Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of malignant meningioma metastasizing through the cerebrospinal pathway is presented. The primary tumor was a parasagittal malignant meningioma invading into the brain. The tumor seeded to the cerebellopontine angle cistern and thoracic spine after multiple operations. Although this type of tumor borders the CSF, metastasis through the cerebrospinal pathway is rare, and only 18 such cases have been reported (2, 3, 10, 12).
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PMID:Malignant meningioma metastasizing through the cerebrospinal pathway. 162 2

Paraneoplastic syndromes affecting the nervous system are rare and their diagnosis is often difficult when the original cancer is unknown. Recently, high levels of antineuronal antibodies (AB) have been found in serum and CSF of some patients with paraneoplastic syndromes. The anti-Yo AB recognizes 2 proteins of 34 and 62 kd in the cytoplasm of Purkinje cells and in malignant cells of patients suffering from paraneoplastic cerebellar degeneration associated with ovarian and breast cancer. The anti-Hu AB recognizes a 37-40 kd protein in nuclei of neurons and in tumor cells of patients suffering from subacute sensory neuronopathy and encephalomyelitis associated with small cell lung cancer. Other antineuronal AB have been more rarely identified. The presence of high titer of one of these AB in a patient with suspected paraneoplastic syndrome is of great practical interest since it confirms the neurological diagnosis and strongly suggests the location of the primary tumor when the malignancy is unknown. The pathogenetic role of the antineuronal AB is unknown but it is likely that some paraneoplastic syndromes affecting the nervous system are due to an immune reaction against antigens shared by the tumor and the nervous system. To date, no efficient treatment has been found.
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PMID:[Autoimmunity and paraneoplastic neurologic syndromes]. 196 63

Studies of twenty-five patients with loculated leptomeningeal tumor metastases diagnosed by CT and/or MR were analyzed retrospectively. Medulloblastoma was the most frequent primary tumor (8/25, 32%). Four subgroups of loculated patterns were identified. Type A included mass(es) limited to the subarachnoid space without obvious direct parenchymal infiltration; this pattern occurred in 12 patients, of whom five had associated diffuse pattern. Type B was characterized by mass(es) still predominantly in the subarachnoid space but with minor transpinal parenchymal infiltration; this pattern was found in five patients. Type C comprised subarachnoid mass(es) with marked transpinal extension mimicking parenchymal lesion; this pattern was observed in three patients. Type D consisted of subarachnoid mass(es) growing along the perineural CSF space; this pattern was noted in two patients. Additionally, two patients presented with combined A and C patterns, and one patient had a combined B and C pattern. More than half the patients (14/25, 56%) presented with a single lesion. The most frequent locations were the suprasellar cistern, ventricular walls, and lateral recesses of the fourth ventricle, Gd-DTPA-enhanced T1-weighted MR images appeared best for demonstrating the site and extent of disease. Recognition of the loculated patterns of leptomeningeal metastases, which are less common than the diffuse pattern, is important to radiologists and clinicians for correct diagnosis and proper management of patients with this disease.
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PMID:Loculated intracranial leptomeningeal metastases: CT and MR characteristics. 210 30

Although radiation therapy has been universally accepted as the treatment of choice for primary intracranial germinomas, the optimal treatment technique continues to be a matter of controversy. Some authors advocate routine prophylactic craniospinal irradiation for all patients with localized intracranial germinomas whereas others have recommended partial brain fields. A retrospective analysis was performed on all 30 patients with tumors of the pineal and suprasellar regions irradiated at our institution between 1967 and 1987. Definitive histology was obtained in 23 patients. There were 13 germinomas and 10 non-germinomatous germ cell tumors. The 13 patients with biopsy proven germinomas constitute one of the largest modern series and will form the basis of this report. In all 13 patients, the tumor was confined to a single site within either the pineal region (9 patients) or the suprasellar region (4 patients). One patient with a pineal germinoma had CSF cytology positive for malignant cells and this patient was treated with craniospinal irradiation. Twelve patients were treated using partial brain fields encompassing either the tumor plus margin (10 patients) or the entire ventricular system followed by a boost to the primary tumor (2 patients). With the exception of one patient, all patients received a total dose between 4900 and 5500 cGy to the primary (median 5040 cGy). The patient receiving less (3960 cGy) suffered from both local and spine relapse 26 months after diagnosis. The remaining 12 patients were free of disease at 81 months median follow-up time. We suggest that for patients with biopsy proven pineal or suprasellar germinomas, irradiation of partial brain is sufficient and craniospinal irradiation should be administered only to those patients with disease involving more than one intracranial site, demonstrated meningeal seeding, or positive CSF cytology.
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PMID:Radiation therapy for intracranial germinoma: the case for limited volume treatment. 239 20

