Cases reported "Choroid Plexus Neoplasms"

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1/39. Chordoid glioma of the third ventricle: confirmatory report of a new entity.

    The term "chordoid glioma" was recently introduced to denote a circumscribed, apparently low-grade neoplasm arising in or preferentially involving the third ventricle of middle-aged women. We report biopsy and postmortem findings in a 60-year-old woman with symptoms of forgetfulness, headache, and lethargy. neuroimaging showed a contrast-enhancing third ventricular mass with obstructive hydrocephalus. The tumor was subtotally resected. Microscopically, it consisted of clusters and strands of epithelioid cells in a mucoid matrix. Its margins were remarkably discrete and showed little tendency to infiltrate surrounding brain parenchyma. The majority of neoplastic cells were glial fibrillary acidic protein (GFAP) and vimentin positive, whereas S100 protein labeled only individual cells. Stains for epithelial membrane antigen (EMA) and cytokeratin were nonreactive. There was no evidence of neuroendocrine differentiation or expression of estrogen and progesteron receptors. Lymphoplasmacellular infiltrates were noted throughout the lesion and at the tumor-brain interface. The MIB-1 labeling index averaged 1.5%. At present, chordoid glioma is considered a glial neoplasm of uncertain histogenesis with distinct clinicopathologic features.
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keywords = brain
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2/39. Chordoid glioma of the third ventricle: immunohistochemical and molecular genetic characterization of a novel tumor entity.

    Chordoid glioma of the third ventricle was recently reported as a novel tumor entity of the central nervous system with characteristic clinical and histopathological features (Brat et al., J Neuropathol Exp Neurol 57: 283-290, 1998). Here, we report on a histopathological, immunohistochemical and molecular genetic analysis of five cases of this rare neoplasm. All tumors were immunohistochemically investigated for the expression of various differentiation antigens, the proliferation marker Ki-67, and a panel of selected proto-oncogene and tumor suppressor gene products. These studies revealed a strong expression of GFAP, vimentin, and CD34. In addition, most tumors contained small fractions of neoplastic cells immunoreactive for epithelial membrane antigen, S-100 protein, or cytokeratins. The percentage of Ki-67 positive cells was generally low (<5%). All tumors showed immunoreactivity for the epidermal growth factor receptor and schwannomin/merlin. There was no nuclear accumulation of the p53, p21 (Waf-1) and Mdm2 proteins. To examine genomic alterations associated with the development of chordoid gliomas, we screened 4 tumors by comparative genomic hybridization (CGH) analysis. No chromosomal imbalances were detected. More focussed molecular genetic analyses revealed neither aberrations of the TP53 and CDKN2A tumor suppressor genes nor amplification of the EGFR, CDK4, and MDM2 proto-oncogenes. Our data strongly support the hypothesis that chordoid glioma of the third ventricle constitutes a novel tumor entity characterized by distinct morphological and immunohistochemical features, as well as a lack of chromosomal and genetic alterations commonly found in other types of gliomas or in meningiomas.
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keywords = central nervous system, nervous system
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3/39. choroid plexus metastasis from carcinoma of the bladder: case report and review of the literature.

    We present here a case report of a 40-year old male with adenocarcinoma of the bladder and solitary metastasis to the choroid plexus of the right lateral ventricle. This is the first such report of such a metastasis in association with bladder carcinoma. Systemic metastases frequently occur in patients with carcinoma of the bladder but involvement of central nervous system is relatively uncommon: less than 1% of patients with carcinoma of the bladder present an intracerebral metastasis. In the majority of cases there are either multiple CNS metastases or other distant metastases. A few cases present with solitary metastases to the CNS without evidence of recurrent or disseminated disease.
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keywords = central nervous system, nervous system
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4/39. Chordoid glioma: a novel tumor of the third ventricle.

    Chordoid glioma of the third ventricle is a recently characterized primary neoplasm of the central nervous system. We present a case and discuss the pathologic and radiologic features. We are aware of only 16 other cases documented in the world literature. This radiologic-pathologic correlation alerts pathologists and radiologists to recognize chordoid glioma as a distinct clinicopathologic entity restricted to the third ventricular area of adult patients.
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keywords = central nervous system, nervous system
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5/39. [prenatal diagnosis of a brain tumour--an example of diagnostic and therapeutical algorithm]

    The authors describe their cooperation in the diagnosis and treatment of a newborn with malignant brain tumour (rare case of carcinoma of the choroid plexus) recognised by means of prenatal sonography and magnetic resonance. The case history is an example of modern algorithm of diagnostic and therapeutic procedures in perinatal medicine and the necessary multicentre collaboration.
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6/39. choroid plexus papilloma diagnosed by crush cytology.

    In a 32-yr-old man, an infratentorial cystic lesion with a mural nodule was interpreted to be either a hemangioblastoma or a cystic astrocytoma on CT scan. Intraoperative crush cytology revealed it to be a choroid plexus papilloma (CPP). The utility of crush cytology in the rapid diagnosis of central nervous system (CNS) tumors and the differential diagnosis of CNS papillary lesions are highlighted in this report.
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keywords = central nervous system, nervous system
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7/39. choroid plexus carcinoma presenting as an intraparenchymal mass.

