Midline pineal tumors and suprasellar germinomas: highly curable by irradiation.
TL;DR: Sixty-one patients with midline pineal tumors and 16 patients with suprasellar germinomas were treated with surgical decompression and relatively high-dose radiotherapy of the primary site, and there were no long-term complications.
Abstract: Sixty-one patients with midline pineal tumors and 16 patients with suprasellar germinomas were treated with surgical decompression and relatively high-dose radiotherapy of the primary site Results were excellent, and there were no long-term complications Ten per cent of midline pineal tumors and 37% of suprasellar germinomas metastasized to the cerebral or spinal subarachnoid space within 6 months to 5 years Irradiation of the entire neural axis is recommended for locally extensive tumors, simultaneous pineal and hypothalamic lesions, and all biopsy-proved germinomas The five-year survival rate was 79% for midline pineal tumors and 77% for suprasellar germinomas
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TL;DR: The pathogenesis of GCT's may be revealed by their specificity of origin within the positive (suprasellar cistern-suprachiasmatic nucleus) and negative (pineal) regulatory centers for gonadotropin secretion within the diencephalon.
Abstract: ✓ The natural history of primary intracranial germ-cell tumors (GCT's) is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7% endodermal sinus tumors, and 5% choriocarcinomas. Intracranial GCT's display specificity in site of origin. Ninety-five percent arise along the midline from the suprasellar cistern (37%) to the pineal gland (48%), and an additional 6% involve both sites. The majority of germinomas (57%) arise in the suprasellar cistern, while most nongerminomatous GCT's (68%) preferentially involve the pineal gland (p < 0.0001). The age distribution of afflicted patients is unimodal, centering with an abrupt surge in frequency in the early pubertal years; 68% of patients are diagnosed between 10 and 21 years of age. Nongerminomatous GCT's demonstrate an earlier age of onset than do germinomas (p < 0.0001). Prolonged symptomatic intervals prior to diagnosis are common in germinomas (p = 0.0007), in suprasellar GCT's (p = 0.001), and a...
732 citations
TL;DR: While the available data suggest a favorable influence and outcome, randomized studies are needed to further optimize radiation therapy techniques and to integrate new therapeutic modalities.
Abstract: The effectiveness and complications of radiation therapy for brain neoplasms are reviewed. While the available data suggest a favorable influence and outcome, randomized studies are needed to further optimize radiation therapy techniques and to integrate new therapeutic modalities.
361 citations
TL;DR: Germinomas have a good prognosis, as over 90% of patients can be effectively treated with radiation therapy, and recent reports suggest that the dose and volume of radiation therapy required for disease control can be lessened with the addition of adjuvant chemotherapy.
Abstract: Intracranial germ cell tumors are a heterogeneous group of lesions which occur in children and adults. Within the classification of intracranial germ cell tumors, there are a variety of different tumor types which carry different prognoses. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor markers, including α-fetoprotein and β-human chorionic gonadotropin, which may obviate the need for surgical intervention. The management of intracranial germ cell tumors in both children and adults remains unsettled. Germinomas have a good prognosis, as over 90% of patients can be effectively treated with radiation therapy. The dose and volume of radiation therapy needed for disease control is not well established, and controversy exists concerning the need for whole brain or craniospinal radiation therapy for localized tumors. Germinomas are also chemosensitive and recent reports suggest that the dose and volume of radiation therapy required for disease control can be lessened with the addition of adjuvant chemotherapy. The outcome for patients with nongerminomatous germ cell tumors is less favorable. Radiation therapy alone will result in disease control in 40%-60% of patients. The addition of chemotherapy to radiation therapy may improve the rate of survival. The Oncologist 2000;5:312-320
345 citations
TL;DR: Radiotherapy directed toward the craniospinal axis or tumor site alone at decreased dose levels is effective and further attempts to decrease total doses are justified to reduce the risk of late side effects.
