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1/255. Surgically created fourth-third cranial nerve communication: temporary success in a child with bilateral third nerve hamartomas. Case report.

    Shortly after birth, an otherwise healthy infant developed eye deviation and ptosis due to a hamartomatous lesion of the interpeduncular segment of the right oculomotor nerve. The left nerve became similarly involved when the child was 1.5 years of age. Direct nerve repair was not possible. Instead, the trochlear nerve was divided and its proximal end was attached to the distal end of the third nerve. Elevation of the upper eyelid and partial adduction of the eye developed gradually over the ensuing 3 to 5 months. Both functions were lost after an additional 2 months, presumably as a result of tumor recurrence or neuroma formation. This case report shows that surgically created fourth-third cranial nerve communication is feasible and may merit consideration under similar circumstances.
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2/255. Follicular dendritic cell tumor of the parapharyngeal region.

    BACKGROUND: Follicular dendritic cell (FDC) tumors are rare. A majority of the reported cases were confined to the lymph nodes. We report a case of FDC tumor occurring in the parapharyngeal region in a 45-year-old woman. methods: Characteristic histopathologic features of the excised primary and recurrent parapharyngeal tumors in conjunction with immunohistochemistry and electron microscopy helped us to arrive at a diagnosis of FDC tumor. RESULTS: Histopathology of primary excision revealed a lobulated tumor with a suggestion of ill-defined whorls. The most striking feature was regular occurrence of aggregates of lymphocytes within the tumor, especially around the blood vessels. The anatomic location together with the histology indicated the possibilities of either a meningioma, a salivary gland tumor, or a nerve sheath tumor. Immunostains for cytokeratin (CK), S-100 protein, and smooth muscle actin (SMA) were negative. However, the tumor cells showed strong immunoreactivity for epithelial membrane antigen (EMA) and vimentin. A diagnosis of parapharyngeal meningioma appeared to be the closest possibility. One year later, the patient developed a recurrence at the same site. A reexcision showed an identical tumor with an additional feature of lymphatic embolization and angioinvasion. A review of the entire case with further immunoreactivity for CD21 and CD35 confirmed the diagnosis of FDC. CONCLUSIONS: Follicular dendritic cell tumor has distinctive morphologic features and immunohistochemical profile. It is also characterized by considerable potential for recurrences.
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3/255. The place of irradiation in the treatment of malignant tumors of the salivary glands.

    1. radiation therapy is not indicated after surgical removal with adequate margins of low-grade tumor. 2. radiation therapy is indicated with a) Inadequate surgical margins in low-grade tumors b) All high-grade tumors c) All recurrent malignant tumors 3. Irradiation of nerve pathways is indicated with demonstrated nerve and perineural invasion and/or with adenoicystic carcinoma. 4. Irradiation of the entire ipsilateral neck is indicated a) Wtih high-grade tumors unless radical neck dissection shows negative nodes b) In the place of radical neck dissection.
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4/255. A leptomeningeal metastasis revealed by sciatica.

    Meningeal metastatic disease usually occurs as a complication of a brain tumor and is exceptionally isolated in patients with solid tumors. We report the case of a 74-year-old woman admitted for mechanical S1 sciatica refractory to drug therapy. She had been treated for breast cancer three years earlier. Physical findings were pain upon hyperextension of the lumbar spine and absence of the ankle jerks. Analysis of cerebrospinal fluid sampled during an intrathecal glucocorticoid injection showed 1 g/L of protein and 11 normal cells per mm3. Grade 3 L5-S1 spondylolisthesis was seen on plain radiographs, computed tomography scans, and magnetic resonance imaging scans. At that point, the patient developed sphincter dysfunction and motor loss in the left lower limb in the distribution of several nerve roots. Findings were normal from a myelogram and a magnetic resonance imaging study of the brain. A repeat cerebrospinal fluid analysis showed 1.1 g/L of protein and 5 cells/mm3. Because of the discrepancy between the clinical and imaging study findings, the patient was transferred to a neurology department. A third cerebrospinal fluid study showed numerous adenocarcinoma cells, and a repeat magnetic resonance imaging demonstrated a mass in the dural sac opposite L2. A program of monthly intrathecal methotrexate injections was started. A fatal meningeal relapse occurred eight months later. CONCLUSION: This case shows that a leptomeningeal metastasis can cause isolated nerve root pain, and demonstrates the diagnostic value of magnetic resonance imaging and cerebrospinal fluid cytology in patients with atypical symptoms, particularly when there is a history of malignant disease.
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5/255. Rapid enlargement and recurrence of a preexisting intrasellar craniopharyngioma during the course of two pregnancies. Case report.

    Enlargement of preexisting pituitary adenomas during pregnancy is well documented, but this phenomenon is unusual for nonendocrine pituitary tumors such as craniopharyngiomas. Only six cases of craniopharyngioma have been reported as presenting during pregnancy. The authors describe a 19-year-old woman who presented with amenorrhea and galactorrhea caused by an intrasellar mass. Seven months later, when she was 20 weeks pregnant, the patient developed sudden visual dysfunction. Emergency transsphenoidal surgery was performed to restore visual function, and the tumor was found to be a craniopharyngioma. The patient had spontaneous labor and delivered a healthy infant at term. The tumor recurred 4 years later, during her second pregnancy, and was again entirely removed via a second transsphenoidal approach. She again had a normal term delivery. During the 5-year follow-up period she has demonstrated no endocrinological or visual dysfunction. Control magnetic resonance images have revealed no recurrence of the tumor. The transsphenoidal approach seems to be the safest procedure to use during pregnancy to achieve an immediate optic nerve decompression and to preserve pituitary function.
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6/255. The bifrontal olfactory nerve-sparing approach to lesions of the suprasellar region in children.

