Diagnosis and management of pineal tumors

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.

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.


2005 ◽  
Vol 102 ◽  
pp. 175-179 ◽  
Author(s):  
Beatriz E. Amendola ◽  
Aizik Wolf ◽  
Sammie R. Coy ◽  
Marco A. Amendola ◽  
Daryl Eber

Object. The authors evaluate their results when using gamma knife surgery (GKS) in the management of patients with tumors in the pineal region. Methods. This is a retrospective clinical evaluation of 20 patients with primary tumors of the pineal region treated with GKS from November 1994 through August 2003. There were 13 germ cell tumors, two pineoblastomas, two low-grade gliomas, one primitive neuroectodermal tumor, one teratoma, and one pineocytoma. There were 10 male and 10 female patients. Their median age was 15.5 years (range 5–71 years). The median margin dose was 11 Gy (range 8–20 Gy). The median target volume was 3.1 cm3 (range 0.1–49.9 cm3). Five patients received sequential systemic chemotherapy and four underwent adjuvant conventional radiation therapy. Seventeen (85%) of 20 patients are alive with a median survival of 30.4 months (range 0–-85.7 months). Two patients required retreatment. Three patients died: one of unrelated causes, one who presented with extensive local disease, and the other of meningeal carcinomatosis with local control of the primary tumor. No complications from GKS were noted. Conclusions. This initial experience suggests that GKS is a valuable treatment modality for the management of pineal region tumors. This technique offers excellent local tumor control and minimal patient morbidity, allowing for immediate use of systemic chemotherapy and/or conventional radiation if indicated.


2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 175-179 ◽  
Author(s):  
Beatriz E. Amendola ◽  
Aizik Wolf ◽  
Sammie R. Coy ◽  
Marco A. Amendola ◽  
Daryl Eber

Object. The authors evaluate their results when using gamma knife surgery (GKS) in the management of patients with tumors in the pineal region. Methods. This is a retrospective clinical evaluation of 20 patients with primary tumors of the pineal region treated with GKS from November 1994 through August 2003. There were 13 germ cell tumors, two pineoblastomas, two low-grade gliomas, one primitive neuroectodermal tumor, one teratoma, and one pineocytoma. There were 10 male and 10 female patients. Their median age was 15.5 years (range 5–71 years). The median margin dose was 11 Gy (range 8–20 Gy). The median target volume was 3.1 cm3 (range 0.1–49.9 cm3). Five patients received sequential systemic chemotherapy and four underwent adjuvant conventional radiation therapy. Seventeen (85%) of 20 patients are alive with a median survival of 30.4 months (range 0–-85.7 months). Two patients required retreatment. Three patients died: one of unrelated causes, one who presented with extensive local disease, and the other of meningeal carcinomatosis with local control of the primary tumor. No complications from GKS were noted. Conclusions. This initial experience suggests that GKS is a valuable treatment modality for the management of pineal region tumors. This technique offers excellent local tumor control and minimal patient morbidity, allowing for immediate use of systemic chemotherapy and/or conventional radiation if indicated.


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.


1971 ◽  
Vol 35 (2) ◽  
pp. 197-202 ◽  
Author(s):  
Bennett M. Stein

✓ In six patients with pineal tumors, a posterior fossa approach was used to explore the pineal region. This approach avoided the deep venous system and led to no mortality and minimal morbidity. Because a certain percentage of pineal lesions are benign and amenable to resection, it is recommended that all pineal tumors be explored prior to a decision regarding radiotherapy.


1991 ◽  
Vol 74 (4) ◽  
pp. 545-551 ◽  
Author(s):  
Harold J. Hoffman ◽  
Hiroshi Otsubo ◽  
E. Bruce Hendrick ◽  
Robin P. Humphreys ◽  
James M. Drake ◽  
...  

