特点及显微手术治疗。方法：回顾性分析2002。2008年收治的21例松果体区肿瘤患者的临床资料。结果：肿瘤全切除15例,次全切除4例,部分切除2例。21例的手术病理结果中,生殖细胞瘤9例、松果体母细胞瘤5例、胶质瘤2例及其他肿瘤5例。结论：松果体区肿瘤的诊断须以病理组织学分类为依据．松果体区静脉系统是阻碍手术操作的主要结构．显微外科手术应可良好地保护此区静脉．是松果体区肿瘤全切除的关键所在。     

中枢神经细胞瘤1 7例临床分析

目的：报道17例中枢神经细胞瘤，结合文献讨论并总结神经细胞瘤的临床特点和治疗方法。方法：回顾性分析1997年1月至2000年10月17例神经细胞瘤，并复习文献。结果：17例肿瘤14例全切除，3例近全切除，死亡1例。术后均行放射治疗。结论：中枢神经细胞瘤手术结合放疗为最佳治疗方法，预后良好。     

颅内非松果体区生殖细胞瘤10例临床分析

目的：分析颅内非松果体区生殖细胞瘤临床表现、检查、诊断和治疗的特点。方法：对我院1993—2007年收治的10例颅内非松果体区生殖细胞瘤进行回顾性分析。结果：男性7例,女性3例;发病部位：鞍区8例,基底节区2例。经手术或脑组织活检病理诊断确诊5例,经诊断性放疗拟诊5例。5例行肿瘤部分或全部切除术;5例行放疗,其中3例联合化疗,症状均有不同程度缓解。结论：颅内生殖细胞瘤多发于青少年,男性多于女性,放射治疗是有效的治疗方法,压迫症状明显患者可行手术治疗。     

原发性阴道恶性黑色素瘤18例报道

目的探讨原发性阴道恶性黑色素瘤的诊断、治疗特点及影响预后的因素。方法我们对收治的１８例阴道恶性黑色素瘤的临床资料进行了回顾性分析,平均年龄５１岁,主要症状为阴道出血排液和肿物,原发肿瘤多位于阴道前壁的下１／３处;１２例行手术＋放疗／化疗,２例行单纯放疗,１例行单纯化疗,化疗＋放疗３例。结果本组的５年生存率为１１％,３年生存率为３３％,平均生存２６个月,６例存活３年以上者均为Ⅰ、Ⅱ期,其中１例为单纯放疗者肿瘤消退存活４８个月,Ⅲ、Ⅳ期患者无１例存活超过２年,１２例手术病人中有和无淋巴结转移及脉管瘤栓者,平均生存分别为８和４２、１６和４０个月。结论阴道恶性黑色素瘤治疗以手术为主,大单次量少分割放疗可使肿瘤消退或缩小,其预后与分期、淋巴结转移及脉管瘤栓有关。     

保留肢体的四肢恶性骨肿瘤段截除（附27例报告）

对２７例四肢恶性骨肿瘤患者应用４种术式的保肢肿瘤段截除术。所有病例均病理证实，其中骨肉瘤８例，软骨肉瘤３例，纤维肉瘤２例，恶性纤维组织细胞瘤２例，骨巨细胞瘤Ⅱ～Ⅲ级６例，网状细胞肉瘤与骨髓瘤各１例，骨转移癌４例。手术方式：肩胛带整块切除１３例，前臂远端截除再植１例，骨盆部分切除６例，大腿中下段截除小腿旋转成形７例。２４例随访１６个月～１２年以上，平均６年８个月。结果：随访２４例２０例存活２年以上，２年存活率８３．３％。５年仍健在９例，５年生存率３７．５％。Ｅｎｎｅｋｉｎｇ分期ⅠＢ期４例，ⅡＢ期５例，且患肢有较好的功能。对四肢恶性骨肿瘤应用保留肢体的肿瘤段截除，配合化疗与放疗可获得较满意的疗效     