    Suprasellar masses in children include lesions such as craniopharyngiomas and germ cell neoplasms. The difficult location of these lesions and their proximity to important neural and vascular structures pose challenges to resection. We operated on 14 patients using a bifrontal craniotomy with removal of both orbital rims to provide access to suprasellar masses, even those with significant extension into the third ventricle. A complete resection was possible in 8/14 patients and 8/10 craniopharyngiomas. In 13 patients, the optic nerves were identified and preserved, and vision was stable or improved postoperatively. Postoperatively, 1 patient with hydrocephalus developed a CSF leak which was successfully treated with a ventriculoperitoneal shunt. No patient had a cosmetic defect related to orbital rim removal. The bifrontal approach used here enhanced the exposure of the suprasellar region and minimized manipulation of the optic apparatus and the carotid arteries. It may be used alone, or in conjunction with other approaches, to resect lesions in the suprasellar region.
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keywords = nerve
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7/255. Malignant peripheral nerve sheath tumour in the maxilla associated with von Recklinghausen's disease.

    Malignant transformation of neurofibromatosis is one of the most serious complications of von Recklinghausen's disease (VRD). The most common associated malignancy is the malignant peripheral nerve sheath tumour (MPNST). Few cases of MPNST associated with VRD in the maxillary region have been reported. This report describes a rare case of MPNST in the maxilla and the aggressive nature of MPNST associated with VRD.
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keywords = nerve
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8/255. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.

    A demarcated 6 x 5 cm right paravertebral tumor at the level of T6 in a 39-year-old male was removed surgically. Histologically, the tumor consisted of monomorphous benign-looking, low-cellular spindle cells embedded in desmoplastic stroma. Ten years later, the tumor recurred locally with metastasis to systemic organs, including the occipital skin. Malignancy was histologically evident by the increased cellularity, cellular atypia and mitotic activity. The patient died of respiratory failure at the age of 49. Retrospectively reviewed, the primary lesion was low-grade fibrosarcoma-like spindle cell tumor, with secondary transformation into a highly malignant form. The differential diagnoses included sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma and malignant peripheral nerve sheath tumor. Immunohistochemically, the spindle cells in the primary and recurrent tumors consistently expressed epithelial membrane antigen, vimentin, type 4 collagen and laminin. The tumor cells in the present case showed a differentiation toward perineurial cells, which are normally positive for these immunohistochemical markers. Hence, the appropriate diagnostic term should be 'malignant perineurioma', a subtype of malignant peripheral nerve sheath tumor.
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9/255. Uncommon otological manifestations of nasopharyngeal carcinoma.

    Nasopharyngeal carcinoma (NPC) is significant for the otologist although the nasopharynx is located outside the anatomical confines of the ear. Middle-ear effusion resulting from NPC is well-known. There are however, other less common ear symptoms of NPC that many physicians are not sufficiently aware of. A personal series of patients with NPC presenting with uncommon symptoms relating to the ear is presented. These include NPC presenting as a) haemotympanum b) a peri-auricular mass c) referred sensation to the ear d) blocked ear e) barotrauma f) an ear polyp g) sudden sensorineural hearing loss. These symptoms may pose diagnostic difficulties, resulting in the diagnosis of NPC being delayed. It was concluded that a high index of suspicion for NPC is warranted in high risk patients presenting with unexplained otological symptoms.
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ranking = 0.0016553698142838
keywords = block
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10/255. Stereotactic radiosurgery. IX. craniopharyngioma: durable complete imaging responses and indications for treatment.

    The previous literature on radiosurgical treatment for craniopharyngioma is sparse, because the involvement of the radiosensitive optic chiasm within the target volume (in most cases) precludes safe high single dosage. The relocatable frame, introduced at St Bartholomew's Hospital in 1989, utilizes the dosimetric advantages of stereotactic isodosimetry to treat with the safer fractionated therapy; this may well be the most advantageous methodology for larger and critically situated tumours around the chiasm. We believe that radical high dose radiosurgery (either single dose or fractioned) has a role in the treatment of selected craniopharyngiomas and our first six patients treated are presented here. There were two patients with discrete, small, solid lesions and both achieved complete responses that maintain at 1-2 years. The other four patients are more difficult to assess: one patient was treated for disease within the clivus and there is no evidence of disease progression at 18 months; one child, who was treated by fractionated radiosurgery to a solid component of a complex partly solid, partly cystic craniopharyngioma enveloping the chiasm, suffered tumour progression and died; one patient died of a hemisphere cerebovascular accident, which we believe was unrelated to the therapy, and one patient suffered malignant change 1 year after radiosurgery (a time point that we consider too early to ascribe to the radiation from this therapy). We discuss the indications for this technology in the multi-disciplinary therapy of this complex disease. It seems clear that, for low lying lesions, well below the optic apparatus, radiosurgery has an important role (possibly a primary radical radiotherapeutic role for small bulk solid masses). Where there is an inoperable solid tumour enveloping the chiasm the dosimetric advantages of the x-knife (10% internal dose gradient versus the 100% internal dose gradient of the gamma knife) plus the fractionation facility using the relocatable frame, argue for the x-knife (linear accelerator) as being the optimal radiosurgery system. These arguments also apply to acoustic neuroma therapy and preservation of hearing, the other clinical situation where a radiosensitive special sensory nerve lies within the target volume.
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