Cases reported "Brain Neoplasms"

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1/987. Surgical management of pediatric tumor-associated epilepsy.

    Brain tumors are a common cause of seizures in children. Early surgical treatment can improve seizure outcome, but controversy exists regarding the most appropriate type of surgical intervention. Some studies suggest tumor resection alone is sufficient, while others recommend mapping and resection of the surrounding epileptogenic foci to optimize seizure outcome. To address this issue, we reviewed the charts of 34 pediatric patients aged 18 months to 20 years with medically intractable epilepsy and primary brain tumors. The average age at operation was 12.6 years, and patients had seizures for an average of 6.4 years. The majority of tumors were located in the temporal lobe. Seventeen patients, because of tumor location near an eloquent area, underwent extraoperative mapping using subdural electrode grids prior to definitive tumor resection. Fourteen of these patients had a gross total tumor resection, yet only two had a distinct zone of ictal onset identified and resected. The remaining 17 patients had tumors either in the nondominant hemisphere or far removed from speech-sensitive areas, and therefore did not undergo extraoperative subdural electroencephalograph mapping. Fourteen of these patients also had a gross total tumor resection, while none had intraoperative electrocorticography to guide the resection of additional nontumoral tissue. overall, of the 28 patients treated with a gross total tumor resection, 24 (86%) are seizure free, while the other four are significantly improved. Of the six patients who had a subtotal tumor removal, five have persistent seizures. The mean follow-up was 3.6 years. We conclude that in children and adolescents, completeness of tumor resection is the most important factor in determining seizure outcome. The routine mapping and resection of epileptogenic foci might not be necessary in the majority of patients. As a corollary, the use of subdural electrode grids in pediatric patients with tumor-associated epilepsy should be limited to cases requiring extraoperative cortical stimulation for localization of nearby eloquent cortex.
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2/987. central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.

    central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2]. We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.
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3/987. Middle ear adenocarcinoma with intracranial extension. Case report.

    Middle ear adenocarcinoma is a very rare, locally invasive neoplasm assumed to arise from the middle ear mucosa. Although endolymphatic sac tumor (aggressive papillary middle ear tumor) and jugulotympanic paraganglioma may show brain invasion, intracranial extension of histologically confirmed middle ear adenocarcinoma has not been previously reported. The authors describe a 53-year-old man who suffered from otalgia and tinnitus for more than 10 years and from neurological deficits for 1 year due to a large temporal bone tumor that invaded the temporal lobe. A combined neurosurgical and otolaryngological resection was performed. Pathological analysis revealed a low-grade adenocarcinoma of a mixed epithelial-neuroendocrine phenotype, which showed a close histological similarity to, and topographical relationship with, middle ear epithelium. The authors conclude that middle ear adenocarcinoma belongs to the spectrum of extracranial tumors that have possible local extension to the brain.
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4/987. Integration of preoperative and intraoperative functional brain mapping in a frameless stereotactic environment for lesions near eloquent cortex. Technical note.

    The authors present a method of incorporating preoperative noninvasive functional brain mapping data into the frameless stereotactic magnetic resonance (MR) imaging dataset used for image-guided resection of brain lesions located near eloquent cortex. They report the use of functional (f)MR imaging and magnetic source (MS) imaging for preoperative mapping of eloquent cortex in difficult cases of brain tumor resection such as those in which there are large expansive masses or in which reoperations are required and the anatomy is distorted from prior treatments. To correlate methods of preoperative and intraoperative mapping localization directly, the authors have developed techniques of importing preoperative MS and fMR imaging data into an image-guided frameless stereotactic computer workstation. The data appear as a seamless overlay on the same preoperative volumetric MR imaging dataset used for stereotactic guidance during the operation. Intraoperatively identified functional locations mapped by cortical stimulation are recorded as digitally registered points. This approach should prove useful in assessing the accuracy and reliability of various preoperative functional brain mapping techniques.
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5/987. Microvascular reconstruction of the skull base: indications and procedures.

