Pineal region tumors in children

Department of Neurological Surgery, School of Medicine, University of California, San Francisco.
Journal of Neurosurgery (Impact Factor: 3.74). 06/1988; 68(5):689-97. DOI: 10.3171/jns.1988.68.5.0689
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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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    • "As a very important neuroendocrine organ, pineal gland can influence many physiological activities, such as circadian rhythm, aging and sexual maturation (Hasegawa et al., 1987; Korf, 1994; Lew, 1987; Pevet, 1988; Reyes, 1982). This gland can be involved by several kinds of lesions, in particular tumors and cysts (Cho et al., 1998; Edwards et al., 1988; Yamamoto, 2001). The prevalence of pineal tumors is about 3–8% of intracranial tumors in children and 0.4–1.0% of brain tumors in adults (Cho et al., 1998; Maher and Raffel, 2004; Packer et al., 2000). "
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    ABSTRACT: It is usually difficult to distinguish small pineal tumors via routine or enhanced magnetic resonance (MR) scan. The knowledge of normal pineal size is helpful to detect small pineal lesions, while very few true volumetric data of pineal glands have been reported. Therefore, we obtained the accurate reference range of normal pineal volumes in 112 individuals aged 20-30 years recruited randomly from a healthy community sample. Transverse and sagittal 3.0T magnetic resonance data were obtained using three-dimensional (3D) T1-weighted FSPGR and T2-weighted SE sequences. True pineal volumes were measured from T1-weighted images, while estimated volumes were calculated using pineal length, width and height. All the glands were divided into three types according to the maximum inner diameter of pineal cysts. The prevalence of asymptomatic pineal cyst is 25.00%, with a slight female predominance. In the whole sample, we found no gender differences of pineal volume, but a significant gender difference of pineal volume index. A significant correlation between pineal volume and asymptomatic cyst was found. After excluding cases with big pineal cysts, there were significant correlations between pineal volume and head circumference, body height and body weight, respectively. This study suggests that asymptomatic pineal cysts may exert an important influence on pineal volume.
    International journal of developmental neuroscience: the official journal of the International Society for Developmental Neuroscience 09/2009; 27(7):655-60. DOI:10.1016/j.ijdevneu.2009.08.002 · 2.58 Impact Factor
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    • "The use of chemotherapy for intracranial NGGCTs has been suggested as it has been effective in the treatment of testicular non-seminomatous germ cell tumors, although there have been contradictory report as to the success of this approach [11]. NGGCTs are often included in one category because of their rarity, but the patterns of relapse are reported to be different among subtypes [3] [6] [12] [16]. We analyzed intracranial NGGCTs retrospectively to identify the prognosis and to investigate the optimal irradiation method for each histopathological subtype of this rare disease. "
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    ABSTRACT: The treatment outcome of 24 patients with pathologically-proven non-germinomatous germ cell tumor was retrospectively investigated to determine the effectiveness of radiotherapy. The patients were divided into three groups as follows: group 1, five patients with mature teratoma with or without germinoma; group 2, six patients with immature teratoma with or without germinoma; group 3, 13 patients with other highly malignant tumors. The overall actuarial survival and relapse-free rates at 5 years were 82% and 59%, respectively, with a median follow-up period of 62 months. The actuarial relapse-free rate at 5 years was 100% for group 1, 63% for group 2 and 44% for group 3. There was no difference in the relapse-free rates between total resection and partial resection. Usage of chemotherapy was adversely related to survival probably due to selection bias. No local failure was observed with 10 Gy or more for group 1,40 Gy or more for group 2 and 54 Gy or more for group 3. In groups 1 and 2, there was no spinal relapses without craniospinal irradiation. In group 3, three of eight patients who did not receive craniospinal irradiation and none of five patients who received craniospinal irradiation experienced spinal relapse. In conclusion, highly malignant GCTs show a high incidence of spinal metastasis and craniospinal irradiation may reduce the risk of spinal metastasis. Radiation dose and volume are to be determined according to the histopathological aggressiveness.
    Radiotherapy and Oncology 11/1998; 49(1):55-9. DOI:10.1016/S0167-8140(98)00081-4 · 4.36 Impact Factor
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    • "The estimated risk of neurocognitive injury to the developing brain [27] does not justify the use of 20 Gy irradiation as a substitute for recent surgical diagnosis that is associated with minimal mortality and morbidity [11]. Thus, the importance of pathological confirmation of tumors has been increasingly emphasized in the past decade [11] [17] [19] [21] [30]. "
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    ABSTRACT: A retrospective multi-institutional study was conducted to survey what percentage of intracranial germinomas were treated with pathological confirmation before radiotherapy and to investigate the influence of field selection on outcome. Thirty-seven percent of patients (41 of 110 patients) were pathologically confirmed before radiotherapy during the past 16 years at eight institutions in Northern Japanese prefectures. Pathological confirmation was obtained in 26, 37 and 53% of cases during 1978-1983, 1984-1989 and 1990-1994, respectively. All 110 patients were examined using computed tomography (CT) scans. Among the 41 patients with pathologically confirmed germinoma, radiation fields were craniospinal in 23 patients, whole-brain in 10 patients and local without ventricle inclusion in eight patients. For the 41 patients with pathologically confirmed germinoma, the actuarial and cause-specific survival rates were 91/94% at 5 years and 87/90% at 10 years, respectively. The relapse-free survival rate at 10 years was 90. 76 and 22% for the craniospinal field, whole-brain field and local field without ventricle inclusion, respectively. Pathological confirmation was obtained in only 37% of CT-scan era cases, although the confirmations were more commonly carried out later in the study period. Limited local irradiation alone without ventricle inclusion cannot be recommended for localized tumors even with the help of CT scanning.
    Radiotherapy and Oncology 06/1998; 47(2):201-5. DOI:10.1016/S0167-8140(98)00017-6 · 4.36 Impact Factor
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