手术治疗松果体区肿瘤临床分析

目的观察松果体区肿瘤患者施行神经外科手术治疗的效果及其预后。方法对1985～2001年收治的30例松果体区肿瘤患者的病理类型、临床资料、治疗方法及预后进行回顾性分析。结果30例患者中,典型Parinaud综合征患者6例;X线检查显示颅内高压者3例;CT及MRI均示第三脑室后部占位性病变,两侧脑室及第三脑室扩大。全部患者均施行手术治疗,经枕下幕上入路者19例,幕下小脑上入路者11例;19例同时行第三脑室后部造瘘;肿瘤全切除11例,次全切除8例,部分切除8例,活检加内分流3例;病理检查结果显示,生殖细胞肿瘤15例,松果体实质肿瘤5例,脑膜瘤3例,室管膜瘤1例,星形细胞瘤2例,其它病变4例。随访11例(1～6年),其中生存5年以上者7例;复发4例,其中3例死亡。结论松果体区最常见的肿瘤是生殖细胞肿瘤,临床表现多无特异性。手术既可切除肿瘤,解除梗阻性脑积水,又可获得充足的组织标本明确肿瘤性质,以指导下一步的治疗。松果体区肿瘤患者的预后大多良好。     

Germ cell tumors of the thalamus and the basal ganglia

Two cases of germ cell tumors (GCTs) of the basal ganglia are presented and 40 previously reported cases are reviewed. The incidence of GCTs of the basal ganglia and thalamus was estimated as less than 14% of all intracranial GCTs. All patients except for two (95%) were male, aged 7–19 years. The clinical course was usually slow. The major symptoms were hemiparesis, mental deterioration such as dementia or character change, precocious puberty, diabetes insipidus, oculomotor palsy, speech disturbance, and hemianopsia. Signs of intracranial hypertension did not occur until the late stages of the disease. The plain CT finding was characterized by an irregularly defined, slightly high-density area frequently accompanied by central low-density areas without significant mass effect. The tumors showed mild to moderate and nonhomogeneous contrast enhancement. An ipsilateral cerebral hemiatrophy was often found. MR images demonstrated the corresponding findings. GCTs of the basal ganglia had a high possibility of containing components other than germinoma such as choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma. Thus, tumor markers in the serum, CSF, or cyst fluid were frequently positive. With recent refinement of microsurgical techniques as well as immunohistochemical study and measurements of tumor markers of serum, CSF, and cyst fluid, major resections of tumor, accurate pretreatment histologic diagnosis, and early determination of the specific types of this tumor appear to be readily possible. This is essential for effective treatment of patients not only with radiosensitive germinoma, but also those with radioinsensitive nongerminoma variants and a combination of them located in this region.     

基底节区生殖细胞瘤

目的加深对基底节区生殖细胞瘤的认识。方法报告经手术病理证实的基底节区生殖细胞瘤１０例，均为男性，平均年龄１４岁，平均病程２．６年。主要临床症状为一侧肢体无力、共济失调、头痛及性早熟。手术切除肿瘤９例，定向活检１例。术后均予放疗。结果临床症状改善６例，无变化３例，加重１例。随访３个月至５年，除１例于术后第３年死亡外，其余９例均生活良好。结论基底节区生殖细胞瘤的诊断主要依据临床表现、肿瘤标记物检测及ＣＴ检查，必须与该部位常见的胶质瘤相鉴别。治疗为手术加放疗。     

Germinoma in cerebral hemisphere associated with Down syndrome

A Down syndrome patient with germinoma developing in the cerebral hemisphere is reported. A review of the literature yielded only 14 cases of Down syndrome with brain tumors, including our case. This finding of brain tumors in patients with Down syndrome may reflect chance occurrence. However, it is of interest in this regard that in 6 of the 14 (43%) reported cases the lesions were intracranial germ cell tumors. Received: 15 March 1996 Revised: 20 May 1996     

Nonsurgical treatment of huge suprasellar germinoma

A case describing a huge, suprasellar germinoma with subfrontal extension that produced striking emaciation of the patient as an initial symptom is discussed. Diagnosis of germinoma was made on the basis of a computed tomography scan and cytology of the ventricular fluid. The tumor responded well to radiation treatment, which may indicate that it is not always necessary to reduce tumor bulk by direct surgical intervention before radiation therapy.     