✓ All patients with confirmed intracranial germ-cell tumors treated at the Hospital of Sick Children during the period January, 1952, to December, 1989, were reviewed. Of the 51 tumors reviewed, 16 were located in the suprasellar region, 32 in the pineal region, and three in both the pineal and the suprasellar regions. Forty-nine patients underwent surgical resection which was total in seven and partial in 20, and consisted of a biopsy in 22. Two patients were managed on the basis of serum and cerebrospinal fluid markers. Surgical tools such as the operating microscope, the ultrasonic surgical aspirator, and the laser beam allowed safe debulking and removal of the deep-seated tumors in the pineal region. There were no operative deaths in the 36 patients treated since 1972, who included 23 with pineal tumors. Twenty-five patients with germinomas received radiotherapy and had a 5-year survival rate of 85.1%. Thirteen patients with non-germinoma germ-cell tumors received radiotherapy and had a 5-year survival rate of 45.5%. On the basis of this review, the authors recommend resection of pineal and suprasellar germ-cell tumors in order to firmly establish an accurate histological diagnosis to guide the extent of adjuvant therapy. In the case of a pure germinoma without evidence of dissemination, adjuvant therapy consists only of local radiotherapy. On the other hand, for malignant non-germinoma germ-cell tumors, adjuvant therapy must include chemotherapy as well as craniospinal axis radiotherapy.


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.


1979 ◽  
Vol 51 (5) ◽  
pp. 597-607 ◽  
Author(s):  
Edward A. Neuwelt ◽  
Mark Glasberg ◽  
Eugene Frenkel ◽  
W. Kemp Clark

✓ Eight patients with primary malignant pineal tumors have been seen at this institution over the past 6 years; six of them underwent definitive surgical exploration. Complete gross microsurgical excision of well encapsulated tumors was possible in four of these patients. In two cases of pineal germinomas, a biopsy and a subtotal resection were carried out because of the known radiosensitivity of this tumor. These six surgical patients all received postoperative craniospinal radiation and continue to do well up to 6 years postoperatively. Two nonoperative patients were initially treated at other institutions by ventriculoperitoneal shunt and radiation and were the only ones to develop metastatic disease. One patient had metastasis of her pineoblastoma to her unirradiated spinal canal and the other patient had metastasis of his germinoma to the peritoneum. The former patient was quadriplegic on admission, although her pineal tumor was no longer visible on computerized tomography (CT), and she died of pneumonia. The latter patient's tumor secreted the beta chain of human chorionic gonadotropin (HCG). This patient's massive metastatic tumor burden completely regressed as determined by body CT scan and HCG levels after four courses of chemotherapy with bleomycin, vinblastine, and cis-platinum. In 20 patients with lesions of the pineal region, craniotomy was associated with only one death (a patient with metastatic adenocarcinoma). Thus, microsurgery for pineal tumors provides either a reasonably safe potential for complete tumor extirpation and possible cure, or a tissue diagnosis which is necessary for appropriate therapeutic planning for radiotherapy and/or chemotherapy. The traditional therapeutic approach of empiric radiotherapy without a tissue diagnosis for pineal lesions may no longer be warranted.


1989 ◽  
Vol 71 (4) ◽  
pp. 601-604 ◽  
Author(s):  
Jan Watterson ◽  
John R. Priest

✓ Primary intracranial germ-cell tumors are infrequently occurring neoplasms which most often arise in the pineal or sellar regions. Germinomas are seen more frequently than nongerminomatous germ-cell tumors; they are often curable with radiotherapeutic approaches, or with chemotherapy in the rare instance of extraneural metastasis. Nongerminomatous germ-cell tumors are relatively radioresistant and when extraneural metastasis has occurred, they have been fatal in all of the 32 previously reported cases. The case of a 14-year-old girl with a mixed malignant germ-cell tumor arising in the pineal region is reported. Extraneural metastasis to the lung developed 12 months after whole-brain radiotherapy was completed. She was treated with etoposide (VP-16), high-dose cisplatin, vinblastine, and bleomycin and is currently without evidence of disease 46 months postmetastasis.


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.


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