Cases reported "Nervous System Neoplasms"

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1/73. Autonomic nerve tumour with skeinoid fibres: ultrastructure of skeinoid fibres examined by quick-freezing and deep-etching method.

    A case of gastrointestinal autonomic nerve tumour with skeinoid fibres (SFs) of the jejunum in a 79-year-old Japanese man, was examined by the quick-freezing and deep-etching (QF-DE) method. The tumour consisted of spindle cells with immunohistochemical reactions for vimentin, NSE and CD34. Electron microscopically, features of the neural cells of the myenteric plexus were observed. The QF-DE method demonstrated intercellular meshwork structures, consisting of thin filaments (7-15 nm), with granular deposits. Fully developed parts of the deposits formed nodular aggregates composed of irregularly surfaced thick fibrils (30-48 nm) with a tendency to linear arrangement (SFs). We detected many interconnecting thin filaments (ICTFs) between the SFs, which were pre-existing components in the meshwork, avoiding the granular deposits. The focal thickening formed by the connection between SFs and ICTFs revealed a periodicity typical of SFs (33-45 nm). We conclude that SFs are formed by decoration of the granular deposits along pre-existing intercellular meshwork structures.
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2/73. Cervical sympathetic chain schwannomas masquerading as carotid body tumors.

    Cervical sympathetic chain (CSC) schwannoma is a rare neurogenic tumor that can mimic a carotid body tumor (CBT). Two male patients aged 33 and 49 years old were referred for a cervical mass thought to be a CBT. Both patients were found to have an asymptomatic, pulsatile, nontender mass located at the level of the angle of the mandible. Both patients were neurologically asymptomatic and cranial nerves were normal. Both patients underwent neck exploration and resection of the mass involving the CSC. Postoperatively, a Horner's syndrome was present. Histopathology confirmed both tumors to be schwannomas of the CSC. At follow-up, both patients are asymptomatic with no recurrence. The main imaging criterion to differentiate a CBT from a CSC schwannoma is the lack of hypervascularity of the latter. Malignant transformation is extremely rare. Tumor excision gives excellent results with no local recurrence. Horner's syndrome is an expected postoperative complication.
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3/73. carpal tunnel syndrome due to an intraneural perineurioma in a 2-year-old child.

    We report a 2-year-old girl with carpal tunnel syndrome due to a large intraneural perineurioma that required resection and nerve reconstruction.
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4/73. Ancient schwannoma masquerading as a thyroid mass.

    Schwannomas are benign, encapsulated nerve sheath cell neoplasms. Cervical sympathetic chain (CSC) schwannomas are rare, with less than 50 cited cases in the literature. CSC schwannomas may mimic a number of parapharyngeal masses. We report a rare variant, "ancient" schwannoma, which presented cytologically and radiologically as a thyroid mass. This is the first report of a CSC schwannoma mimicking a thyroid mass and the first report of an ancient schwannoma of the CSC.
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5/73. Neural fibrolipoma of the foot.

    Neural fibrolipoma is a benign tumor comprised of hypertrophied fibrofatty tissue with intermixed nerve tissue. We present the case of a neural fibrolipoma of the foot that underwent above-ankle amputation and review the characteristic features of this unique form of localized gigantism.
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6/73. An unusual presentation of a rare nerve cell tumour.

    We present a rare case of a schwannoma in a pre/para-aortic position resembling a thrombosed saccular abdominal aortic aneurysm.
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7/73. Gastrointestinal autonomic nerve tumors: a surgical point of view.

