Pineal region tumors in children

1988 ◽  
Vol 68 (5) ◽  
pp. 689-697 ◽  
Author(s):  
Michael S. B. Edwards ◽  
Roger J. Hudgins ◽  
Charles B. Wilson ◽  
Victor A. Levin ◽  
William M. Wara

✓ 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 α-fetoprotein and β-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.

1983 ◽  
Vol 58 (5) ◽  
pp. 654-665 ◽  
Author(s):  
Rashid Jooma ◽  
Brian E. Kendall

✓ The management of pineal region tumors remains controversial. Advocates of a conservative approach emphasize the excellent results of radiotherapy, particularly with germinomas, while a number of recent reports have demonstrated the safety of direct surgery. In order to improve treatment planning, attempts have been made to distinguish the various tumor types by computerized tomography (CT) and by the use of markers for germ-cell tumors. This paper reports a study of 35 patients with pineal tumors including two ectopic germinomas. Pretreatment CT was performed in all 35 patients, and human chorionic gonadotropin and alpha-fetoprotein levels were assayed in 11. Histological verification was available for 33 tumors; the other two were characterized by clinical and CT features plus tumor markers. Of the 27 tumor operations, 21 were for a pineal mass and six for ectopic germinoma or metastasis. There was no operative mortality. Morbidity was minimal, and surgery did not increase the incidence of subarachnoid seeding. Each histological type of tumor has a typical appearance on CT scanning, although, in the individual case, a firm diagnosis is not always possible. However, consideration of CT scans together with the clinical features, cerebrospinal fluid cytology, tumor markers, and (if there is still doubt) response to a small dose of irradiation will generally allow a specific diagnosis with a high degree of probability. In this way, germinomas may be selected for radiotherapy and the tumors less likely to respond may be subjected to primary surgery.


2000 ◽  
Vol 93 (2) ◽  
pp. 245-253 ◽  
Author(s):  
Shizuo Oi ◽  
Masayoshi Shibata ◽  
Jiro Tominaga ◽  
Yumie Honda ◽  
Masaki Shinoda ◽  
...  

Object. This prospective study is based on a consecutive series of 20 patients with pineal region tumors who underwent minimally invasive preferential management. The purpose of this report is to discuss the role of neuroendoscopic procedures in the management of pineal region tumors.Methods. If the tumor markers α-fetoprotein and human chorionic gonadotropin were not detected in serum and there was significant ventricular dilation visualized on neuroimages, neuroendoscopic surgery was first applied for tumor debulking with tissue diagnosis and gross morphological analysis of the tumor and the intraventricular structures, followed by third ventriculostomy.Subsequent procedures were determined on the basis of verified individual tumors. For treatment of germinomas and pineoblastomas, if no tumor dissemination was confirmed by pre-, intra-, or postoperative findings, stereotactic radiotherapy or radiosurgery was performed after one course of chemotherapy with the ICE regimen (isofomid, cisplatin, and etoposide) and followed by two additional courses of chemotherapy. For treatment of malignant germ cell tumors, after extensive surgery, adjuvant chemotherapy with the ICE regimen was performed in three courses in all cases. Then radiotherapy was started using various methods, depending on the evidence of tumor dissemination. For treatment of teratomatous and neuroectodermal tumors other than pineoblastomas, extensive surgical removal was performed. As for adjuvant therapy, if the tumor was a low-grade glioma or if the patient was younger than 5 years of age, postoperative treatment did not include radiotherapy. If the tumor was a malignant teratoma or high-grade glioma, conventional focal radiotherapy was performed, followed by chemotherapy with ICE for 1 year.All but two treated patients had ventriculomegaly. Neuroendoscopic procedures were performed in six of 15 treated patients. Neuroendoscopic biopsy with tumor debulking offered enough material for tissue diagnosis, including immunohistochemical analysis and, in one case, revealed evidence of tumor dissemination undetectable on neuroimaging. With one exception, no shunt was required in any patient undergoing endoscopic third ventriculostomy. Stereotactic radiotherapy was performed in indicated cases. Favorable therapeutic outcomes were obtained in all cases of germinoma and pineoblastoma, with follow-up periods ranging from 24 months to 6.5 years.Conclusions. Our minimally invasive preferential regimen clarified the precise indication for neuroendoscopic procedures, and the majority of our patients with dilated ventricles and no evidence of tumor markers were treated satisfactorily with effective neuroendoscopic procedures as the initial procedure, avoiding unnecessary craniotomy and radiotherapy and promising excellent therapeutic outcomes. The treatment for malignant pineal region tumors remains a subject for further study.


1997 ◽  
Vol 87 (4) ◽  
pp. 525-534 ◽  
Author(s):  
Lawrence L. Wald ◽  
Sarah J. Nelson ◽  
Mark R. Day ◽  
Susan E. Noworolski ◽  
Roland G. Henry ◽  
...  

