Cases reported "Brain Diseases"

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1/56. Intracranial extracerebral neuroglial heterotopia: A case report and review of the literature.

    Heterotopic masses of neuroglial tissue are uncommon and most frequently involve extracranial midline structures. We report an unusual case of an intracranial, extracerebral neuroglial heterotopia involving the middle and anterior cranial fossae of a 5-year-old girl who presented with facial asymmetry. The lesion was composed of mature but disorganized gray and white matter admixed with surrounding soft tissues and exhibited histologic features reminiscent of cortical dysplasia. These rare lesions have been postulated to arise from a protrusion of tissue from the neuraxis through a pial defect, from abnormalities in the migration of embryonic neuroepithelial tissue, or from an accessory evagination of the neural tube inferior to the telencephalic vesicles. Regardless of the underlying pathogenic mechanism, these lesions must be histologically distinguished from both teratomas and primary central nervous system neoplasms.
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ranking = 1
keywords = neoplasm
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2/56. Intracranial papillary endothelial hyperplasia: occurrence of a case after surgery and radiosurgery.

    Papillary endothelial hyperplasia (PEH) is considered a form of endothelial proliferation rather than a true neoplasm and is usually located in the skin or subcutis. We report a case of intracranial PEH that occurred after surgery for glioma and subsequent radiosurgery. CT and MR revealed an enhancing extra-axial mass located left posterolateral to the brainstem. Intracranial PEH is rare; to our knowledge, development of an intracranial PEH after surgery and radiosurgery has not been previously reported.
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ranking = 1
keywords = neoplasm
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3/56. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.

    This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.
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ranking = 4
keywords = neoplasm
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4/56. Pleomorphic xanthoastrocytoma derived from glioneuronal malformation in a child with intractable epilepsy.

    Malformative lesions as well as neoplasms can cause intractable epilepsy in childhood. Even though the neoplastic nature of a lesion is evident in most cases, the distinction can be difficult in some patients. We present the case of a child with intractable epilepsy caused primarily by a glioneuronal malformation. Years after the first surgical intervention, a pleomorphic xanthoastrocytoma evolved from remnants of this lesion. This case suggests that glioneuronal malformations might be precursor lesions of pleomorphic xanthoastrocytomas.
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ranking = 1
keywords = neoplasm
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5/56. Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature.

    We present a unique case of biopsy-proven necrotizing sarcoidosis involving the central nervous system (CNS) in a 52-year-old woman. The patient presented with a 3-month history of left-sided headache and sharp, shooting pains on the left side of her face. She also has a previous history of sarcoidosis, histopathologically confirmed on parotid gland biopsy 24 years before. Imaging studies of the present lesion revealed a 1.8 x 1.4-cm mass in the left temporal lobe with signal intensity suggestive of meningioma or low-grade glial neoplasm. Surgical resection was initiated, and intraoperative consultation with frozen sections revealed granulomata. The lesion was biopsied, and surgical intervention was terminated. Permanent sections failed to reveal bacteria, mycobacteria, fungi, or foreign bodies. A diagnosis of necrotizing neurosarcoidosis was rendered. The patient was administered steroid therapy and clinically responded favorably. At the most recent follow-up almost 2 years later, there was no evidence of recurrence or progression. Necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems. We report the first histologically proven case involving the CNS as well as a rare example of sarcoidosis and necrotizing sarcoid granulomatosis in the same patient. sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of a granulomatous mass lesion involving the CNS, particularly in the context of a history of systemic disease.
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ranking = 5
keywords = neoplasm
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6/56. September 2000: 15 year old girl with intracranial lesion.

    The September COM: We reported the case of a 15 year old white girl with solitary brain cortical lesion clinically mimicking meningioma. Microscopic examination revealed histiocytic lesion with foamy and giant cells predominately of Touton type. Subsequent clinical examination did not reveal any similar lesion in the skin or in other location. Expression of S-100 protein in histiocytes is unusual finding, but has been reported recently in juvenile xanthogranulomas (JXG). The only specific ultra-structural finding differing JXG from histiocytic neoplasm of Langerhans' cells histiocytosis type seems to be Birbeck granules.
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ranking = 1
keywords = neoplasm
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7/56. Atypical focal non-neoplastic brain changes in neurofibromatosis type 1: mass effect and contrast enhancement.

    Children and young adults with neurofibromatosis type 1 often have small high-signal foci on T2-weighted images of the brain. We describe follow-up of two patients in whom one of the foci had atypical features, commonly regarded as signs of a neoplasm. In the first, one lesion showed temporary contrast enhancement and decreasing mass effect. The second developed an expanding lesion that increased minimally in size over 4.5 year's follow-up. The borderline between neoplastic and non-neoplastic lesions seems to be indistinct.
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ranking = 1
keywords = neoplasm
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8/56. Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity.

    Subacute diencephalic angioencephalopathy (SDAE) is a rare and fatal disease of unknown etiology that involves the thalami bilaterally. To date, there have been four cases reported, in which the diagnosis was established only after post mortem examination of the brain. We report two male patients, ages 69 and 41 years, who presented with progressive dementia and somnolence. Radiological evaluation revealed enhancing lesions involving both thalami. The differential diagnosis included a number of neoplastic, inflammatory and vascular processes. In both cases, pathological evaluation of biopsy specimens suggested the diagnosis of SDAE. Despite supportive care, the disease progressed rapidly and both patients died within weeks after initial presentation. The diagnosis was confirmed at autopsy in both cases. SDAE is a rare cause of bithalamic disease that can be mistaken for a neoplasm as well as a number of conditions that necessitate different treatment choices. The histopathological findings can establish the diagnosis when combined with radiological and clinical information. This report emphasizes the utility of stereotactic biopsy in early diagnosis of SDAE.
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ranking = 1
keywords = neoplasm
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9/56. Tumor-like presentation of multiple sclerosis.

    multiple sclerosis patients may present with clinical data suggestive of cerebral tumor, however, most of the lesions do not show expansive signs in computerized tomography of brain or magnetic resonance imaging. We report in this paper, 2 patients who had shown expansive radiological signs suggestive of neoplasm. Cerebral biopsy was an important diagnostic procedure in these 2 cases which revealed the diagnosis of demyelinating disease.
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ranking = 1
keywords = neoplasm
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10/56. Calcifying pseudoneoplasm of the CNS.

    Calcifying pseudoneoplasm or fibro-osseous lesion of the CNS is uncommon. We report a case in a 6-year-old boy who presented with generalized tonic-clonic seizures for 6 months. There was a calcified mass in the depth of the left temporal lobe, which on histology showed collagenous nodules surrounded by spindle cells and seperated by chondromyxoid matrix. The only other report of such a lesion in a child is of a 12-year-old boy with an epidural lesion at C-6 level. It is important to recognize this entity since it is slow growing with a good prognosis.
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ranking = 5
keywords = neoplasm
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