Abstract: PURPOSE: A multicenter prospective trial was conducted (Maligue Keimzelltumoren [MAKEI] 83/86/89) to assess outcome in intracranial germinoma after treatment with radiotherapy alone at reduced doses. PATIENTS AND METHODS: Between 1983 and 1993, 60 patients with histologically (n = 58) or cytologically (n = 2) confirmed germinoma were enrolled onto the study. Patients received radiotherapy alone (craniospinal axis/local boost). In the MAKEI 83/86 study (involving 11 patients), the dose to the craniospinal axis was 36 Gy and the dose to the tumor region was 14 Gy. In the MAKEI 89 study (involving 49 patients), doses were 30 and 15 Gy, respectively. RESULTS: Median patient age was 13 years (range, 6 to 31 years). Complete remission was achieved in all patients. The estimated (Kaplan-Meier) 5-year relapse-free survival rate was 91.0% ± 3.9% at a mean follow-up of 59.5 months (range, 3 to 180 months); the estimated overall survival rate was 93.7% ± 3.6%. Relapse occurred in five patients 10 to 33 months (mean,...
234 citations
TL;DR: Computerized tomographic scanning is revealing more clearly the response and resistance of certain intracranial tumors to radiotherapy and to chemotherapy, and attempts to overcome the remarkable resistance to treatment of these tumors are described.
Abstract: Computerized tomographic (CT) scanning is revealing more clearly the response and resistance of certain intracranial tumors to radiotherapy and to chemotherapy. Pure germinomas in the pineal and suprasellar regions are very radiosensitive and highly curable and, following whole central nervous system (CNS) irradiation, a 5-year survival rate in excess of 80 % may be reached. Chemical markers may reveal the presence, persistence or recurrence of more resistant teratoid tissue in germ cell tumors. Medulloblastomas are also very radiosensitive, but the presence of local residual resistant tumor has restricted cure rates to approximately 40% following post-operative megavoltage irradiation. Adjuvant chemotherapy may increase the survival rate of children with these tumors to 60% or 70%: children surviving a period of time equivalent to their age at diagnosis plus 9 months are likely to be cured. Ependymomas are moderately radiosensitive tumors and following high dose and, in certain circumstances, whole CNS irradiation, the 5-year survival rate is approximately 50 % : at present, adjuvant chemotherapy for patients with high grade ependymomas appears to increase length of survival but not cure rate. Low grade astrocytomas of the cerebral hemispheres, widely regarded as radioresistant, do respond to irradiation and also to chemotherapy. Optic gliomas should not be regarded as self-limiting malformations: they destroy vision, and lesions in the chiasma may prove fatal. They are not entirely radio-resistant and good responses may follow radiotherapy. High grade astrocytomas (glioblastoma) remain resistant and virtually incurable. While there is a chance with post-operative irradiation of extending survival for up to 5 years in grade III patients, there is no such hope for patients with grade IV lesions. Attempts to overcome the remarkable resistance to treatment of these tumors are described. Changes in dose/time/volume factors in radiotherapy, and the use of hyperbaric oxygen, chemical radio-sensitizers, adjuvant chemotherapy, immunotherapy and particle beam therapy have all, so far, failed to significantly improve overall prognosis. However, it has been shown that the tumor can be destroyed by neutron beam irradiation, but at the expense of normal brain damage and with no improvement in survival. Inoperable deep gliomas arising in adjacent areas of the brain can vary widely in their response to irradiation. For example, in children the 5-year survival rate for intrinsic hypothalamic tumors is 83%, compared with only 20% for thalamic tumors. Inoperable tumors of the brain-stem present a special challenge to the radiotherapist since they are generally limited in size, show a good initial response to irradiation, but recur and kill within 1–2 years. A trial of radiosensitizers such as misonidazole would seem indicated in such cases. Craniopharyngiomas and meningiomas are benign lesions and are expected to be radioresistant. They do, in fact, respond to irradiation. Following conservative surgery the operative mortality and morbidity for craniopharyngioma is greatly reduced and with radiotherapy the post-operative recurrence rate is substantially decreased: a 5-year survival rate of 90 % may be reached. The response of meningiomas to radiotherapy is often difficult to assess, but worthwhile regression may be seen in patients with large inoperable or recurrent tumors. Apart from tumor response to treatment, consideration must also be given to reaction of normal tissue to irradiation, to chemotherapy and to a combination of these modalities: these may produce late effects leading to disturbance of neurological and endocrine function and of intellectual and emotional status. Factors in the host, particularly age and certain pre-existing endocrine disturbances, may influence tissue reactions. Some intracranial gliomas appear to respond better to treatment in children than in adults, and survival rates for patients under 16 have become substantial. Of a total of 415 patients under the age of 16 completing radiotherapy for all types of intracranial tumor at the Royal Marsden Hospital, 53 % survived 5 years, 45 % 10 years, and of 41 % 20 years. It is particularly in very young patients that serious late effects are more likely to occur. Continued clinical efforts will undoubtedly increase survival rates further in those tumor groups which already show a substantial response to current treatments. It will, however, probably require considerable further progress in radio- and chemico-biological research, as well as some bold clinical experiments, before we can effectively overcome the resistance of glioblastoma without exceeding therapeutic tolerance of the normal brain. Cell culture and xenograft laboratory models for pre-clinical studies are being used to assess glioma responsiveness to new cytotoxic and radiosensitizing agents and to combination treatments.
