23 research outputs found

    Primary temporal bone angiosarcoma: a case report.

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    We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.Peer reviewe

    Solitary fibrous tumour of the vagus nerve.

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    We describe the complete removal of a foramen magnum solitary fibrous tumour in a 36-year-old woman. It originated on a caudal vagus nerve rootlet, classically described as the 'cranial' accessory nerve root. This ninth case of immunohistologically confirmed cranial or spinal nerve SFT is the first of the vagus nerve

    Acquired tonsillar herniation and syringomyelia after pleural effusion aspiration: case report.

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    peer reviewedOBJECTIVE: We present a case of brachial plexus avulsion and reconstructive surgery with cerebrospinal fluid leak between the cervical subarachnoid space and the pleural cavity responsible for tonsillar herniation and syringomyelia. CLINICAL PRESENTATION: A 17-year-old man presented with headaches when he was positioned upright, simultaneously with a persistent right pleural effusion for about 4 months after reconstructive surgery for a right brachial plexus avulsion. In addition, the headaches had worsened considerably after two aspirations of the pleural effusion. Magnetic resonance imaging (MRI) demonstrated signs of chronic intracranial hypotension and tonsillar herniation with a presyrinx cavity from vertebral level C1 to C7. None of those abnormalities were seen on the MRI scan obtained a few days after the initial trauma 7 months previously. Plexus brachial MRI confirmed the presence of a cerebrospinal fluid leak between the avulsed root of C8 and the pulmonary apex. INTERVENTION: The leak was treated by surgical closure of the dural tear of the C8 root. Postoperatively, the patient's headaches immediately resolved, and MRI 4 months later showed resolution of cerebellar tonsil herniation and regression of the syrinx. CONCLUSION: Resolution of acquired tonsillar herniation and syringomyelia can be achieved by closure of the dural tear responsible of the cerebrospinal fluid leak

    Clinical case of the month. Miller-Dieker (lissencephaly) syndrome

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    We present the case of a 10-year-old boy who presents with a severe epilepsy resistant to medical treatment in the context of a Miller-Dieker syndrome. This patient underwent the implantation of a pneumogastric nerve stimulator. We describe the patient's clinical history and the main characteristics of lissencephaly syndrome

    Multifocal choroid plexus papilloma: a case report.

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    BACKGROUND: Multiple choroid plexus papillomas (CPPs) are rare. Usually, they correspond to villous hypertrophy or metastasis occurring during cerebrospinal dissemination. Multiple CPPs have rarely been reported as synchronous tumors. CASE REPORT: Three synchronous CPPs were resected in a 59-year-old female 6 years after their first imaging description. Pathology showed mucus-producing CPP in all 3, 1 of the 3 presenting some signs of atypia. No p53 or hSNF5/INI1 mutation, or signs of polyoma viruses infection were found. CONCLUSION: Although no clear cause for the multifocality was found, the simultaneous presence of the three tumors and their benign histology suggest that they were synchronous and not metastatic. The issue of differentiating synchronous CPPs from metastatic CPP is discussed
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