Primary intracranial germ cell tumours

Summary A histological study has been made of a retrospective series of 17 primary intracranial germ cell tumours found in a collection of 3550 intracranial neoplasms (incidence of 0.48%). All, except for two differentiated teratomas (one extracerebral in a neonate and another in the lateral ventricle), were situated in the midline in persons aged 5 to 37 years (13 males, 4 females). 12 tumours were located in or originated from the (para)pineal region, two of them also invaded the hypothalamus, while three germinomas occupied the retrochiasmal (supra/intrasellar) region without pineal involvement. There were 11 rather pure tumours (7 germinomas, 4 teratomas of various differentiation) and six mixed neoplasms (2 germinomas with teratoid areas, 3 embryonal carcinomas containing elements of endodermal sinus tumour, choriocarcinoma and germinoma, and one teratocarcinoma with endodermal sinuses). Only one case showed prominent features of endodermal sinus tumour, but characteristic elements of this type were present in four other mixed tumours. All germinomas and germinomatous parts of mixed neoplasms showed an inflammatory reaction of varying intensity, in 6 cases associated with multinucleated giant cells, which may be related to the prognosis of these tumours (one patient with hypothalamic germinoma is alive 6 years after radiotherapy). The close structural similarities between the various types of intracranial and gonadal dysgerminomas and their frequent combination within the same tumour support the concept of a common histogenesis of germ cell tumours regardless of their site of origin. Difficulties of classification may arise from the rather frequent occurrence of mixed germ cell neoplasms.Dedicated to E. Frauchiger, on the occasion of his 70th anniversary.     

Infundibular germinoma

Summary Five cases of germinoma occurring in the infundibular region are presented. The clinical symptoms and treatment as well as the cytological and histological pictures, including the ultrastructural findings, are described. When unassociated with other neoplastic germ cell elements, infundibular germinoma is considered to be a highly radiosensitive tumour with a good prognosis.     

Podoplanin expression in primary central nervous system germ cell tumors: a useful histological marker for the diagnosis of germinoma

Podoplanin, a mucin-like transmembrane sialoglycoprotein, promotes platelet aggregation and may be involved in cancer cell migration, invasion, metastasis, and malignant progression. Podoplanin/aggrus is highly expressed in testicular seminoma, suggesting that it may be a sensitive marker for testicular seminomas. Here we investigated the expression of podoplanin in central nervous system (CNS) germ cell tumors (GCTs) by immunohistochemical staining of tumor samples from 62 patients. In 40 of 41 (98%) germinomas (including germinomatous components in mixed GCTs), podoplanin was diffusely expressed on the surface of germinoma cells; lymphocytes, interstitial cells, and syncytiotrophoblastic giant cells were negative for podoplanin. Except for immature teratomas (12/17; 71%), podoplanin expression was absent in non-germinomatous GCTs, including seven teratomas, seven embryonal carcinomas, seven yolk sac tumors, and seven choriocarcinomas. In immature teratomas, focal podoplanin staining was observed in fewer than 10% of immature squamous and columnar epithelial cells. Thus, podoplanin expression may be a sensitive immunohistochemical marker for germinoma in CNS GCTs. As such, it may be useful for diagnosis, for monitoring the efficacy of treatment, and as a potential target for antibody-based therapy.Kazuhiko Mishima and Yukinari Kato contributed equally to this work.     

鞍区生殖细胞瘤MRI影像分析(附11例报告)

目的:分析鞍区生殖细胞瘤的MRI表现及临床特征。方法:选择经病理或临床实验室检测及试验性治疗证实的11例鞍区生殖细胞瘤患者作为研究对象,结合文献回顾性分析鞍区生殖细胞瘤治疗前MR影像表现。结果:11例患者均表现为垂体柄增粗和垂体后叶正常短T1高信号消失,9例有中枢性尿崩症。MRI图像:T1WI肿瘤呈等信号,但在T2WI上信号不均匀表现为多样性。3例肿瘤延伸入第三脑室并囊变坏死,1例肿瘤沿四脑室及侧脑室播散转移。结论:青少年女性出现的鞍区实性病变,T2上信号不均匀,有显著强化,伴垂体柄增粗,垂体后叶短T1高信号消失,高度提示生殖细胞瘤,影像与临床结合有利于早期诊断,改善生殖细胞瘤预后。