The recurrence of primary intracranial germinomas

Summary Twenty three cases of primary intracranial germinomas including five cases of germinomas with syncytiotrophoblastic giant cells are studied and analyzed, with special reference to the recurrence under radiotherapy. The follow-up period for all cases was 7 months to 12 years (average: 5.8 years) with that for pure germinomas ranging from 8 months to 12 years (average: 5.7 years) and that for germinomas with syncytiotrophoblastic giant cells ranging from 7 months to 11 years (average: 6.3 years). Late recurrence was observed in three cases (3/23, 13%), developing outside of the initial irradiation field.With regard to recurrence, significant correlation to radiation fields was evident, while it was not to radiation doses. Furthermore, germinoma with syncytiotrophoblastic giant cells showed a more significant tendency to recur than pure germinoma. The radiotherapy of germinomas is discussed and the clinical features of germinoma with syncytiotrophoblastic giant cells are presented.     

Radiation therapy for intracranial germinoma: the case for limited volume treatment

Although radiation therapy has been universally accepted as the treatment of choice for primary intracranial germinomas, the optimal treatment technique continues to be a matter of controversy. Some authors advocate routine prophylactic craniospinal irradiation for all patients with localized intracranial germinomas whereas others have recommended partial brain fields. A retrospective analysis was performed on all 30 patients with tumors of the pineal and suprasellar regions irradiated at our institution between 1967 and 1987. Definitive histology was obtained in 23 patients. There were 13 germinomas and 10 non-germinomatous germ cell tumors. The 13 patients with biopsy proven germinomas constitute one of the largest modern series and will form the basis of this report. In all 13 patients, the tumor was confined to a single site within either the pineal region (9 patients) or the suprasellar region (4 patients). One patient with a pineal germinoma had CSF cytology positive for malignant cells and this patient was treated with craniospinal irradiation. Twelve patients were treated using partial brain fields encompassing either the tumor plus margin (10 patients) or the entire ventricular system followed by a boost to the primary tumor (2 patients). With the exception of one patient, all patients received a total dose between 4900 and 5500 cGy to the primary (median 5040 cGy). The patient receiving less (3960 cGy) suffered from both local and spine relapse 26 months after diagnosis. The remaining 12 patients were free of disease at 81 months median follow-up time. We suggest that for patients with biopsy proven pineal or suprasellar germinomas, irradiation of partial brain is sufficient and craniospinal irradiation should be administered only to those patients with disease involving more than one intracranial site, demonstrated meningeal seeding, or positive CSF cytology.     

Suprasellar germinomas in childhood. A reappraisal

Germinomas in childhood may arise in both the suprasellar and pineal region, and outcome has been reported to be worse for suprasellar germinomas with a 5-year survival rate of 20% as compared to 60% for pineal germinoma. To determine the factors impacting on outcome, the results of a uniform treatment approach were evaluated and included primary surgical debulking (PSD) and systemic craniospinal axis radiation (CSRT) for suprasellar germinomas. Between 1976 and 1985 ten consecutive patients (seven females, three males) with the pathologically confirmed diagnosis of suprasellar germinoma were treated. Outcome was compared to four male patients with pineal germinoma treated over the same time period and series of patients reported in the literature. At diagnosis the mean age of patients with suprasellar germinoma was 13.9 years (range, 8.9 to 9.4 years). Symptoms were present for a mean of 18 months (range, 2 to 72 months) prior to diagnosis and included diabetes insipidus, anterior pituitary dysfunction, decreased vision, headache, vomiting, and diplopia. Staging studies, including myelography (n = 4) and cerebrospinal fluid cytology (n = 7), disclosed dissemination in only one child. Surgical treatment included biopsy in three cases, partial resection in five, and total resection in two; no permanent postsurgical complications were noted. The mean radiation therapy dose to the tumor site was 4953 cGy (range, 4400 to 5250 cGy) and to the spine 3354 cGy (range, 3000 to 4000 cGy). Patients were followed for a mean period of 5.1 years (range, 1.9 to 10.5 years). One patient with SG who did not receive treatment initially developed a pineal tumor after diagnosis; she was treated with PSD and CSRT and is asymptomatic 5 years later. All the remaining patients are alive and remain disease-free. Surgical resection and CSRT results in excellent disease control for children with suprasellar germinomas, and outcome is similar to those patients with pineal germinoma.     

