1/39. Fibromatosis.Fibromatosis represents a diverse group of fibroproliferative tumours. Their behaviour and pathological qualities are situated in an intermediate position between benign and malignant disease. The following represents the pathological and radiological presentation of a 29-year-old female with fibromatosis.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/39. Multiple anterolateral cervical meningoceles associated with neurofibromatosis.In neurosurgical practice, the term 'spinal meningocele' is generally used to describe a congenital spinal malformation including protrusion of the spinal meninx from the congenitally dysraphic vertebrae. Although non-dysraphic meningocele is very rare, it is usually associated with neurofibromatosis or Marfan's syndrome in the literature. Thoracic and/or lumbar spinal levels are the most common localization. Anterolateral localization of meningocele is very rare in the cervical region. Operative treatment is indicated if the lesion is symptomatic. Detailed radiological assessment of the meningocele is necessary since it may be associated with neuroma in the sac. If the neuroma is found in the meningocele, an anterior surgical approach should be considered for the treatment of both of the lesions.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
3/39. Vertebral scalloping in neurofibromatosis.The classical abnormalities of von Recklinghausen disease (VRD) or neurofibromatosis are well recognized. However, vertebral scalloping and gauge defects of the anterior and lateral aspects have not been reviewed, to the authors' knowledge. case reports of 3 patients with vertebral scalloping are presented. The classical posterior scalloping from dural ectasia is also discussed. These concavities can occur either in isolation, or contiguous to a tumor. Recognition of them can aid in the diagnosis and evaluation of patients with suspected VRD.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
4/39. Malignant triton tumor in the thoracic spine.We present a 15-year-old patient diagnosed with peripheral neurofibromatosis (NF-1), who was admitted with paraparesis caused by a large intrathoracic tumor with an intracanalicular component that affected the spinal cord. After surgery his condition improved, but a year later he suffered a relapse and died. Histologically the tumor was diagnosed as malignant with neurogenic and myogenic differentiation ("malignant triton tumor"). Malignant triton tumors (MTT) are infrequent; those found in the head and neck and the upper or lower extremities have a better prognosis than those in the retroperitoneum, buttock, or trunk. It is not clear whether this variation is due to a difference in tumor grade, stage, or resectability, or whether it is a consequence of therapy.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
5/39. Segmental neurofibromatosis-induced spinal cord compression. Case report.Spinal segmental neurofibromatosis (NF) is a rare entity. To date, patients in reported cases of segmental NF (or NF5) have harbored neurofibromas involving the peripheral nerves only. The author reports a rare case of segmental NF that caused spinal cord compression in a 40-year-old woman who presented with a 6-month history of intercostal neuralgia. Examination revealed mild lower-extremity weakness and dysesthesia in the right-sided T-9 dermatome. magnetic resonance imaging revealed three neurofibromas involving the T-9 region, which were excised, and the patient's neuralgic pain was resolved postoperatively. Traditionally, it has been believed that segmental NF involved only the peripheral nerves. The present case illustrates that although rare, spinal cord compression can also occur in patients with segmental NF.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
6/39. A case of neurofibromatosis with severe osseous disease of the thoracic spine.A case of neurofibromatosis in a 7-year-old boy from greenland is presented. The patient suddenly developed a severe thoracic scoliosis because of a total luxation of the upper thoracic vertebrae caused by erosion from soft tissue tumours in the region. tomography revealed the bone changes, and CT whole-body scanning showed the extent of the soft tissue tumours and also the destruction of the vertebrae. The diagnosis was verified by biopsy.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
7/39. Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
8/39. Metastatic spinal neurofibrosarcoma.Neurofibrosarcomas are rare tumors usually arising in somatic soft tissues or peripheral nerves. Four cases of metastatic neurofibrosarcoma to the spine have been reported before. The current case is unusual because of the presence of two distinct, metachronous spinal metastasis and lung metastasis. A 30-year-old woman with neurofibromatosis and a history of previous neurofibrosarcoma resection presented with back pain. Radiologic evaluation revealed a lytic lesion of the eleventh thoracic vertebra. A transthoracal corpectomy, reconstruction by Harms' cage and posterior instrumentation, and fusion were carried out. After the completion of adjuvant chemotherapy, a solitary pulmonary nodule was detected. Shortly after resection of the metastatic pulmonary nodule, the patient complained of acute onset low-back pain. Radiologic assessment revealed another lytic lesion in the L5 vertebra after 6 months. Again, a corpectomy, anterior and posterior instrumentation, and fusion were carried out. Eight months after the second spinal resection, another solitary pulmonary metastasis was diagnosed and resected. The patient's health status suddenly deteriorated 26 months after the initial spinal metastatectomy, and she died. Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis. The current patient survived 50 months after the initial resection of a forearm neurofibrosarcoma. Despite achieving local control, she died due to systemic recurrence. Prolonged survival with the help of chemo- and radiotherapy justifies our aggressive surgical strategy for the treatment of spinal metastasis in order to achieve neurologic cure and spinal stability.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
9/39. Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves.Schwannomas from cranial nerves and spinal roots are most often benign, malignant schwannomas being uncommon in this location. This report describes a unique case of multiple cellular and malignant schwannomas of the cranial and spinal nerves in a patient with features of neurofibromatosis 2. The tumors were arising from left optic, bilateral oculomotor, trochlear, abducent and vestibular nerves, the left facial and the spinal lumbar nerve roots. The tumor arising from the right trochlear nerve was seen excavating into the hippocampus and the left vestibular nerve into the medulla. In addition, the patient had nodular schwannomas adherent to the inferior surface of the optic chiasm and the cerebellum away from the cranial nerves. Hyperplastic schwannosis was noted in perivascular spaces of thalamus, cerebellum and hippocampus on both sides away from the main lesions. The immunohistochemical and ultrastructural profiles of the tumors suggest that neurofibromas and schwannomas are probably not distinct tumors but lie within a spectrum that differs histologically depending on the predominant cell type.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
10/39. quadriplegia in a 10 year-old boy due to multiple cervical neurofibromas.STUDY DESIGN: A case report of a child with quadriplegia as a result of neurofibromatosis type I who had cervical laminectomy for spinal cord decompression followed by occipitocervical instrumentation is described. OBJECTIVES: To describe the consequences of severe neurofibromatosis type I and an effective surgical technique of occipitocervical instrumentation. SUMMARY OF BACKGROUND DATA: Neurofibromatosis type I is one of the most commonly inherited genetic disorders in the human population. Extensive intraspinal involvement by neurofibromas can cause significant distortion of normal spinal structure as well spinal cord compression. Extensive laminectomy (with subsequent risk of postsurgical kyphosis) is often required for adequate decompression of the spinal cord. methods: The clinical and radiographic presentation of a child with severe neurofibromatosis type I resulting in quadriplegia is described. The severe neurologic deficit was caused by compression of the spinal cord by intraspinal neurofibromas. Extensive laminectomy was required to adequately decompress the spinal cord. Occipitocervical fusion from the occiput to C6 was done to stabilize the spine and prevent future kyphosis. RESULTS: decompression of the spinal cord led to complete neurologic recovery, and instrumentation of the cervical spine was successful in preventing the development of postlaminectomy kyphosis in this pediatric patient. CONCLUSIONS: The reported case emphasizes the need for treating acute neurologic symptoms caused by spinal cord compression in neurofibromatosis type I as well as addressing the future risk of spinal deformity following laminectomy.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
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