✓ The utility of three-dimensional (3-D) proton magnetic resonance spectroscopy (1H-MRS) imaging for detecting metabolic changes after brain tumor therapy was assessed in a serial study of 58 total examinations of 12 patients with glioblastoma multiforme (GBM) who received brachytherapy. Individual proton spectra from the 3-D array of spectra encompassing the lesion showed dramatic differences in spectral patterns indicative of radiation necrosis, recurrent or residual tumor, or normal brain. The 1H-MRS imaging data demonstrated significant differences between suspected residual or recurrent tumor and contrast-enhancing radiation-induced necrosis. Regions of abnormally high choline (Cho) levels, consistent with viable tumor, were detected beyond the regions of contrast enhancement for all 12 gliomas. Changes in the serial 1H-MRS imaging data were observed, reflecting an altered metabolism following treatment. These changes included the significant reduction in Cho levels after therapy, indicating the transformation of tumor to necrotic tissue. For patients who demonstrated subsequent clinical progression, an increase in Cho levels was observed in regions that previously appeared either normal or necrotic. Several patients showed regional variations in response to brachytherapy as evaluated by 1H-MRS imaging. This study demonstrates the potential of noninvasive 3-D 1H-MRS imaging to discriminate between the formation of contrast-enhancing radiation necrosis and residual or recurrent tumor following brachytherapy. This modality may also allow better definition of tumor extent prior to brachytherapy by detecting the presence of abnormal metabolite levels in nonenhancing regions of solid tumor.


1983 ◽  
Vol 59 (5) ◽  
pp. 875-878 ◽  
Author(s):  
Ian R. Whittle ◽  
John L. Allsop ◽  
Michael Besser

✓ Computerized tomography (CT) revealed obstructive hydrocephalus and a pineal mass in a 14-year-old girl who presented with headaches and a Parinaud's syndrome. Although there was no major evidence of extracranial tuberculosis, and cerebrospinal fluid obtained during third ventriculostomy contained no leukocytes, suboccipital transtentorial biopsy of the lesion revealed it to be a tuberculoma. Serial CT scans showed resolution of the lesion following subtotal resection and antituberculous therapy. The implications of this case with regard to difficulties in the diagnosis of cerebral tuberculoma and the management of pineal region tumors are discussed.


2000 ◽  
Vol 93 (2) ◽  
pp. 330-334 ◽  
Author(s):  
Dan X. Cai ◽  
Manuela Mafra ◽  
Robert E. Schmidt ◽  
Bernd W. Scheithauer ◽  
Tae Sung Park ◽  
...  

✓ The authors report on two patients with classic medulloblastoma, each of whom underwent extensive therapy-associated neuronal maturation. The first patient presented at 3 months of age with hydrocephalus caused by a 5-cm tumor in the cerebellar vermis. He underwent a gross-total resection of a desmoplastic medulloblastoma. No mature elements were identified. Despite adjuvant chemotherapy, a 1.5-cm recurrent tumor developed 6 months later. Sections from the subtotally resected tumor demonstrated exclusively mature neuronal elements, ranging from neurocytes to ganglion cells. Four months later, a second recurrent tumor was resected. The specimen collected this time demonstrated classic medulloblastoma morphological characteristics. The patient was subsequently treated with radiation therapy, which seemed to have an effect; however, the tumor eventually progressed and the patient died. The second patient presented at 3 years of age with a midline medulloblastoma and was treated with subtotal resection, radiation therapy, and chemotherapy. Although the tumor remained stable on radiographic imaging, a second resection was performed 8 years later to alleviate hydrocephalus. Histological examination revealed predominantly small mature neurons with scattered ganglion cells and extensive calcification. No adjuvant therapy was given and the patient is alive and well as of his last follow-up examination.The mature neuronal neoplasms resected in both patients demonstrated negligible proliferative indices and stained appropriately with neuronal immunohistochemical markers. The smaller neuronal population resembled those of a central neurocytoma and medullocytoma/cerebellar neurocytoma. Analogous to neuroblastoma, our cases suggest that adjuvant therapy can induce extensive or complete neuronal maturation in medulloblastoma. Additional cases must be studied to determine the prognostic significance of this rare phenomenon.


1991 ◽  
Vol 74 (3) ◽  
pp. 447-453 ◽  
Author(s):  
Douglas L. Arnold ◽  
Joseph F. Emrich ◽  
Eric A. Shoubridge ◽  
Jean-Guy Villemure ◽  
William Feindel

✓ Phosphorus magnetic resonance (MR) spectroscopy allows noninvasive measurement of phosphate-containing compounds and pH within brain cells. The authors obtained localized phosphorus MR spectra from 10 normal brains, four low-grade astrocytomas, six glioblastomas, four meningiomas, and three pituitary adenomas and found differences in the spectra of each tumor type. Compared to normal brain, the spectra from low-grade astrocytomas showed a significant reduction of the phosphodiester (PDE) peak. Glioblastomas were characterized by a significant reduction of the PDE peak, elevation of the phosphomonoester (PME) peak, and a relatively alkaline intracellular pH. The spectra from meningiomas and pituitary adenomas were markedly different from the glial tumors. Meningiomas showed significant reductions in phosphocreatine, PDE, and inorganic phosphate, as well as a relatively alkaline pH. Pituitary adenomas resembled meningiomas, but had a much higher PME peak. Although the number of tumors studied was small, there appears to be a characteristic spectrum associated with these different tumor types. The present findings can be useful in the preoperative identification of these tumors and in furthering understanding of their growth and metabolism in vivo.


