Cases reported "Meningeal Neoplasms"

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1/14. meningioma of the fourth ventricle presenting with intermittent behaviour disorders: a case report and review of the literature.

    Intraventricular meningiomas are rare, representing 0.5-5% of all intracranial meningiomas. They arise mostly within the lateral ventricles and more rarely in the third ventricle. Meningiomas of the fourth ventricle are exceptional. They are clearly defined as meningiomas arising from the choroid plexus and lying strictly within the fourth ventricle. We report a 76 year old male patient presenting with a 2-week history of headache and cognitive disorders with agitation and restlessness particularly exacerbated at night or when lying down. CT scan and MR imaging showed a contrast-enhancing lesion located purely within the whole fourth ventricle, with slight ventricular enlargement. At surgery, we totally removed a well-vascularised, greyish encapsulated mass attached to the choroid plexus. Pathological examination revealed a WHO grade I fibroblastic meningioma. We reviewed the literature concerning this unusual meningioma location.
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2/14. gout of the temporomandibular joint: pitfalls in diagnosis.

    gout is a frequent benign disease that rarely affects the temporomandibular joint (TMJ) alone. When it does, the disease is usually confined to the joint space and leads to pain and limitation of jaw opening (acute gout). The case described in this report is atypical in so far as it extended beyond the joint capsule into the pterygoid muscle and destroyed the head of the mandible, the temporal bone and the greater wing of the sphenoid bone. This clinical behaviour in combination with the radiographic appearance created the appearance of a benign but osteolytic lesion. The clinical, radiographic and biological features of gout in the TMJ are reviewed and treatment options are discussed.
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3/14. Late relapse of adrenocortical carcinoma in beckwith-wiedemann syndrome. Clinical, endocrinological and genetic aspects.

    We report on a girl with an unusual beckwith-wiedemann syndrome (BWS) and hemihypertrophy, who developed an adrenocortical carcinoma with atypical clinical behaviour. At 4 y of age the girls was admitted to hospital with cushingoid features, virilization, increased excretion of steroids and low serum ACTH. A right-sided adrenocortical carcinoma was removed. At age 12.5 y the cushingoid features reappeared together with a tumour in the left thigh. A CT scan of the thorax and abdomen revealed pulmonary metastasis only. Corticosteroid excretion was increased and serum ACTH level suppressed. The femoral and the pulmonary metastases were removed and histology showed adrenocortical carcinoma. Excretion of corticosteroids subsequently normalized. Meningeal and pulmonary metastases with similar histologies appeared one year later with normal hormone values. Twenty-two months after the recurrence the girl died of an intracranial metastasis. Southern blot analysis of the LITI transcript in the KvLQT1 gene in the BWS region on chromosome 11p15 revealed hypomethylation of the maternal allele. CONCLUSION: adrenocortical carcinoma in childhood may recur years after onset and at rare sites and hormonal levels may be an insufficient indicator of small metastases.
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4/14. A scalp lesion over an extracerebral mass: a sign of a radiation-induced meningioma.

    radiation-induced meningiomas have a characteristic biological behaviour, so that their recognition is important as regards follow-up. We stress the importance of a scalp lesion over the meningioma on magnetic resonance imaging as a sign of previous radiotherapy.
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5/14. Meningeal melanocytoma of the temporal lobe. An uncommon tumor in an unusual location. Case report.

    Meningeal melanocytomas are rare pigmented tumors of the central nervous system. These tumors are benign melanotic lesions that derive from the melanocytes of the leptomeninges. They may occur anywhere in the cranial and spinal meninges; however, they are found prevalently in the posterior fossa and in the spinal cord. Their epidemiological features, natural history and response to treatment remain poorly understood, even if, in the last 2 years, some reviews have been published about it. We report a new case of intracranial supratentorial meningeal melanocytoma, in the temporal lobe, occurring in a 27-year-old man, admitted to our Institute with a long-time history of seizures. We report histological and radiological characteristics of our case, and briefly review the therapeutical options reported in literature. Preoperative neuroradiological finding is unclear; the preoperative diagnosis is usually meningioma, because of the long duration of symptomatology and the radiological appearance of the lesion as an extra-axial mass. diagnosis of these lesions, as in our case, is made intraoperatively by the gross, jet-black appearance of the tumor and by histological examination. In spite of the benign biologic behaviour, the prognosis remains uncertain, because of the possible local recurrences. According to the results of some works of the last years, it seems appropriate to use postoperative radiotherapy for those patients with symptomatic residual, progressive or recurrent tumors not amenable to further resection.
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6/14. Chordoid meningiomas of a different histopathological pattern: a report of two cases.