222 citations
References
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TL;DR: Although medulloblastoma has long been known as the most sensitive primary brain tumor to ionizing radiation, the radiotherapy results for this tumor with respect to long-term survival is still very disappointing.
Abstract: CEREBELLAR MEDULLOBLASTOMA is one of the most common types of primary intracranial tumors in children. The treatment of this tumor, unfortunately, is considered as “one of the darkest chapters in pediatric neurosurgery” (1). Cushing (2) reported an average survival of five and six-tenths months in 14 patients with medulloblastoma who received no postoperative radiotherapy. Ingraham and Matson (3) stated that “to our knowledge, no patient with cerebellar medulloblastoma has ever been cured.” Although medulloblastoma has long been known as the most sensitive primary brain tumor to ionizing radiation, the radiotherapy results for this tumor with respect to long-term survival is still very disappointing. In order to better evaluate the response of medulloblastoma to irradiation and to improve the survival rate, the operative records of 100 consecutive cases of tissue-proved medulloblastoma at the Neurological Institute, Columbia-Presbyterian Medical Center were arbitrarily taken in a period from 1940 to 1967 ...
650 citations
146 citations
TL;DR: Fifty-three cases of pineal and posterior-third ventricular tumors seen at the Massachusetts General Hospital from 1920 to 1972 are reviewed and a plan of management is proposed based on a correlation between the clinical and pathological features and the response of each type of tumor to the currently available modes of therapy.
Abstract: ✓ Fifty-three cases of pineal and posterior-third ventricular tumors seen at the Massachusetts General Hospital from 1920 to 1972 are reviewed. This series includes 35 pathologically verified tumors and 18 cases accepted as pineal neoplasms on clinical and radiographic grounds. A plan of management is proposed based on a correlation between the clinical and pathological features and the response of each type of tumor to the currently available modes of therapy.
142 citations
Journal Article•
TL;DR: The present report proposes to extend Dorothy Russell's interpretations in accord with the teratologic concepts gained through study of nearly 1,000 tumors of the testis at the Army Institute of Pathology and to propose the designation "germinonla," which has been suggested for the tumor which occurs in the gonads.
Abstract: In 1944 Dorothy Russell (15) published the resuits of a study of pineal tumors. She presented a rational explanation for the well known similarity in histologic appearance of \"pinealomas\" and \"seminomas.\" She suggested that in'any \"pincalomas\" ucre in truth teratoid tumors. The present report proposes to confirln h er.~obscrvations and to extend her interpretations in accord with the teratologic concepts gained through study of nearly 1,000 tumors of the testis at the Army Institute of Pathology (6) . The files of the Institute contain pathologic material from 23 patients with tumors of the pineal or ectopic \"pinealomas.\" Fifteen tumors were submitted by military installations ~ (Group 1), and 8 were obtained from civilian sources e (Group 2). The essential data in all 23 cases arc listed in Table I. Seven of the 15 tumors in group 1 were identical with the tcsticular tumor termed \"spheroidal cell carcinoma,\" or \"atypical teratoma,\" by Russell (1 ~) and called \"~scminoma\" by many pathologists; this neoplasm is identical with the disgerminolna of the ovary. Because this type of new growth is probably made up of germ cells, possibly even of primordial germ cells, the designation \"germinonla,\" which has been suggested (6) for the tumor which occurs in the gonads, is equally appropriate for that which appears in the pineal. Two neoplasms in group 1 wcrc teratomatous tumors; 1 was an adult teratoma, and the other was a teratocarcinoma which also had germinomatous areas. Although there were 6 neural tumors in the group, only 2 prcscnted the biphasic cellular mosaic of the classic pinealoma. Of the 8 neoplaslnas in group 2, 3 were germinomas and 3 wcrc tcratocarcinomas. One of the lattcr also contained both chorioepitheliomatous and
139 citations