1998 ◽  
Vol 88 (6) ◽  
pp. 1050-1057 ◽  
Author(s):  
Ibrahim M. Ziyal ◽  
Laligam N. Sekhar ◽  
Eduardo Salas ◽  
Wayne J. Olan

Object. The authors sought to confirm that the combined supra/infratentorial—transsinus approach offers a safer means of resecting large pineal region tumors than other approaches currently being used. The aforementioned method provides a wider exposure of the pineal region with less brain retraction than the infratentorial—supracerebellar or the occipital—transtentorial approach alone and is applicable to some large and giant tumors of this area. This combined approach was used in six patients to remove large pineal region tumors including four tentorial meningiomas, one pineocytoma, and one epidermoid cyst. Methods. The transverse sinus and tentorium were sectioned after review of preoperative angiographic studies, after taking intraoperative measurements of the venous pressure in the nondominant transverse sinus before and after clipping and while monitoring the somatosensory evoked potentials. The occipital lobe cortex and cerebellum were retracted slightly along the tentorium. Deep veins of the galenic system, the quadrigeminal area, and the tumor were well exposed. Before it was used for tumor resection the approach was studied in five cadaveric head specimens, and the projection of different approaches was compared radiologically. The tumors were removed in a gross-total manner in all patients, and none of the major veins of the galenic system was injured. Resuturing of the nondominant transverse sinus was performed postoperatively in one patient. One of the six patients experienced transient visual loss, and another suffered mild right sixth cranial nerve paresis; however, both recovered in 3 weeks. The wide exposure of the combined approach was also confirmed on radiological and anatomical studies. Conclusions. The combined supra/infratentorial—transsinus approach is preferred for the resection of certain large pineal region tumors.


1982 ◽  
Vol 57 (2) ◽  
pp. 164-167 ◽  
Author(s):  
Yutaka Tsutsumi ◽  
Yukihiko Andoh ◽  
Norio Inoue

✓ An easy, accurate, and safe biopsy technique for deep-seated brain tumors using a newly devised real-time ultrasonic apparatus is presented. Three lesions, one large pituitary adenoma and two pineal region tumors, were biopsied successfully by this technique.


1992 ◽  
Vol 77 (1) ◽  
pp. 139-142 ◽  
Author(s):  
Juji Takeuchi ◽  
Kiyoshi Kikuchi ◽  
Yuta Shibamoto ◽  
Ichiro Fujisawa

✓ A case of juvenile pilocytic astrocytoma of the pituitary stalk is reported. The patient presented with diabetes insipidus and growth retardation. Magnetic resonance (MR) imaging was useful in diagnosing the lesion. Although the tumor could not be completely removed by surgery, radiotherapy was effective and the residual tumor disappeared 6 months after irradiation. The patient's condition has remained stable and no recurrence has been observed by MR imaging in the 4 years since radiation therapy.


2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 37-41 ◽  
Author(s):  
William F. Regine ◽  
Roy A. Patchell ◽  
James M. Strottmann ◽  
Ali Meigooni ◽  
Michael Sanders ◽  
...  

Object. This investigation was performed to determine the tolerance and toxicities of split-course fractionated gamma knife radiosurgery (FSRS) given in combination with conventional external-beam radiation therapy (CEBRT). Methods. Eighteen patients with previously unirradiated, gliomas treated between March 1995 and January 2000 form the substrate of this report. These included 11 patients with malignant gliomas, six with low-grade gliomas, and one with a recurrent glioma. They were stratified into three groups according to tumor volume (TV). Fifteen were treated using the initial FSRS dose schedule and form the subject of this report. Group A (four patients), had TV of 5 cm3 or less (7 Gy twice pre- and twice post-CEBRT); Group B (six patients), TV greater than 5 cm3 but less than or equal to 15 cm3 (7 Gy twice pre-CEBRT and once post-CEBRT); and Group C (five patients), TV greater than 15 cm3 but less than or equal to 30 cm3 (7 Gy once pre- and once post-CEBRT). All patients received CEBRT to 59.4 Gy in 1.8-Gy fractions. Dose escalation was planned, provided the level of toxicity was acceptable. All patients were able to complete CEBRT without interruption or experiencing disease progression. Unacceptable toxicity was observed in two Grade 4/Group B patients and two Grade 4/Group C patients. Eight patients required reoperation. In three (38%) there was necrosis without evidence of tumor. Neuroimaging studies were available for evaluation in 14 patients. Two had a partial (≥ 50%) reduction in volume and nine had a minor (> 20%) reduction in size. The median follow-up period was 15 months (range 9–60 months). Six patients remained alive for 3 to 60 months. Conclusions. The imaging responses and the ability of these patients with intracranial gliomas to complete therapy without interruption or experiencing disease progression is encouraging. Excessive toxicity derived from combined FSRS and CEBRT treatment, as evaluated thus far in this study, was seen in patients with Group B and C lesions at the 7-Gy dose level. Evaluation of this novel treatment strategy with dose modification is ongoing.


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