Cases reported "Brain Neoplasms"

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1/1162. endodermal sinus tumor of the pineal region: case report.

    This is a case report of an endodermal sinus tumor occurring in the pineal region of a 12-year-old Caucasian boy. Germ cell tumors of this type demonstrate a selective overgrowth of yolk sac endoderm associated with extraembryonic mesoblast and arise perferentially in the gonads of young children. This is the ninth known report of this tumor in the world literature. Its occurrence in the posterior third ventricle again confirms that pineal region germ cell tumors are histologically analogous to germ cell tumors originating in the gonads. A disturbance in the region of the primitive streak is thought to account for the exclusive midline location of this tumor in extragonadal sites. This patient was treated with a combination of vincristine, actinomycin D, and cyclophosphamide, and concomitant cobalt irradiation. For endodermal sinus tumors in the pineal region, this combination appears more effective than previously reported treatment by irradiation alone.
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2/1162. Increased risk of erythema multiforme major with combination anticonvulsant and radiation therapies.

    erythema multiforme major (EMM; stevens-johnson syndrome) is a cutaneous disorder associated with a wide variety of factors including ingestion of drugs such as phenytoin and exposure to intracranial radiation therapy. Based on observations of a 47-year-old black man with brain metastases who developed EMM after combined phenytoin and radiation therapy, we conducted a medline literature search for articles on similar cases from 1966 to the present. Twenty cases were identified that support the hypothesis that EMM is associated with combined phenytoin and radiation therapy. The reaction, or its severity, has no relationship to the phenytoin or radiation therapy dosage, or to the histologic type of brain tumor. Also, EMM has no apparent age or gender predisposition in association with phenytoin-radiation therapy. Thus this is a clinical phenomenon that occurs with unusual frequency in patients with brain tumor who undergo radiation therapy while taking phenytoin. phenytoin and other anticonvulsants such as phenobarbital and carbamazepine induce cytochrome P450 3A and produce oxidative reactive intermediates that may be implicated in hypersensitivity reactions such as EMM. Both carbamazepine and barbiturates have shown cross-sensitivity with phenytoin; furthermore, a case of EMM in a patient receiving carbamazepine and whole brain radiation therapy has been reported. As carbamazepine, valproate, and barbiturates have been associated with EMM, gabapentin may be considered as alternative anticonvulsant therapy when appropriate.
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3/1162. Secondary glioblastoma remarkably reduced by steroid administration after anaplastic transformation from gliomatosis cerebri--case report.

    A 45-year-old female presented with gliomatosis cerebri manifesting as hemiballismus-like involuntary movement in the arm, motor weakness in the leg, and hypesthesia in her left side. Computed tomography showed only diffuse swelling of the right cerebral hemisphere, but T2-weighted magnetic resonance imaging revealed a diffuse lesion spreading from the right thalamus to the temporal, parietal, and occipital lobes on the same side. No abnormal enhancement was recognized. cerebral angiography showed no specific finding. A right occipital lobectomy was performed to confirm the diagnosis of gliomatosis cerebri. Anaplastic transformation was recognized 5 months later. The disease did not resolve with radiation or interferon administration, but steroid therapy achieved remarkably effective tumor regression. The patient died due to pneumonia. autopsy showed the features of diffuse glioblastoma. Steroid therapy may be an effective treatment for gliomatosis cerebri before the terminal stage.
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4/1162. anesthesia for cesarean section in two patients with brain tumours.

    PURPOSE: To describe two patients with brain tumours where general anesthesia was used for cesarean sections under emergency and urgent conditions. CLINICAL FEATURES (CASE #1): The first patient presented at 38 wk gestation with an acute intracranial tumour herniation, requiring emergency craniotomy and simultaneous cesarean section. General anesthesia was induced with thiopental and vecuronium, maintained with enflurane 1% in O2 100%. Maternal P(ET)CO2 was maintained at 25 mmHg. After delivering a healthy infant, she was given syntocinon, mannitol and dexamethasone i.v. anesthesia was maintained with fentanyl, nitrous oxide 50% in O2 and isoflurane 1% during frontal-lobe tumour resection. CLINICAL FEATURES (CASE #2): The second patient presented at 37 wk gestation for urgent cesarean section because of placental insufficiency. She had had a brain tumour resection four years earlier. An increase in intracranial pressure necessitated craniotomy for decompression at 20 wk gestation. She was further treated with dexamethasone, carbamazepine and radiation for control of cerebral oedema at 34 wk. cesarean section was performed under general anesthesia; rapid-sequence-induction with thiopental and succinylcholine, followed by isoflurane 1% in O2 100%. Syntocinon, fentanyl and atracurium i.v. were administered after delivery of a healthy infant. Although neurosurgeons stood by, their intervention was unnecessary. CONCLUSION: General anesthesia remains safe and dependable for operative delivery in parturients with intracranial tumour. Tracheal intubation allows maternal hyperventilation thereby controlling raised intracranial pressure. Hemodynamic stability is readily achieved to maintain cerebral perfusion. However, a multidisciplinary-team approach is critical for successful patient management.
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5/1162. Cerebral lymphomatoid granulomatosis. A report of two cases, with disseminated necrotizing leukoencephalopathy in one.

    Neuropathologic findings in 2 cases of cerebral lymphomatoid granulomatosis with sequelae are presented. A 30-year old male with macular rash and pulmonary lymphomatoid granulomatosis responded to prednisone terapy but developed acute intracranial hypertension with coma. A necrotizing hemorrhagic lesion was evident in the left putamen surrounded by diffuse and perivascular atypical lymphoplasmacytic infiltration. An 18-year old girl developed pulmonary lymphomatoid granulomatosis, diplopia, slurred speech and right hemiparesis. brain scan, angiography and EEG suggested a left fronto-parietal mass assumed to represent lymphomatoid granulomatosis. She responded well to cerebral irradiation, intrathecal methotrexate and cytoxan but relapsed with seizures and increasing respiratory insufficiency. At autopsy, stigmata of cerebral lymphomatoid granulomatosis were absent but a parenchymatous degeneration consistent with disseminated necrotizing leukoencephalopathy following antileukemic therapy in children, was found.
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6/1162. Auditory brainstem response and neuropathology in a case of systemic T-cell lymphoma with intracranial metastasis.

