Cases reported "Neurofibromatoses"

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1/45. Pediatric eighth cranial nerve schwannoma without evidence of neurofibromatosis.

    Schwannomas of the eighth cranial nerve are rare in children. We report a 4 10/12 - year-old girl with no evidence of neurofibromatosis who presented with facial droop. Radiographic studies revealed a large cerebellopontine angle tumor. At surgery, the tumor was attached to the eighth cranial nerve and histologically was a schwannoma. This is the youngest reported case of unilateral eighth cranial nerve schwannoma in a patient without the stigmata of neurofibromatosis.
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keywords = nerve
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2/45. 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 = 0.71428571428571
keywords = nerve
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3/45. 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 = 31.606447059488
keywords = peripheral nerve, peripheral, nerve
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4/45. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation.

    Neurofibromatosis is rare in the general population. Its clinical manifestations are systemic and variable. The clinical presentation of cutaneous lesions is even more variable. Some patients have giant tumors in the trunk or limbs (so-called "elephant neurofibromatosis"). The pathological findings are diffuse neurofibromatosis of the nerve trunk associated with overgrowth of subcutaneous tissue and skin. The associated vascular malformations make most surgeons hesitant to address them because bleeding to death is possible if the bleeding is not well controlled. According to the authors' experience in treating this complication of neurofibromatosis, they noted that there are three key points to reducing the amount of hemorrhage to a minimal level: (1) hypotensive anesthesia, (2) preliminary sutures around the lesion, (3) ligation of the limited numbers of feeding vessels in the vascular malformation of the neurofibroma. Ligating these pedicles can decrease bleeding during resection of the neurofibroma, as demonstrated in their patient.
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ranking = 0.14285714285714
keywords = nerve
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5/45. Segmental neurofibromatosis is caused by somatic mutation of the neurofibromatosis type 1 (NF1) gene.

    Segmental neurofibromatosis (NF) is generally thought to result from a postzygotic NF1 (neurofibromatosis type 1) gene mutation. However, this has not yet been demonstrated at the molecular level. Using fluorescence in situ hybridisation (FISH) we identified an NF1 microdeletion in a patient with segmental NF in whom cafe-au-lait spots and freckles are limited to a single body region. The mutant allele was present in a mosaic pattern in cultured fibroblasts from a cafe-au-lait spot lesion, but was absent in fibroblasts from normal skin as well as in peripheral blood leukocytes. These findings prove the hypothesis that the molecular basis of segmental cutaneous NF is a mutation in the NF1 gene and that the regional distribution of manifestations reflects different cell clones, commensurate with the concept of somatic mosaicism.
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ranking = 0.78317488650639
keywords = peripheral
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6/45. 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 = 6.3212894118977
keywords = peripheral nerve, peripheral, nerve
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7/45. Ruptured internal carotid aneurysm resulting from neurofibromatosis: treatment with intraluminal stent graft.

    PURPOSE: This report shows a method of treatment for life-threatening hemorrhage due to rupture of an aneurysm in the cervical internal carotid artery caused by neurofibromatosis. methods: Ten days after delivery of healthy twins, a 28-year-old woman with known neurofibromatosis had sudden massive swelling in the left neck. After initial tracheostomy, angiography confirmed rupture of the mid cervical internal carotid artery as well as contribution to the resultant pseudoaneurysm from external carotid branches. Treatment began with coil embolization of the external carotid branches. The internal carotid lesion, a defect approximately 1 cm in length, was then closed through use of two stent grafts, each made from Palmaz stents and 3-mm polytetrafluorethylene grafts predilated to 6 mm. The neck hematoma was then evacuated surgically. RESULTS: Completion angiography and computed tomographic scanning confirmed control of the hemorrhage. The patient survived neurologically intact with the exception of cranial nerve deficits caused by the hemorrhage. The tracheostomy tube was removed 3 weeks postoperatively. Follow-up computed tomographic scanning showed a gradual decrease in the size of the cervical soft tissue and no recurrent aneurysm. CONCLUSION: Neurofibromatosis is a rare cause of aneurysmal degeneration of blood vessels. Repair of a ruptured cervical internal carotid artery aneurysm, though feasible, is difficult with stent grafts; however, this is a better option than surgical intervention in inaccessible vessels.
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ranking = 0.14285714285714
keywords = nerve
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8/45. Segmental neurofibromatosis in association with nevus sebaceus of Jadassohn.

    We describe an unusual case involving the simultaneous occurrence of segmental neurofibromatosis (Type V NF) in a patient with a large nevus sebaceus of Jadassohn in the same physical distribution. Causative mechanisms of development of these 2 genetic disorders have not been definitively linked. Factors producing these diseases probably involve similar tissues at the same point in development because both have been reported in association with central nervous system anomalies and have been classified among the neurocutaneous syndromes. This is a case of a nevus sebaceus occurring in association with and in the same physical distribution as segmental NF. These disorders most likely represent a spectrum of disease within the phakomatoses.
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ranking = 0.020289163667883
keywords = nervous system
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9/45. Localized pruritus: a presenting symptom of a spinal cord tumor in a child with features of neurofibromatosis.

    central nervous system (CNS) and spinal cord tumors are not uncommon in patients with neurofibromatosis (NF); however, it is impossible to select patients with NF who are at a particularly high risk. Localized pruritus may be a clue to the presence of a spinal cord or CNS tumor. This is the first report of an infant with features of NF, whose presenting symptom of a spinal cord tumor was localized symmetrical dermatomal itch. Moreover, we review the literature of localized pruritus in CNS and spinal cord tumors and peripheral nervous system conditions.
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ranking = 0.82375321384216
keywords = peripheral, nervous system
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10/45. Functional reconstruction of the lateral face after ablative tumor resection: use of free muscle and musculocutaneous flaps.

    BACKGROUND: Wide resection of tumors of the middle third of the face often results in complex three-dimensional defects and facial paralysis either due to removal of the facial nerve within the tumoral tissue or to extensive resection of the facial muscles. methods: We report the cases of three patients who underwent wide excision of tumors of the cheek region, operations that resulted in tissue defects and facial palsy. Defect reconstruction and facial reanimation was accomplished in one stage through functional muscle transplantation. RESULTS: Follow-up of more than 1 year showed good symmetry at rest and reanimation of the corner of the mouth in all cases, but one patient, in which the ipsilateral facial main trunk was used as motor nerve supply to the transplanted muscle, developed significant muscle contracture and binding of the cheek skin. CONCLUSIONS: Every effort should be made to optimize the functional and cosmetic outcomes of neurovascular muscle transfers through precise planning and careful execution of the intricate details of the surgical technique for muscle transplantation.
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ranking = 0.28571428571429
keywords = nerve
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