Cases reported "Brain Neoplasms"

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1/845. Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings.

    Primary central nervous system lymphoma (PCNSL) usually presents with clinical and neuroimaging findings consistent with single or multiple intracranial mass lesions. On cranial magnetic resonance imaging (MRI), such lesions are nearly always contrast enhancing, reflecting disruption of the blood-brain barrier at the site of tumor nodules. We describe 2 cases from the UCLA Medical Center who developed a rapidly progressive dementia due to extensive gray and white matter cerebral lesions involving much of the brain. In the patient who came to autopsy, widely infiltrating, focally necrotic B-cell plasmacytoid lymphoma was noted throughout the cerebral neuraxis. MRI findings in case 2 were consistent with diffuse lymphomatous brain infiltration without mass lesions, which was biopsy proven. We conclude that PCNSL may occur in a diffusely infiltrating form which may occur without MRI evidence of mass lesions or blood-brain barrier compromise. We refer to this entity as 'lymphomatosis cerebri' and add it to the differential diagnosis of a rapidly progressive dementia.
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2/845. Successfully treated central nervous system Burkitt's lymphoma with minimal extraneural disease in a child.

    Burkitt's lymphoma, the most common childhood non-Hodgkin's lymphoma, usually presents with abdominal tumors. central nervous system (CNS) involvement in Burkitt's lymphoma is mostly secondary to advanced systemic disease, rarely with brain parenchymal lesions, and was previously recalcitrant to treatment. We report an unusual case of CNS Burkitt's lymphoma with minimal extraneural disease. This 10-year-old immunocompetent boy presented with increased intracranial pressure and seizures and was found to have multifocal intracerebral lesions on brain imaging studies. cerebrospinal fluid studies confirmed the presence of Burkitt's lymphoma cells. Abdominal computed tomography showed bilateral nephromegaly with left intrarenal lesions that disappeared after three doses of intravenous dexamethasone. The patient was treated for 6.5 months according to the LMB 89 group C protocol of the French Pediatric Oncology Society. The response was brisk and complete. The patient has been disease free for more than 4 years, and is believed to be cured.
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keywords = nervous system
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3/845. 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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keywords = nervous system
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4/845. 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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keywords = nervous system
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5/845. Primary intracerebral Hodgkin's disease: report of a case with Epstein-Barr virus association and review of the literature.

    A case of primary intracerebral Hodgkin's disease (HD) without dural attachment in a 54-year-old immunocompetent patient is described. The infiltrate was located superficially in the occipital lobe and corresponded to the histologic type of nodular sclerosis. A typical immunohistochemical profile (membrane and cytoplasmic staining with dotlike Golgi enhancement of CD30, moderate cytoplasmic staining of CD15 in the Golgi area, membrane staining of CD20 of <10% of blastic cells, CD45RB negative) and in addition Epstein-Barr virus (EBV) latent membrane protein was detectable in reed-sternberg cells. Staging revealed no other organ sites of involvement. After combined surgery, postoperative radiotherapy, and chemotherapy, there are no signs of recurrence or systemic disease on follow-up for >1 year. To the authors' best knowledge, an association of EBV with primary central nervous system HD has not been demonstrated before.
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keywords = nervous system
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6/845. Surgical resection of a cerebral metastasis from cervical cancer: case report and review of the literature.

    AIMS AND BACKGROUND: central nervous system metastasis from cervical carcinoma is uncommon. CASE REPORT: We report the case of a 51-year-old woman who developed a solitary cerebral metastasis 29 months after radical hysterectomy with pelvic lymphadenectomy for a stage IB, grade III cervical cancer. The patient suddenly complained of headache, confusion and dizziness; she was submitted to emergency surgical resection of a 2 x 3 cm metastasis in the right frontal lobe. The postoperative course was uneventful and she completely recovered from her neurological deficit. Following surgery the patient underwent careful restaging. Massive bilateral involvement of the pelvic wall was diagnosed, and the patient received three courses of cisplatin-based chemotherapy. She developed liver and lung metastases and died 10 months later of progressive disseminated disease, without, however, any sign of recurrent or persistent cerebral involvement. CONCLUSION: Neurosurgical resection should be considered in cervical cancer patients with solitary brain metastasis in the absence of systemic disease.
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7/845. Cerebral metastasis from ovarian carcinoma. Some observations about treatment.