    A 6-year-old girl with a history of a nondisplaced skull fracture diagnosed with computerized tomography (CT) scanning 3 years previously presented with a 6-week history of headaches and decreased use of her right side. On admission CT scans, a large cystic mass was identified in the left frontal lobe region of the brain. A connection between the mass and the ventricular system was not seen on radiological examination or during surgery. Gross-total resection of the mass was achieved. The histological and immunohistochemical findings in the resected tissue confirmed a diagnosis of choroid plexus carcinoma (ChPC). This is the first reported case of a ChPC arising in an extraventricular location not associated with the choroid plexus.
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8/39. Disseminating histologically benign multiple papilloma of the choroid plexus: case report.

    A rare case of extensively disseminating multiple benign choroid plexus papilloma is shown. The patient first reported with high-grade hydrocephalus and two tumours in the 3rd and 4th ventricle was treated by atrioventricular shunt insertion, subtotal resection of the 4th ventricle tumour, and adjuvant 60Co irradiation of the posterior fossa. The dissemination that followed was revealed by computerised tomography and magnetic resonance imaging, and involved both the supra- and infratentorial ventricular systems, spinal canal, and brain parenchyma. Three years after the resection of the 4th ventricle tumour, the patient underwent excision of a temporal lobe lesion for relief of neurological symptoms, but showed no improvement and died 5 years after the primary diagnosis of CNS tumour. An autopsy was not performed. Analysis of the primarily resected mass showed distinct papillary pattern with no anaplasia, mitoses, multinucleation orgiant cell formation, and cytokeratin positivity at the absence of vimentin and glial fibrillary acidic protein. Analysis ofthe temporal lobe tumour again showed definite papillary formation with no signs of malignisation and virtually no mitotic figures, and the presence of cytokeratin, but not vimentin or glial fibrillary acidic protein. On both occasions, the diagnosis was choroid plexus papilloma (WHO grade I).
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9/39. Chordoid glioma of the third ventricle: a report of two new cases, with further evidence supporting an ependymal differentiation, and review of the literature.

    The term chordoid glioma of the third ventricle was first used to describe a rare and slowly growing neoplasm of uncertain histogenesis, with chordoid appearance, occurring preferentially in middle-aged women. Herein we report two additional examples of this novel entity together with a literature review based on the 25 cases previously published. Our review fully confirms the strikingly stereotyped clinical, neuroradiologic, and pathologic features of this unique tumor. The female/male ratio was 1.7:1, and the age range was 24-70 years (mean 44.9 years). In all 27 cases imaging findings were similar showing a well-defined mass (mean 2.8 cm in largest dimension), ovoid in shape, hyperdense on CT scans, with uniform and intense contrast enhancement, arising in the hypothalamic/suprasellar/third ventricular region. Histologically, the main consistent characteristics were cords and clusters of epithelioid cells within an abundant mucinous and often vacuolated background. Mitoses were sparse or absent and anaplastic features, endothelial proliferation, and necrosis were not identified. Lymphoplasmacytic infiltrates with Russell bodies were frequent throughout the tumor and its interface with adjacent brain parenchyma. Most of the tumor cells revealed a strong and diffuse expression of vimentin and glial fibrillary acidic protein. Additionally, the vast majority of tumors showed focal coexpression of cytokeratins, CD34, S-100 protein, and epithelial membrane antigen; the MIB-1 labeling indices were uniformly low. Surprisingly for a glioma assigned WHO grade II, the 19 patients with an available but short follow-up (mean 22.5 months; range 6-68 months) experienced a rather poor outcome (three recurrences and seven deaths), probably reflecting the anatomic site of the neoplasm that precludes a complete surgical excision rather than its histologic composition. Ultrastructural examination of 10 cases demonstrated findings in line with a glial derivation and a putative ependymal origin such as cytoplasmic intermediate filaments, microvilli, intermediate junctions or desmosomes, and focal basal lamina formation. In our case no. 1, and for the first time in this tumor, we observed sparse and abnormal cilia in an aberrant juxtanuclear location, a further argument for considering chordoid glioma as a subtype of ependymoma. However, a better understanding of the biologic behavior and histogenesis of this distinctive clinicopathologic entity needs to be investigated with a larger series. Nevertheless, taking into account its strikingly consistent anatomic localization, its unique histopathologic and immunohistochemical profile, in conjunction with the most recent and convincing ultrastructural arguments, we suggest that chordoid glioma of the third ventricle could be better classified as chordoid ependymoma of the lamina terminalis area.
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10/39. Efficacy of sequential chemotherapy including methotrexate and doxorubicin in an infant with partially resected choroid plexus carcinoma.

    This report refers to a 3-month-old male, with a residual choroid plexus carcinoma following partial resection, who was successfully treated with sequential chemotherapy without any postoperative radiation therapy. Along with carboplatin, we also used doxorubicin and methotrexate, hypothesizing that, given the patient's age, the blood-brain barrier should not hamper drug delivery to the tumor. According to this hypothesis, the treatment achieved complete remission of the disease, which lasts 27 months after the diagnosis. This result deserves further studies to assess the possible curative role of chemotherapy in very young patients suffering from choroid plexus carcinoma.
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