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A solitary plexiform neurofibroma localized on the vestibular side of the right mandible in a 37-year-old woman is described. The tumor was excised, and there was no recurrence after 2 years.
A case of neurofibroma arising from the cervical sympathetic chain and associated with ipsilateral Horner's syndrome in a patient with von Recklinghausen's disease has been presented. The clinical features and the pathology of the lesion have been discussed.
This paper is a retrospective study of 15 patients with parapharyngeal neurofibromas operated over a six year period. No patient presented with a neurological deficit. CT scans revealed a well encapsulated moderately enhancing mass in ten cases. The tumour was removed by a transcervical approach in ten cases while in five a cervical-transpharyngeal route with mandibulotomy was used; in three of the latter group the tumour was retropharyngeal. In two cases a recurrence was successfully excised. It was realized that a mandibulotomy was required when the internal carotid was displaced medially, or if the tumour extended to the base of skull or when its vertical diameter exceeded 8 cm.
Laryngeal neurofibroma (LNF) is an unusual tumour and only approximately two dozen cases have been reported previously. Isolated LNF not associated with von Recklinghausen's disease is even more unusual. According to a review of the available literature, the case presented is the first bilateral one and the first originating from the ventricles. The tumour was removed completely by microlaryngoscopy without tracheostomy. The macroscopic and light macroscopic findings are presented and a review of the literature on neurogenic tumours in the larynx is presented.
Oncogenic mutations resulting in activated Ras Guanosine Triphosphate (GTP) are prevalent in 30% of all human cancers, but not primary nervous system tumors. Several growth factors/receptors are implicated in the pathogenesis of malignant astrocytomas including epidermal growth factor (EGFR) and platelet derived growth factor (PDGF-R) receptors, plus the highly potent and specific angiogenic vascular endothelial growth factor (VEGF). A significant proportion of these tumors also express a truncated EGFR, which is constitutively activated. Our work demonstrates that the mitogenic signals from both the normal PDGF-R and EGFR and the truncated EGFR activate Ras. Inhibition of Ras by genetic or pharmacological strategies leads to decreased astrocytoma tumorgenic growth in vitro and decreased expression of VEGF. This suggests that these agents may be potentially important as novel anti-proliferative and anti-angiogenic therapies for human malignant astrocytomas. In contrast to astrocytomas, where increased levels of activated Ras GTP results from transmitted signals from activated growth factor receptors, the loss of neurofibromin is postulated to lead to functional up-regulation of the Ras pathway in neurofibromatosis-1(NF-1). We have demonstrated that NF-1 neurofibromas and neurogenic sarcomas, compared to non-NF-1 Schwannomas, have markedly elevated levels of activated Ras GTP. Increased Ras GTP was associated with increased tumor vascularity in the NF-1 neurogenic sarcomas, perhaps related to increased VEGF secretion. The role of Ras inhibitors as potential therapy in this tumor is also under study.
This case report describes a melanin-containing neurofibroma involving a spinal nerve root. Electron microscopy of the tumor shows that neoplastic Schwann cells are capable of melanogenesis. Although this capability is suggested in the literature, few reports provide ultrastructural confirmation. The likely identity between reported "cellular blue nevi of spinal nerve roots" and nerve sheath tumors is discussed. It is possible that pigmented nerve sheath tumors behave more aggressively than nonpigmented ones, although it is debatable.
We present an unusual case of solitary renal mass that histological examination following nephrectomy confirms that it was a neurofibroma. Neoplasmatic markers though, were found elevated and chromosomal analysis revealed a Karyotype similar to the one found in adenocarcinomas of the kidney.
Acute paraplegia due to the intratumour haemorrhage of spinal neurofibroma is a rare condition. We describe 2 patients with this unusual presentation. Surgical treatment was successful in both patients. These 2 cases are reported, and the literature is reviewed.
