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.

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The first known surviving case of acrania is presented. The patient was the first child of a 29-year-old Japanese woman. Fetal ventriculomegaly was documented in the 35th gestational week. Prenatal sonography and magnetic resonance imaging suggested hydrocephalus with a wide encephalomeningocele. The baby was born at 38 weeks of gestational age by vaginal delivery. The patient had no calvarium, but did have a complete skull base with a partial defect in the occipital scalp and an underlying dural defect. Subsequently, the patient underwent repair of the scalp defect. At 3 months of age, after hydrocephalus developed, a subduroperitoneal shunt was placed because of cosmetic and nursing problems. His developmental quotient was 10 at 3 years.

The authors treated a patient with a huge, ventrally located spinal meningioma, extending from the foramen magnum to the second thoracic vertebral body. Vertebral body corpectomies were performed between the bottom of the second cervical vertebral body and the top of the first thoracic vertebral body, and the tumor was completely resected. Neurological symptoms and signs improved postoperatively.

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