    Chordoid meningioma is an uncommon histopathological variant of meningioma with a peculiar chordoma-like appearance. Its association with systemic inflammatory disorder linked to Castleman's syndrome was confirmed in the majority of young patients, however such a relationship in adults remains enigmatic. We report two cases of chordoid meningiomas in adult patients without manifestation of Castleman's syndrome. One tumour was almost totally composed of chordoma-like areas whereas the second one exhibited the unique combination of chordoma- and chondroma-like pattern. This is the first description of chordoid meningioma combined with extensive cartilaginous metaplasia. Both tumours exhibited histological evidence of infiltrative growth, accompanied by a relatively high proliferative index within structures of chordoid appearance. The designation of the chordoid component in meningioma is very important as this subtype of meningioma exhibits a more aggressive biological behaviour and higher risk of recurrence.
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7/14. Intracranial non-Hodgkin's MALT lymphoma mimicking a large convexity meningioma.

    Primary presentation of an intradural Non-Hodgkin's lymphoma is rare. Recently these B cell lymphomas of mucosa associated lymphoid tissue (MALT) have gained acceptance as an important pathological subtype and are distinguishable from other primary CNS lymphomas that exhibit aggressive behaviour. Over the past decade a number of these lesions have been reported to resemble a meningioma both intra-operatively and radiologically. The authors outline such a case of marginal zone B cell lymphoma that clinically and radiologically resembled a meningioma. This case illustrates the rare occurrence of low grade dural B cell lymphoma and the need to consider this entity in the differential diagnosis of CNS lesions, if appropriate targeted therapy is to be administered.
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8/14. Anaplastic secretory meningioma: a case report.

    Secretory meningiomas are rare histological subtypes of meningiomas with benign biological behaviour. In this study, the authors describe the first case of secretory meningioma with many mitotic figures and brain invasion, and discuss the clinicopathologic features including immunohistochemical staining profile and ultrastructural appearance of this tumour. A case of a 54-year-old man diagnosed with an intracranial tumour located in the left frontal lobe is presented. On pre-contrast CT scans, the tumour was hypodense and the contrast enhancement was marked in the pseudo membrane. The tumour was partially removed. The histological diagnosis was secretory meningioma with many mitotic figures, a high MIB-1 labeling index and a brain invasion.
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9/14. Metastases from carcinoma of the breast involving the central nervous system.

    A general discussion is made of the incidence, biological behaviour, diagnosis and management of metastatic disease involving the central nervous system secondary to malignant disease of the breast. Illustrative case histories selected from patients treated for carcinoma of the breast in the period 1978-88 are included.
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10/14. Meningeal melanocytoma. Report of a case and review of the literature.

    A primary localized, partly encapsulated melanotic tumour broadly attached to the occipital dura and tentorium was removed from a 27 year-old woman. It recurred almost four years later in the posterior fossa and was resected again. Although grossly resembling a meningioma, the tumour lacked the histological, immunocytochemical and ultrastructural features of meningiomas and was characterized by the presence of numerous melanosomes and premelanosomes in the cytoplasm of tumour cells and macrophages and was, therefore, classified as "meningeal melanocytoma". The clinical and pathological features of this and 15 other cases in the literature are reviewed. The neoplasm, mainly occurring in the posterior cranial fossa and spinal canal, may cause neurological deficits through expanding, but non-invasive growth. Its biological behaviour is variable, and recurrence may occur after incomplete resection, but transition into malignant melanoma has not been observed. Total resection of this rare pigmented tumour arising from the pial melanocytes is recommended.
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