    PURPOSE: The aim of the current study was to review the use of free tissue transfer for reconstruction of the skull base and for coverage of intracranial contents. patients AND methods: From 1990 until 1996, revascularized flaps were transferred to the skull and the skull base in 11 patients in whom intracranial/extracranial resection of tumors of the skull base was performed in cooperation with the Department of neurosurgery. The defects resulted from removal of squamous cell carcinomas (n = 4), basal cell carcinomas (n = 4), malignant melanoma, malignant schwannoma, and malignant meningioma. Defect repair was accomplished by revascularized transfer of latissimus dorsi muscle flaps in seven cases and rectus abdominis flaps and forearm flaps in two cases each. In five patients with extensive intracranial tumor spread, reconstruction was performed for palliative reasons. RESULTS: A safe soft tissue closure of the intracranial and intradural space was achieved in all patients, whereas the contour of the facial skull and the neurocranium was satisfactorily restored at the same time. By using the entire length of the grafted muscle, the vascular pedicle could be positioned next to the external carotid artery and conveniently connected to the cervical vessels. The mean survival time of the patients with palliative treatment was 8.4 months, with an average duration of hospital stay of 24.5 days. CONCLUSIONS: Despite the increased surgical effort of revascularized tissue transfer, microvascular reconstruction of large skull base defects appears to be justified, even as a palliative measure.
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6/987. 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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7/987. Progressive multifocal leukoencephalopathy (PML) and cerebral toxoplasmosis in an adult patient, with no symptoms of underlying immunosuppressing illness.

    We present a case of the coincidence of progressive multifocal leukoencephalopathy (PML) and central nervous system (CNS) toxoplasmosis in an adult patient, without a detectable cause of cell-mediated immunity impairment. The proper diagnosis was made postmortem on the basis of histological changes typical of both pathological processes. PML was characterized by the presence of subcortical focal demyelination, containing enlarged, densely basophilic oligodendrocyte nuclei, often with intranuclear inclusion, and bizarre astrocytes, mimicking neoplastic cells. PML was confirmed by detecting numerous papova virus particles in oligo- and astroglial nuclei by thin-section electron microscopy. Cerebral toxoplasmosis was characterized by the presence of multiple well-circumscribed necrotizing abscesses. Numerous toxoplasma gondii (T. gondii) cysts and free, non-encysted protozoan parasites were found among the inflammatory infiltrates. The diagnosis of cerebral toxoplasmosis was further confirmed by immunocytochemistry. In order to detect putative immunosuppressive background underlying both pathological processes, hiv infection was taken into consideration, however, no histopathological changes indicative of AIDS either in the CNS or in the peripheral organs were eventually found. Moreover no hiv provirus genome was identified in the formalin-fixed, paraffin embedded brain tissue by the polymerase chain reaction (PCR). Current view on the selected aspects of the pathogenesis of both disorders were discussed.
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8/987. MRI diagnosis of gliomatosis cerebri.

    Until recently the diagnosis of gliomatosis cerebri has been made on postmortem examination. This article reviews the use of serial magnetic resonance imaging studies to suggest premorbid diagnosis of this condition. The following is a case report of a 14-year-old female who had a subtotal cortical resection of tumor and several years later developed a progressive dementia. At postmortem examination the diagnosis of gliomatosis cerebri was made. Diffuse progressive white matter changes involving both hemispheres and brainstem, with increased thickness of the corpus callosum and without changes in cortical markings on T2-weighted magnetic resonance images, in this patient were highly suggestive of the diagnosis of gliomatosis cerebri.
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9/987. Composite ganglioglioma and dysembryoplastic neuroepithelial tumor.

    Both ganglioglioma and dysembryoplastic neuroepithelial tumors are well-recognized glial-neuronal neoplasms associated with chronic epilepsy and cortical dysplasia (neuronal migration abnormalities). The exact relationship between these 2 glial-neuronal tumors continues to be debated. This article reports a case of a composite ganglioglioma and dysembryoplastic neuroepithelial tumor occurring in a 36-year-old woman in the left temporal lobe region. The resection histologically demonstrated distinct areas of ganglioglioma and dysembryoplastic neuroepithelial tumor. A focal area of cortical dysplasia is also identified. The MIB-1 labeling indexes in both components were low (<1% of tumor cell nuclei). The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors.
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10/987. A case of a malignant melanoma with late metastases 16 years after the initial surgery.

    We report a case of a pulmonary metastasis 16 years after the initial surgery for a malignant melanoma. The patient was a 58-year-old Japanese man. In 1976, he had a pigmented skin lesion with a diameter of 8 mm on his right third finger. He received an amputation of the finger and a dissection of the right axillary. Histological examinations of the tumor revealed a feature of a malignant melanoma with infiltration of the papillary layers of the dermis, 1.5 mm in thickness. The histological subtype was considered to be an acral lentiginous melanoma with a mixed spindle-epithelioid cell pattern. There was no regional lymph node metastasis. In December 1992, when he was 74-years-old, a round tumor in the left lower lung was discovered by chest radiography. In February 1993, he received a left lower lobectomy of the lung. Histological examination revealed a feature of a malignant melanoma with predominantly epithelioid cells and this was considered to be a metastasis from the initial skin lesion. Five months after the lobectomy, he died from a hemorrhage of a metastatic brain tumor. This case indicated the importance of periodic, life-long follow-up in treating malignant melanomas.
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