    AIM: Gastrointestinal autonomic nerve tumors are uncommon stromal tumors of the intestinal tract. Their histological appearance is similar to that of other gastrointestinal stromal tumors. We report two cases and performed an analysis of the literature by comparing our findings with the available case reports in the medical literature. methods: Two patients were admitted with abdominal tumor masses. One occurred in the stomach with large multiple liver metastases and the second originated in Meckel's diverticulum. The latter site has never been reported previously. Both patients underwent surgery. In one patient gastrectomy, right liver resection and colon transversum resection were performed to achieve aggressive tumor debulking. In the other patient the tumor bearing diverticulum was removed. RESULTS: Postoperative recovery of both patients was uneventful. Histological examination, immunohistochemical analysis and electron microscopy revealed the diagnosis of a gastrointestinal autonomic nerve tumor. The patient with the tumor in Meckel's diverticulum died 6 mo after surgery because of pneumonia. The patient with liver metastases have been alive 13 years after initial tumor diagnosis and 7 years after surgery with no evidence of tumor progression. In light of our results, we performed a thorough comparison with available literature reports. CONCLUSION: Radical surgical resection of gastrointestinal autonomic nerve tumors seems to be the only available curative approach to date, and long term survival is possible even in large metastasized tumors.
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8/73. Perineural invasion of squamous cell carcinoma of the lip with occult involvement of the infra-orbital nerve detected by PET-CT and treated with MRI-based IMRT: a case report.

    A 51 year old male with a history of right facial numbness developed progressive upper lip swelling for one year, but an MRI of the head was unremarkable. A wide local excision of the upper lip was performed and pathology revealed a 1.7 cm mass, poorly differentiated squamous cell carcinoma with perineural invasion. Surgical margins were free of tumor. Two months postoperatively, a hybrid PET-CT of the whole body was performed due to the persistent right facial numbness. The CT portion identified an equivocal lesion at the base of the right orbit correlating to the right infraorbital nerve. However, the PET-CT image revealed avid uptake in this location suggesting perineural invasion which was confirmed with biopsy of the right infraorbital nerve demonstrating carcinoma. Subsequently, the patient was treated with Intensity Modulation radiation Therapy (IMRT) using MRI fusion for proper delineation of the right infraorbital nerve to its origin in the base of skull. This case exemplifies the superiority of hybrid PET-CT over CT or MRI alone in head and neck imaging which can lead to significant impact on management for patients with head and neck cancer.
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9/73. ganglioneuroma : primary tumor or maturation of a suspected neuroblastoma?

    ganglioneuroma is a benign neurogenic tumor. These tumors are originating from neuroepithelium along sympathetic ganglia. Main localization is the mediastinum in children older than 10 years. An association with malignant neuroblastoma is rarely observed and it still remains a topic under current discussion. We describe the clinical course of a 17 year-old female patient with a large presacral mass causing amenorrhoea and weight loss. Eleven years before presentation, an incidental urine test showed an elevation of vanillylmandelic acid (VMA) and homovanillic acid (HMA) and a neuroblastoma was suspected. However, further investigations showed no tumor and the test results turned out to be normal within 1 year. Now, a malignant neurogenic tumor was again suspected, but a CT-guided biopsy revealed a benign tumor. The mass was originating from the left sacral nerve roots. A tumor resection via a dorsal approach was performed. Final histology showed a differentiated ganglioneuroma. This is, to our knowledge, the first report describing a patient with elevated VMA/HMA and suspected neuroblastoma who developed a ganglioneuroma 11 years later. The association of ganglioneuroma and neuroblastoma and the abnormal urine tests pointing toward a neuroblastoma 11 years ago remains unclear and the possible answers are discussed in our report.
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10/73. Aggressive basal cell carcinoma with invasion of the parotid gland, facial nerve, and temporal bone.

    Aggressive variants of basal cell carcinoma (BCC), such as infiltrating, morpheaform, and basosquamous types, are associated with invasion of underlying tissues and are often difficult to treat.1 BCCs located in embryonic fusion planes, such as the periauricular region, are thought to exhibit deep extension and, subsequently, high recurrence rates, although this theory has been challenged and remains controversial.2-4 Despite the known features of aggressive BCC, parotid gland invasion and temporal bone and facial nerve involvement are rarely reported occurrences. We describe two patients with morpheaform BCC in the periauricular region demonstrating direct invasion of the parotid gland and concomitant facial nerve involvement. These patients require complex surgical management, as highlighted in this report.
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