Studies of twenty-five patients with loculated leptomeningeal tumor metastases diagnosed by CT and/or MR were analyzed retrospectively. Medulloblastoma was the most frequent primary tumor (8/25, 32%). Four subgroups of loculated patterns were identified. Type A included mass(es) limited to the subarachnoid space without obvious direct parenchymal infiltration; this pattern occurred in 12 patients, of whom five had associated diffuse pattern. Type B was characterized by mass(es) still predominantly in the subarachnoid space but with minor transpinal parenchymal infiltration; this pattern was found in five patients. Type C comprised subarachnoid mass(es) with marked transpinal extension mimicking parenchymal lesion; this pattern was observed in three patients. Type D consisted of subarachnoid mass(es) growing along the perineural CSF space; this pattern was noted in two patients. Additionally, two patients presented with combined A and C patterns, and one patient had a combined B and C pattern. More than half the patients (14/25, 56%) presented with a single lesion. The most frequent locations were the suprasellar cistern, ventricular walls, and lateral recesses of the fourth ventricle, Gd-DTPA-enhanced T1-weighted MR images appeared best for demonstrating the site and extent of disease. Recognition of the loculated patterns of leptomeningeal metastases, which are less common than the diffuse pattern, is important to radiologists and clinicians for correct diagnosis and proper management of patients with this disease.
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PMID:Loculated intracranial leptomeningeal metastases: CT and MR characteristics. 251 78

Several groups of investigators have confirmed the occurrence of antibodies to Purkinje and other cerebellar neuronal populations in the serum and spinal fluid of patients with paraneoplastic cerebellar degeneration. Although this antibody response suggests that paraneoplastic cerebellar degeneration may have an autoimmune basis, it is not known what role anticerebellar antibodies play in the pathogenesis of this disorder or whether the presence of antibodies invariably results in cerebellar injury. We identified 3 patients with ovarian malignancies in whom high titers of circulating anticerebellar antibodies were present without clinical evidence of cerebellar disease. We followed these patients clinically and serologically until their deaths from their neoplasms. All 3 patients remained neurologically normal. In 2 of the patients, anticerebellar antibodies persisted at high titer. CSF obtained from 1 of these patients postmortem did not contain detectable levels of anticerebellar antibody, but histopathologic examination of her cerebellum revealed patchy loss of Purkinje cells. In the 3rd patient, antibody titers fell with removal of the primary tumor and chemotherapy but did not rise with tumor recurrence. Indirect immunofluorescence did not reveal anticerebellar antibodies in the serum or CSF of other patients with neoplasms, patients with other cerebellar disease, or normal controls. The present study demonstrates that patients with ovarian malignancies may occasionally develop antibodies that react with cerebellar neuronal antigens and can maintain this antibody response for protracted periods of time without clinically evident cerebellar injury. Tumor recurrence may not be accompanied by rise in titers of anticerebellar antibodies.
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PMID:Anticerebellar antibodies in neurologically normal patients with ovarian neoplasms. 268 57

