Pineal region tumors in children.

Department of Neurological Surgery, School of Medicine, University of California, San Francisco.
Journal of Neurosurgery (Impact Factor: 3.23). 06/1988; 68(5):689-97. DOI: 10.3171/jns.1988.68.5.0689
Source: PubMed

ABSTRACT The authors believe that the preferred treatment for pineal region tumors in children requires definitive surgery with a histological diagnosis and that a conservative approach consisting of shunting and radiation therapy no longer seems to be appropriate. The results are reported of a retrospective review of the presentation, treatment, and outcome of 36 children under the age of 18 years treated between 1974 and 1986. Eleven children had germinomas (two-cell type), seven had astrocytomas, and the remaining 18 had 15 histologically different tumor types. Surgery was performed on 30 patients; there were no deaths, but a 10% rate of persistent morbidity was found. The median follow-up period was 4 years. Nine (82%) of 11 patients with germinomas are alive without evidence of recurrence; one child died from recurrent tumor in the pineal region and another is presently being treated for recurrent tumor of the spinal cord. Six (86%) of the seven patients with astrocytoma are well after biopsy and radiation therapy. Of the remaining 18 children, five (28%) died from tumor progression. The cerebrospinal fluid (CSF) tumor markers alpha-fetoprotein and beta-human chorionic gonadotropin were helpful in determining the presence of malignant germ-cell tumors, particularly those with a poor prognosis. Magnetic resonance imaging was useful for diagnosis and for planning the operative approach. Magnetic resonance images showed the presence of pineal region tumors in four children with hydrocephalus who had no evidence of tumor on computerized tomography scans. Because the great variety of tumor types found in the pineal region must be treated in different ways and because improved microsurgical and stereotaxic surgical techniques have made mortality and morbidity rates acceptably low, a biopsy diagnosis should be obtained in all patients. Preoperative assessment of CSF tumor markers and cytology is useful for the identification of patients who have a poor prognosis.

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    ABSTRACT: Objective : Pineal region tumors (PRTs) are uncommon, and treatments vary among neoplasm types. The authors report their experience with gamma knife surgery (GKS) as an initial treatment in a series of PRT patients with unclear pathological diagnoses. Method : Seventeen PRT patients with negative pathology who underwent GKS were retrospectively studied. Nine patients had further whole-brain and spinal cord radiotherapy and chemotherapy 6-9 months after GKS. Results : Sixteen of 17 cases were followed up over a mean of 33.3 months. The total response rate was 75%, and the control rate was 81.3%. No obvious neurological deficits or complications were attributable to GKS. Conclusion : The findings indicate that GKS may be an alternative strategy in selected PRT patients who have negative pathological diagnoses, and that good outcomes and quality of life can be obtained with few complications.
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    ABSTRACT: In the current study, we reviewed and re-evaluated the experiences of microsurgical management for pineal region meningiomas via the unilateral occipital transtentorial approach (Poppen's approach). Clinical data were obtained on 15 meningiomas of the pineal region, which underwent microsurgery via unilateral Poppen's approach from March 2009 to June 2012. These patients were hospitalized in our department; their data were collected and analyzed retrospectively. The tumors were removed via the right Poppen's approach in 12 cases and left Poppen's approach in 3 cases, and intraoperative external ventricular drainage was performed for hydrocephalus in 3 cases. As a result, gross total resection was achieved in 11 cases, near total resection in 3 cases and subtotal resection in 1 case. All resected tumors were pathologically confirmed. The postoperative complications included two cases of homonymous hemianopia, and deteriorated Parinaud syndrome and diplopia in one case. Ten cases were followed up (range 1–4 years) and no death occurred. On the basis of the existing literature and our experiences, the unilateral Poppen's approach is appropriate for most meningiomas of the pineal region that are small or intermediate in size. However, gross total resection might be difficult via the unilateral Poppen's approach for large-sized meningiomas with much contralateral infratentorial extension due to limited exposure. For these cases, combined supra-infratentorial or bilateral Poppen's approaches are recommended. Preoperative or intraoperative external ventricular drainage can increase tumor exposure and improve microsurgical effects.
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    ABSTRACT: We present an unusual case of a germinoma of the pineal region arising adjacent to an epidermoid cyst in a 16-year-old male. Initial imaging findings were classic for epidermoid cyst. The patient underwent two partial resections at an outside institution, each specimen demonstrating pure epidermoid cyst. Follow-up imaging over a period of 24 months showed an area of progressive contrast enhancement adjacent to the initial lesion, suggesting the development of a neoplasm. Given the area of contrast enhancement in addition to worsening headaches and visual changes, he underwent a third and final resection at our institution. Pathology revealed a mixed germ cell tumor with prominent germinoma component in addition to a well-differentiated epidermoid cyst. Details of his imaging and pathologic findings are presented, and possible explanations for these findings are explored, the most likely of which is lack of complete resection at the onset failed to identify the whole of the neoplasm. We conclude that pediatric epidermoid cysts of the pineal region should always receive close follow-up, particularly when total resection is not performed.
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