    Systemic metastases from ovarian carcinoma are frequent, but they rarely affect the central nervous system. The treatment of this type of metastases in not clear. Two cases of solitary cerebral metastasis from ovarian carcinoma are reported. Two patients, submitted to therapeutic protocol established for ovarian carcinoma, presented after 17 and 25 months respectively the appearance of symptoms from brain solitary metastasis without other metastases. They underwent surgery, radiotherapy and chemotherapy for solitary cerebral metastasis. Treatment of the brain lesion resulted in KPS improvement (KPS = 90) and survival was 16 and 30 months, respectively. From the 2 cases presented and the review of the literature, it appears that a better outcome may be obtained by a combined treatment of metastases, including surgery, radiotherapy and chemotherapy.
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keywords = nervous system
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8/845. Extramedullary tumor of monoblasts in the central nervous system: presenting feature of simultaneous bone marrow involvement by acute monocytic leukemia.

    An extramedullary tumor of monoblasts in the central nervous system has been described in the literature as a single case report, preceding the development of acute monocytic leukemia by 1 year. We report a previously undescribed presentation of acute monocytic leukemia as a left temporal lobe extra-axial mass with concomitant bone marrow involvement. We describe our findings in order to heighten awareness of this entity, which may be encountered in morphologic evaluation of central nervous system masses.
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keywords = nervous system
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9/845. acquired immunodeficiency syndrome-related intraocular B-cell lymphoma.

    OBJECTIVES: To present the full clinical spectrum of the acquired immunodeficiency syndrome-related intraocular lymphoma as manifested in the eye, specifically retinal lymphoma associated with primary central nervous system lymphoma, isolated ocular lymphoma, and choroidal lymphoma associated with systemic lymphoma. methods: Three patients with acquired immunodeficiency syndrome were noted to have atypical retinal lesions. Diagnostic retinal biopsy in 2 patients and postmortem examination of the eyes in the third case were performed. RESULTS: Diagnostic retinal biopsy in the first 2 patients revealed retinal B-cell lymphoma. Initial systemic evaluation showed the eyes to be the sole site of disease. Later, in 1 of these patients, the lymphoma spread to the brain. The third patient developed an acute abdomen 4 months after the development of his ocular findings. The histological evaluation of the resected bowel revealed high-grade B-cell lymphoma. The patient died 1 week later and postmortem analysis of the eyes disclosed the presence of lymphoma in the choroid of both eyes. CONCLUSIONS: This is the most complete series of patients with acquired immunodeficiency syndrome-related intraocular B-cell lymphoma and, to our knowledge, provides the first 2 cases diagnosed by retinal biopsy. These 3 cases present the full clinical spectrum of the disease as manifested in the eye.
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keywords = nervous system
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10/845. Cytologic diagnosis of a solitary brain metastasis from papillary carcinoma of the thyroid. A case report.

    BACKGROUND: Papillary carcinoma of the thyroid metastasizes to the brain in rare instances. In published series and case reports of metastatic papillary thyroid carcinoma, diagnosis of central nervous system (CNS) metastases has been determined by histologic methods. We present a case of papillary carcinoma metastatic to brain diagnosed by cytologic methods. CASE: A 43-year-old female, initially diagnosed at age 12 with papillary carcinoma of the thyroid metastatic to regional lymph nodes and lung, presented with head aches of increasing frequency and severity. A computed tomography scan confirmed a 1-cm nodule in the right inferior frontal lobe of the brain. For clinical reasons, the patient was followed with serial imaging for five years. At age 48 there was significant progression of the CNS disease, and the patient underwent stereotactic biopsy with drainage of cyst fluid. Cytologic examination of the cyst fluid and immunocytochemical studies confirmed the typical features of papillary thyroid carcinoma, including papillary clusters of cells with finely granular chromatin, micronucleoli, nuclear grooves and an associated psammoma body. CONCLUSION: Neurocytology is a useful technique in the examination of cystic lesions of the brain and may be the sole technique for determination of diagnosis.
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