Seven patients with supratentorial gliomas developed leptomeningeal gliomatosis (LMG) without symptomatic recurrence at the primary tumor site. In all, severe back and radicular pain, often simulating disc disease, preceded the development of spinal cord or cauda equina dysfunction. In 4 instances, intracranial hypertension due to hydrocephalus developed prior to spinal involvement. Cytological examination of the CSF revealed malignant cells in only 2/7 but a myelogram was diagnostic in all 7. All patients received spinal irradiation (RT) and 5 received chemotherapy. Two patients with low-grade gliomas improved transiently; 5 with malignant gliomas responded poorly, became paraplegic over 4 months and eventually died of LMG. When fatal LMG occurs in young adults suffering from supratentorial glioma, the primary tumor is often quiescent. Hydrocephalus is often the first manifestation of LMG and, when it is detected, a myelogram and CSF cytology study should be performed in the hope that diagnosis and treatment of spinal cord lesion at a very early stage will prove beneficial. Irradiation of the entire spinal canal is probably required as there is a high risk of rapid development of new lesions in non irradiated segments of the spinal canal.
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PMID:Leptomeningeal gliomatosis with spinal cord or cauda equina compression: a complication of supratentorial gliomas in adults. 271 19

Between May 1974 and March 1983, 44 children with histologically verified cerebellar medulloblastoma were seen for post-operative cranial-spinal irradiation following attempted total tumor removal. Six patients were excluded from review because they received all or part of their treatment at another institution (3 patients) or did not complete the planned course of irradiation (3 patients). All of the 38 remaining patients were treated by a previously described technique on a 4 MeV Linear Accelerator with 55 Gy delivered to the primary tumor site. Prior to December 1978, 19 consecutive children (Group A) had spinal prophylactic doses of 30-40 Gy and brain prophylactic doses of 40-50 Gy. After the date, 25 Gy was given to the cranial-spinal axis of 19 consecutive children (Group B). This lower dose was arbitrarily selected with the hope of reducing morbidity in treated survivors and achieving the same tumor control. Risk factors that define good and poor prognosis were evaluated for each group, and there were no differences noted. Myelography and CSF cytology were not routinely performed. Follow-up for the 38 patients ranges from 20 months to 124 months. For the low risk patients, survival (12/15 or 80%) was independent of cranial-spinal radiation dose (Group A 6/8, Group B 6/7). For the high risk patients survival was poor (9/23 or 39%), not dependent on cranial-spinal radiation dose (Group A 5/11, Group B 4/12), and associated with failure at the primary site (10/14), often with CSF seeding (8/10). The other 4 failures include 2 who had moved outside the United States (details of failure are unknown), 1 with supratentorial, CSF seeding and distant metastases, and 1 with distant metastasis only. There were no isolated spinal failures. This pilot study shows that the prophylactic radiation dose to the cranial-spinal axis can be decreased to 25 Gy without jeopardizing control rate and survival in patients with medulloblastoma.
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PMID:Long-term results of a pilot study of low dose cranial-spinal irradiation for cerebellar medulloblastoma. 366 70

We studied a 4-year-old boy with symptoms and signs of a posterior fossa tumor. CT showed two separate intracranial tumors: a fourth ventricle choroid plexus papilloma and a frontal subependymal giant-cell astrocytoma. This case emphasizes that, even in the absence of special genetic predisposition to CNS tumors, two separate intracranial masses may not represent CSF metastasis of a single primary tumor.
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PMID:Two primary brain tumors in one child. 394 85

A case of primary diffuse leptomeningeal melanoblastosis in a 46-year-old male is reported. His symptoms included headaches, transient hemiparesis, epileptic seizures and a progressive psychosyndrome. CT brain scans showed a slight enhancement of density in the subarachnoidal space. The disease was diagnosed by CSF cytology, using light microscopy, electron microscopy, autoradiography and cell culture. Systemic combined chemotherapy using Cisplatinum, DTIC, and Vindesine was without any significant response and he died 18 weeks after onset of the first complaints. Autopsy showed a diffuse infiltration of the entire leptomeninges by melanotic melanoblastoma cells invading the sagittal superior sinus. A thorough dissection including the orbital contents and skin nevi failed to reveal a primary tumor outside the CNS.
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PMID:Review and case report: primary melanoblastosis of the leptomeninges. 399 6


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