The authors report the case of a patient harbouring a spinal dural arteriovenous fistula adjacent to an intradural neurofibroma. Only the latter could be demonstrated by the diagnostic modalities employed. Such a coexistence proved misleading and two interventions were needed to cure both lesions. Only one report has appeared in the literature dealing with such a condition. The possibility of such a coexistence is stressed and mechanisms are discussed.
OBJECTIVE: To report a case of lumbar myxopapillary ependymoma in whom neuroradiological and surgical findings strongly suggested neurofibroma. CLINICAL PRESENTATION: The patient presented with a 2 year history of progressive monoparesthesia and monoparesia of his right leg. He reported having minimal fecal and urinary incontinence. INTERVENTION: Total resection of the tumor was achieved by total L1-L2 laminectomies. There was no attachment to the spinal cord and dura. CONCLUSION: In spite of contemporary sophisticated neuroradiological facilities, we may still have diagnostic difficulties in some spinal tumors.
This is a case history of a 38-year-old woman with a dumbbell-shaped C2 neurofibroma associated with right-sided classic migraine headaches (migraine with aura) and cervical trigeminal signs on the affected side. Surgical removal of the tumor was followed by resolution of the migraine headaches and persistence of the signs of cervico-trigeminal involvement.
A rare case of a plexiform neurofibroma in the cauda equina is reported. In literature single cases are described as "redundant or knotted nerve roots", but mainly without microscopic description. Our patient was complained of bilateral pain in the legs for several years. The myelogram revealed a complete block with a serpentine-shaped structure. In operation we found a elongated and redundant nerve root in the cauda equina. The biopsy showed a plexiform neurofribroma. The operative technique is described.
The article describes a case of a solitary neurofibroma of the plexus lumbosacralis causing pain and incomplete leg paresis. Complete relief was obtained by total extirpation of the tumour, a procedure which must be regarded as the method of choice. Pain and pareses in the lower extremities require differential diagnostic consideration of rare neurogenic tumours of the peripheral nervous system in the regions of the pelvis and plexus lumbosacralis. Neurological examination by a specialist is of significant importance preoperatively to determine the seat of the tumour and to assess the postoperative course.
A 4 year-old boy was referred for diagnostic reevaluation with known pulmonary valve stenosis. Physical examination revealed multiple cafe-au-lait spots, inguinal freckling and on the right side in supraclavicular region a softly, non-painful tumour. The boy showed a mild mental and language retardation. Ultrasound and MRT demonstrated supraclavicular a plexiform neurofibroma and intracranial increased intensity lesions in basal ganglia and mesencephalon. In our patient, we have diagnosed a Watson-Syndrome, the overlap and differences to neurofibromatosis type I is discussed.
A huge solitary neurofibroma of the radial nerve was resected in a 74-year-old woman. The nerve was cut across and repaired with epineural suture. The patient made an excellent recovery of nerve function. The ability for regeneration of a neurofibromatous nerve even in the elderly is outlined, and the relevant literature briefly reviewed.
Malignant transformation of a mediastinal neurofibroma, presenting for many years as a mass in the superior mediastinum and arising from the right vagus nerve, was found in a patient with long-standing von Recklinghausen's disease. Surgical management of intrathoracic neurogenic tumours, even if clinically benign, is advocated.
A 13-year-old African-American girl was admitted to the hospital for surgery. She was diagnosed with Type I neurofibromatosis at the age of 1 year after she was noted to have multiple café au lait spots. Her past medical history included a history of neurofibroma in the base of the brain, treated with radiation therapy and ventriculoperitoneal shunt, as well as a recent diagnosis of bilateral optic gliomas, treated with chemotherapy. Family history was negative for neurofibromatosis.
The application of immunohistological techniques that use antibodies highly specific for collagen allows new comparative studies in the distribution and deposition of genetically distinct types of collagens in neurofibromas. The identification of collagen type IV in basement membranes is unique and marks particularly the sheaths or cytoplasma of proliferating Schwann cells. Cells with greater degrees of atypia show increased amounts of intracellular collagen in basement membranes by immunohistologic examination, which provides an additional diagnostic tool in dermatopathology.