    A 58-year-old male with systemic T-cell lymphoma involving the brainstem eccentrically, showed varying abnormal auditory brainstem responses (ABRs). The initial ABR demonstrated a prolonged V wave peak latency and an I-V IPL on admission. Four months later, all waves were absent bilaterally. Neuropathological examination at autopsy revealed that most of the nuclei and tracts associated with the auditory system were destroyed by lymphoid cell infiltration. It suggested that in the early stages of this disease, lymphoid cells could have involved the lower brainstem and, subsequently, in the advanced stages invaded the inferior colliculus.
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7/1162. Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl.

    We report the clinical course of a prepubertal girl with central diabetes insipidus (DI) and consequent panhypopituitarism evolving over a period of 10 years due to lymphocytic hypophysitis and subsequent germinoma. Two years after the diagnosis of central DI was established, MRI revealed a thickened pituitary stalk. Later pituitary enlargement and increasing thickening of the pituitary stalk impinging on the optic chiasm required a trans-sphenoidal biopsy which disclosed active hypophysitis with lymphocytic infiltrates and necrosis. High dose dexamethasone treatment only temporarily halted the disease process. Therefore, stereotactic radiation therapy was performed as a rescue treatment and MRI findings almost reversed. However, the subsequent MRI showed multiple intracranial lesions identified histologically as a germinoma and a standard chemotherapy and radiation was performed. CONCLUSION: The diagnosis of diabetes insipidus in children requires long-term follow up beyond the pubertal age in order to establish the underlying cause. In contrast to lymphocytic hypophysitis in adults, lymphocytic hypophysitis in prepubertal children may represent the first sign of a host reaction to an occult germinoma.
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8/1162. Three AML patients with existing or pre-existing intracerebral granulocytic sarcomas who were successfully treated with allogeneic bone marrow transplantations.

    We report three acute myelogenous leukemia (AML) patients who developed intracerebral granulocytic sarcomas (GS) and were successfully treated with allogeneic BMT (allo-BMT). The diagnosis of one patient was AML M2 with myelofibrosis, and the other two patients were AML M4 with eosinophilia (AML M4 Eo), according to the FAB classification. Two patients first experienced a relapse in the brain that resulted in the formation of GS, followed by a relapse in the bone marrow. The remaining patient developed an optic nerve GS after suffering a bone marrow relapse. All three patients received irradiation for the GS and systemic chemotherapy before the allo-BMT. TBI was used for conditioning, and GVHD prophylaxis was with cyclosporine (CsA) and short-term MTX in all three cases. These patients are currently 9 to 37 months post-BMT without relapse. Thus, our experience suggests that allo-BMT is an effective treatment for AML patients with existing or pre-existing intracerebral GS.
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keywords = radiation
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9/1162. October 1998--61 year old male with brain tumor and oral, lung, and palpebral masses.

    In Jan. 97 a gliosarcoma was diagnosed in a 61-year- old man after a 6-month history with neurological deficits. A total physical examination, laboratory tests, chest x-ray and abdominal ultrasound scanning revealed no gross abnormalities. Surgery was followed by brain radiation therapy and 6 months later there were metastases to the oral cavity, right palpebra and both lungs. The histological findings of the oral and palpebral metastases revealed only the sarcomatous component. We are aware of 15 cases of gliosarcoma with extraneural metastases, and in 4 of these, the metastases contained only the sarcomatous component. We believe that our case represents the fifth case of pure sarcomatous metastases.
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10/1162. dementia following treatment of brain tumors with radiotherapy administered alone or in combination with nitrosourea-based chemotherapy: a clinical and pathological study.

    A retrospective clinical and pathological study of 4 patients who developed the syndrome of radiation induced dementia was performed. All patients fulfilled the following criteria: (1) a history of supratentorial irradiation; (2) no evidence of symptomatic recurrent tumor; (3) no other cause of progressive cerebral dysfunction and dementia. The clinical picture consisted of a progressive "subcortical" dementia occurring 3-12 months after a course of cerebral radiotherapy. Examination revealed early bilateral corticospinal tract involvement in all patients and dopa-resistant Parkinsonian syndrome in two. On CT scan and MRI of the brain, the main features consisted of progressive enlargement of the ventricles associated with a diffuse hypodensity/hyperintensity of the white matter best seen on T2 weighted images on MRI. The course was progressive over 8-48 months in 3 patients while one patient had stabilization of his condition for about 28 years. Treatment with corticosteroids or shunting did not produce sustained improvement and all patients eventually died. Pathological examination revealed diffuse white matter pallor with sparing of the arcuate fibers in all patients. Despite a common pattern on gross examination, microscopic studies revealed a variety of lesions that took two basic forms: (1) a diffuse axonal and myelin loss in the white matter associated with tissue necrosis, particularly multiple small foci of necrosis disseminated in the white matter which appeared different from the usual "radionecrosis"; (2) diffuse spongiosis of the white matter characterized by the presence of vacuoles that displaced the normally-stained myelin sheets and axons. Despite a rather stereotyped clinical and radiological course, the pathological substratum of radiation-induced dementia is not uniform. Whether the different types of white matter lesions represent the spectrum of a single pathological process or indicate that the pathogenesis of this syndrome is multifactorial with different target cells, remains to be seen.
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