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1/123. Granular cell tumour of the ulnar nerve.

    Although granular cell tumours have been demonstrated to have a neural origin, they rarely arise in peripheral nerve trunks. We report a case of granular cell tumour of the ulnar nerve in a 51-year-old man. Though dissectable from the nerve, this intraneural tumour showed microscopic involvement of focal nerve fibres. This tumour tended to infiltrate the nerve in the same manner as a neurofibroma.
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ranking = 1
keywords = neurofibroma
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2/123. Surgical treatment of multiple neurofibromas of the ulnar nerve in segmental neurofibromatosis. Case report.

    The case of an 18-year-old man with numerous neurofibromas along his left ulnar nerve is described. The patient had a painful mass in the medial third of the internal aspect of his left forearm, and two additional symptomatic painful masses were identified during clinical examination: one in the distal portion of the retroepitroclear groove and another near the Guyon tunnel in the wrist. The main symptom was neurogenic pain; however, sensory and motor disturbances were also present. No other stigma of neurofibromatosis (NF) was found, and no cases of NF were known in the patient's family. During surgery many neurofibromas were found; the three painful neurofibromas and some of the other larger lesions were microsurgically excised. The patient's symptoms fit the criteria for segmental NF or NF5. This is a very rare form of NF characterized by lesions located in a particular area of the body.
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ranking = 12
keywords = neurofibroma
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3/123. Plexiform neurofibroma of the cauda equina. Case report.

    Plexiform neurofibroma of the cauda equina has been reported only twice previously. The authors report the first pediatric patient in whom such a tumor has been found. A 4-year-old boy presented with low-back pain that radiated bilaterally into the L-4 and L-5 dermatomes. A dermal sinus noted at the midthoracic level was surrounded by a hemangiomatous lesion. magnetic resonance imaging confirmed the presence of the dermal sinus and revealed a well-defined lumbosacral mass that showed heterogeneous intensity with irregular enhancement. Intraoperatively, a solid mass, which engulfed the entire cauda equina, could not be dissected from the roots. The dermal sinus tract, however, was excised from the thoracic spine. The patient underwent radiotherapy to control the tumor and relieve his pain. Plexiform neurofibromas of the cauda equina are characterized by an insidious and progressive clinical course. The tumor mass may engulf all the roots of the cauda equina. No plexiform neurofibroma of the cauda equina has been reported to be associated with neurofibromatosis Type 1. The authors assume that the thoracic-level dermal sinus observed in this child was an incidental finding.
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ranking = 8
keywords = neurofibroma
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4/123. Molecular analysis of malignant triton tumors.

    Triton tumors are rare variants of malignant peripheral nerve sheath tumor (MPNST) with muscle differentiation, often seen in patients with neurofibromatosis 1 (NF1). Individuals affected with NF1 harbor mutations in the NF1 tumor suppressor gene and develop neurofibromas and MPNSTs. The NF1 gene is expressed in schwann cells and its expression is lost in schwannian neoplasms, suggesting a role in malignant development. Separately, there is evidence that p53 suppressor gene mutations are involved in MPNSTs. To determine the role of the NF1 and p53 genes in the development of the malignant Triton tumor we examined 2 such tumors, 1 from a 3-year-old boy without clinical manifestations of NF1 and another from a 24-year-old man with NF1. Histological analysis of these tumors showed both neural and muscle differentiation with S-100 and desmin immunoreactivity, respectively. Reverse transcribed rna polymerase chain reaction (RT-PCR) of NF1 mRNA showed NF1 expression in the sporadic tumor. Strong nuclear immunoreactivity for p53 was observed throughout the malignant population in both tumors. This was confirmed by loss of heterozygosity for p53 in the non-NF1 patient, suggesting that p53 is involved in both hereditary and sporadic Triton tumors. The finding of preserved NF1 gene expression in the non-NF1-related Triton tumor suggests that different genetic events predispose to the development of this rare neoplasm in sporadic cases.
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ranking = 2
keywords = neurofibroma
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5/123. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases.

    AIMS: Angiosarcomatous differentiation represents the least common form of heterologous differentiation in malignant peripheral nerve sheath tumours (MPNST), and is seen most frequently in patients with neurofibromatosis type 1. More rarely, it has been reported in patients without stigmata of neurofibromatosis, or in benign nerve sheath tumours and peripheral nerves. This study was undertaken to confirm this rare association. methods AND RESULTS: Four cases of angiosarcoma arising in a peripheral nerve, in a long-standing schwannoma and in two MPNST are described. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. An intraneural high-grade epithelioid angiosarcoma arose in the left posterior tibial nerve of a 78-year-old man, a well to moderately differentiated angiosarcoma was seen in an ancient schwannoma of the lateral neck in a 73-year-old women, and an angiosarcoma of varying grades of differentiation developed in a recurrent MPNST in the thigh of 38-year-old man. In addition a high-grade MPNST in the axillary region of a 30-year-old man showed foci of heterologous high-grade angiosarcomatous differentiation. The neural and endothelial lines of differentiation were confirmed in each case by positive immunohistochemical staining for neural and endothelial markers, respectively. In all cases tested, the neural differentiated cells stained immunohistochemically positive for antibodies against vascular endothelial growth factor. CONCLUSIONS: This study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and malignant peripheral nerve sheath tumours.
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ranking = 2
keywords = neurofibroma
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6/123. Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis. Case report.

    Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. patients with NF5 present with cafe-au-lait macules, freckles, and/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain. neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.
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ranking = 8
keywords = neurofibroma
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7/123. Neoplasm as a cause of brachial plexus palsy in neonates.

    Two patients with neonatal onset of arm weakness resulting from neoplastic involvement of the brachial plexus who were initially considered to have obstetric brachial plexus palsies are reported. The first patient was a 7-day-old female who presented with a left supraclavicular mass that was first detected at 2 days of age and left proximal arm weakness. The weakness involved the whole arm within 3 days. The mass was a malignant rhabdoid tumor. The second patient was a 28-month-old male who presented with slowly progressive right arm weakness, which began at 3 weeks of age, and episodes of scratch marks on the arm that began at 4 months of age. magnetic resonance imaging revealed a plexiform neurofibroma of the brachial plexus. The features that are suggestive of a brachial plexus palsy caused by a neoplasm rather than of obstetric brachial plexus palsy include the following: the onset of weakness after the first day of age, with a progressive course; a history of a normal delivery and birth weight; the absence of signs of a traumatic injury or injuries; the appearance before 7 days of age of a growing supraclavicular mass without radiographic evidence of a clavicular fracture; and recurrent scratch marks on the weak arm.
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ranking = 1
keywords = neurofibroma
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8/123. A rapidly growing benign intrathoracic neurofibroma after lung lobectomy.

    A 67-year-old male underwent a right upper lung lobectomy for lung cancer in January 1993. Follow-up chest x-rays revealed a progressive and rapidly growing intrathoracic mass in the right thorax. The mass, however, did not resemble a tumor recurrence, and the patient complained only of shortness of breath. Computerized tomography and magnetic resonance imaging confirmed the presence of the intrathoracic mass and its associated compression of the residual lung. A right thoracotomy was performed in January 1998, and a mass found arising from the sympathetic nerve trunk was resected. Microscopic examination revealed stellate or spindle-shaped cells in myxoid stroma with sparsely distributed collagen fibers. Immunohistochemically, the cells were positive for neuron-specific enolase, and the tumor was identified as neurofibroma. The patient did not suffer from von Recklinghausen's disease, and there was no family history of the disease. After resection of the neurofibroma, the compressed lung was able to re-expand, and the patient's shortness of breath disappeared. At one year postoperative, the patient remains well, and there is no evidence of recurrence.
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ranking = 6
keywords = neurofibroma
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9/123. Deep-seated segmental neurofibromatosis without cafe au lait spots.

    Segmental neurofibromatosis is a rare disease characterized by neurofibromas with or without cafe au lait spots localized to one segment of the body. The majority of reported cases have had cutaneous neurofibromas, and patients with deep involvement have rarely been described. We report on two patients with deep-seated segmental plexiform neurofibromatosis and review the literature. All reviewed cases including the present two had no cafe au lait spots, axillary freckling, Lisch nodules, family history or malignant progression of disease. Differential diagnoses from neuro-fibromatosis 1 (von Recklinghausen disease) and malignant peripheral nerve sheath tumor are important for genetic counseling and avoiding overtreatment.
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ranking = 8
keywords = neurofibroma
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10/123. Bilateral posterolateral approach to mirror-image C-2 neurofibromas. Report of four cases.

    Multiple nerve root tumors are usually present in patients afflicted with neurofibromatosis Type 1. Although rare, upper cervical mirror-image neurofibromas have been reported in the medical literature, and their surgical management has been addressed in several reports; however, little has been mentioned or is known regarding upper cervical or craniocervical stability following resection of these tumors. In this report the authors describe four cases of large mirror-image C-2 neurofibromas resected in two stages via the posterolateral approach. One patient presented with acute neurological deterioration after a biopsy sample had been obtained, whereas the other three presented with gradual onset of lower-extremity weakness over several months. The time interval between the first and second decompressive surgery ranged from 10 days to 12 weeks. There were no surgery-related complications, and all patients recovered motor function in their extremities. During a follow-up period of 16 to 36 months, there was no clinical or radiological evidence of upper cervical spine instability. Although the series is too small to draw any definitive conclusions, in the authors' experience the posterolateral approach provides a direct route for the successful surgical treatment of bilateral craniocervical nerve root tumors without destabilizing the upper cervical segments.
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ranking = 7
keywords = neurofibroma
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