Combined chemotherapy and radiotherapy for intracranial germinomas in adultpatients: a single-institution study

We report on our experience in the treatment of intracranial germinomas (18 pure germinomas and two germinomas with syncytiotrophoblastic giant cells) according to a strategy of radiotherapy doses and fields reduction after a neoadjuvant chemotherapy (Cisplatin-vinblastine and bleomycin combination). Radiation therapy was delivered after the completion of the third and last course of chemotherapy. For the solitary germinoma the target volume was the gross tumour volume. In the five multifocal germinoma patients the whole ventricle volume was irradiated. For the single disseminated germinoma patient we treated the whole central nervous system. The cumulative doses were 30 Gy for the pure germinomas. For the STGCs, a cumulative dose of 35 Gy was used. The median follow-up was 55 months (range 12–120). 18 patients were alive without recurrence of disease. In the two patients with STGCs the death took place 16 and 35 months after diagnosis.     

41例原发鞍上+松果体区生殖细胞瘤治疗分析及策略探讨

目的 分析颅内原发鞍上+松果体区生殖细胞瘤患者的治疗结果及治疗策略的探讨。方法 1996—2013年放疗鞍上+松果体区生殖细胞瘤患者 41例,男 35例、女 6例,年龄 5~39岁。经病灶活检病理诊断 5例,其余为临床诊断。采用常规放疗 33例、IMRT 8例;5例病理诊断者进行化放疗。放疗采用6 MV X线,照射野有全脑室照射+瘤区补量 6例,全脑照射+瘤区补量 16例,全脑脊髓照射+瘤区补量 19例。肿瘤区照射剂量 37.8~50.0 Gy,预防照射区剂量 17.8~35.0 Gy。Kaplan-Meier法进行生存率计算。结果 5年样本数 26例。5年OS率为95%、RFS率为85%。复发转移 8例,其中脊髓转移 4例、脑室播散 1例、脑室周边复发 3例。22例未进行脊髓照射者脊髓转移 4例,19例进行脊髓照射者无脊髓转移。8例IMRT者及 5例化放疗者随访期内均未出现治疗失败。结论 颅内生殖细胞瘤放疗效果好,双部位生殖细胞瘤不进行脊髓照射者失败率较高,建议对双部位病灶的患者进行脊髓照射。     

Pathological features of intracranial germinomas with reference to fibrous tissue and granulomatous change

Intracranial germinomas are accompanied occasionally by a significant granulomatous change and abundant fibrous tissue, and this has made their pathological diagnosis difficult. However, the incidence of the granulomatous reaction and the presence of fibrous tissue together with their clinical characteristics in intracranial germinomas have not been fully investigated. Twenty-four germinomas, none of which had received preoperative treatment, were clinicopathologically examined. The location of the tumor was the pineal region (5 cases), the suprasellar region (13 cases), multiple lesions (2 cases), the basal ganglia region (1 case), and other regions (3 cases). Histologically, the germinomas could be divided into two types: (1) type A (18 cases) consisted mainly of large neoplastic cells and small lymphocytes, showing a two-cell pattern; (2) type B (6 cases) consisted predominantly of fibroinflammatory tissues containing occasional neoplastic cells (5/6 cases) and, rarely, neoplastic cells (1/6 cases). Perioperatively, two-cell-pattern germinomas (type A) were characterized as soft tumors and fibroinflammatory germinomas (type B) as hard tumors. Thus, the fibroinflammatory type B accounted for 25% of the intracranial germinomas. Although there were no topographical and clinical differences between the two types, we conclude that immunohistological studies to detect neoplastic germ cells are warranted in cases of small stereotactic biopsies of hard type B tumors.     

Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation

A retrospective study was performed on all patients with biopsy-proven intracranial germinomas and unbiopsied suprasellar or pineal region tumors treated during the past 30 years in the Department of Radiation Oncology, University of California, San Francisco. A total of 33 patients were treated: 13 with biopsy-proven germinomas, and 20 others who were unbiopsied. All patients were treated with megavoltage equipment; total dose varied between 40-55 Gy. Only two patients were treated with prophylactic spinal irradiation. No patient received initial or adjuvant chemotherapy. Follow-up times for biopsy-proven patients ranged from 0.5 to 16.7 years with a median 5.3 years. No biopsy-proven patient had a recurrence of the tumor or died; thus, actuarial relapse-free and determinate survivals at 5 years were 100%. Although only one patient in this group received prophylactic spinal irradiation, no patient failed in the spinal axis. The 20 unbiopsied patients had follow-up times ranging from 0.1 to 27.5 years with a median of 5.5 years. Six unbiopsied patients died: two from recurrent disease at the primary site, one from distant peritoneal metastases, two from complications of treatment, and one from intercurrent disease. For this group, actuarial relapse-free survival at 5 years was 72%; the corresponding determinate survival was 73%. Nineteen unbiopsied patients were treated without craniospinal irradiation. Only one developed spinal metastases. The results from this and other series indicate that the risk of spinal metastases from intracranial germinoma is too low to warrant routine prophylactic spinal irradiation. However, patients with gross tumor spill causing contamination of the CSF, malignant CSF cytology, or documented subependymal or subarachnoid metastases presumably are at higher risk for leptomeningeal failure. Craniospinal irradiation is recommended for these patients.     

Long-term functional outcome of suprasellar germinomas: Usefulness and limitations of radiotherapy

We investigated the long-term functional outcome of patients with suprasellar germinoma after radiotherapy to determine the usefulness and limitations of radiotherapy for these tumors. From among 54 cases of intracranial germ cell tumor at Kitasato University Hospital, 12 patients with suprasellar germinoma who were treated with radiotherapy but not chemotherapy retrospectively investigated for mental, hormonal and visual functions. The follow-up period ranged from 63 to 262 months (mean, 161.1 months). The mortality rate was zero and there was no recurrence of tumors. However, three patients treated with local irradiation alone showed metastasis to the ventricles or spinal cord. With regard to mental function, 50% of the patients showed remarkably low mental function after radiotherapy. With regard to pituitary hormonal function, deficiency of ADH, GH, ACTH, and TSH was documented in 75%, 41.7%, 16.7% and 8.3% of the patients, respectively, before radiotherapy. Prolactin was elevated in 50% of the patients. After radiotherapy, 91.7% needed hormone replacement. With regard to visual function, most patients showed an improvement or no change after radiotheray. In conclusion, radiotherapy for suprasellar germinomas gave long-time survival. However, such radiotherapy may cause mental and pituitary hormonal dysfunction. Our results show that while radiotherapy is useful for treating suprasellar germinomas, its dose have some limitations.     

Management of primary intracranial germ cell tumors

Primary intracranial germ cell tumors are rare and usually localized in the pineal and the suprasellar regions. They are divided into the following histologic types: germinoma, teratoma (mature, immature, malignant), choriocarcinoma, embryonal carcinoma, endodermal sinus tumor (yolk sac tumor), and mixed tumors. Clinically, they are manifested with ocular signs or signs of obstructive hydrocephalus. Localized germinomas are treated with radiation therapy and exhibit a relatively good prognosis. Chemotherapy is reserved for disseminated germinomas. Mature teratomas are treated with surgery. The rest of germ cell tumors are managed with various combinations of surgery, chemotherapy, and radiotherapy depending on the tumor type. If the tumors secrete beta-human chorionic gonadotrophin (hCG) or alpha-fetoprotein (FP), these tumor markers can be used to accurately monitor response to treatment. Prognosis is best for germinomas and mature teratomas and worst for choriocarcinomas and embryonal carcinomas.     

The short-term effect of low-dose radiation on intracranial germinoma. A pathologic study.

A correlative pathologic and imaging (computed tomography and magnetic resonance imaging) study is reported of a pineal germinoma in a 35-year-old man who died unexpectedly of massive pulmonary embolism on the 8th day during the course of radiation therapy after receiving a total dose of only 1600 cGy. A histologic study of the entire lesion in serial sections revealed no viable tumor cells. The tumor bed was composed of stromal elements with a variable degree of proliferation. The lymphocytic component of the tumor was unaffected by the radiation. This case provided a rare opportunity to show total eradication of tumor cells in a germinoma after a small dose of radiation and supports the notion, based on imaging observations, that histologically proven intracranial pure germinomas may be treated successfully with a much smaller dose of radiation than previously thought.