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.
Extrapontine myelinolysis (EPM) is caused by marked fluctuation of the serum electrolyte level. Patients with suprasellar germ cell tumors frequently present with diabetes insipidus, which is often aggravated by administration of steroid hormone. In addition, cisplatin-based chemotherapy is sometimes accompanied by marked serum electrolyte fluctuation because it needs massive hydration to prevent renal damage. Two children with suprasellar germ cell tumors in whom EPM developed secondary to profound hyponatremia and was rapidly corrected are described. The central pons was spared in both cases. Clinically the children showed transient neurological deficits including confusion, pseudobulbar palsy, and deterioration of consciousness. MRI demonstrated bilateral symmetrical, high-signal-intensity (HSI) lesions on T2-weighted images (T2WI) at the basal ganglia and adjacent cerebral cortex. Follow-up T1WI a few months later revealed newly developed HSI lesions in the basal ganglia. The patients gradually improved, but the neurological deficits did not completely disappear. During the perioperative management of suprasellar germ cell tumors, EPM should be considered when a patient has a significant electrolyte imbalance and neurological deficits, especially confusion and pseudobulbar palsy.
Though intracranial endoscopic surgery has several advantages, poor visibility caused by bleeding from the operative field is one of its limitations. In two cases involving endoscopic III ventriculostomy the authors encountered moderate bleeding, and this was controlled with 'water column tamponade.' Following the application of hydrostatic pressure of 40-50 cmH2O for less than 2 min, the bleeding temporarily stopped and the operative field became clear. Several applications of this tamponade made it possible to complete the procedures. Although the pressure applied by the water column was not high enough to disturb the cerebrovascular autoregulation, blood pressure rose significantly during application of the water column tamponade. This method was valuable for the completion of procedures when bleeding was not at high pressure from large arteries. Careful monitoring of the blood pressure is mandatory, and adjustment of the pressure applied will make the procedure safer.
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