1/151. Neurofibromatosis type 2 with multiple primary brain tumors in monozygotic twins.BACKGROUND: Although monozygotic twins with neurofibromatosis complicated by brain tumors rarely have been reported, none of them fulfilled the diagnostic criteria for neurofibromatosis type 2 (NF2). METHOD: We describe here the first pair of monozygotic twins with NF2, and the result of the molecular analysis of their NF2 gene. RESULTS: One of the brothers (Case 1) developed tetraparesis and cerebellar truncal ataxia at age 12. He had no skin lesions. Radiological examinations revealed, at one time or another, bilateral vestibular schwannomas, a foramen magnum meningioma, five supratentorial meningiomas, and multiple spinal cord tumors. He underwent three operations over a 10-year period to remove tumors. The patient is now 23 years old and is in college. Although asymptomatic when examined at age 12, CT scan revealed that his brother (Case 2) also had multiple brain tumors, including meningiomas, schwannomas, and multiple spinal tumors. Tumors were removed in eight operations over a 10-year period. The patient is now deaf and confined to a wheelchair. An identical nonsense mutation caused by a C to T transition (C169) in a CpG dinucleotide of the NF2 gene was identified in both patients. CONCLUSION: These results led us to speculate that dissimilarities with respect to time of appearance, distribution, and extent of symptoms and tumors between the twins were dependent on the influence of other genetic factors.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/151. patients with spinal cord cavernous malformations are at an increased risk for multiple neuraxis cavernous malformations.OBJECTIVE: To determine the prevalence of multiple neuraxis cavernous malformations in patients who initially presented with intramedullary spinal cord (IMSC) cavernous malformations without knowledge of cavernous malformations elsewhere in the neuraxis. methods: hospital records and radiographic files were analyzed for 17 patients who subsequently underwent surgical resection of an IMSC cavernous malformation (histologically proven) and also underwent brain magnetic resonance imaging studies. These 17 patients represented a subset of 32 patients who underwent surgical resection of an IMSC cavernous malformation during the same period. RESULTS: Of 17 patients, 8 (47%) harbored multiple cavernous malformations. This group was composed of five women and three men (mean age, 35.9 yr). There were four Caucasian and four Hispanic patients. CONCLUSION: The prevalence of multiple cavernous malformations in the neuraxis seems to be increased in patients who harbor IMSC cavernous malformations. This finding has important implications for the evaluation and management of these patients and, in some cases, their family members.- - - - - - - - - - ranking = 2.5keywords = spinal (Clic here for more details about this article) |
3/151. Acute promyelocytic leukaemia complicating multiple myeloma: evidence of different cell lineages.The association of leukemia and multiple myeloma is well described usually as a complication of chemotherapy but also in the absence of chemotherapy or at diagnosis. Such leukemias are typically acute myeloid leukemia (AML), particularly myelomonocytic subtype, and cases of acute promyelocytic leuke (APL) are rarely reported. Controversy exists as to whether myeloma and AML originate from a single haematopoietic progenitor or arise from different cell lineages. We report a case of a 58 year old female who developed APL 10 months following diagnosis of nonsecretory light chain (kappa) myeloma which had been treated with local spinal irradiation and low dose oral melphalan and prednisone. Clonality had originally been demonstrated by light chain restriction (kappa) of her bone marrow plasma cells whilst immunoglobulin heavy chain and T cell receptor genes were germ line. At development of APL cytogenetics revealed t(15;17) and PML-RAR fusion gene was detected by RT-PCR. The patient was treated with all-trans retinoic acid (ATRA) and received 2 cycles of consolidation chemotherapy with idarubicin. Following this therapy the t(15;17) and PML-RAR were both undetectable whilst the clonal population of kappa staining plasma cells persisted. This particular patient represents a rare case of APL complicating multiple myeloma with persistence of the myeloma clone but disappearance of PML-RAR alpha rna following therapy. This case study appears to support the argument that the APL and myeloma originated from distinct cell lineages.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
4/151. Spinal oligodendroglioma with gliomatosis in a child. Case report.The authors present a rare case of oligodendrogliomatosis in a child, which they believe originated from a primary spinal cord tumor. At 2.5 years of age this boy developed poor balance, neck stiffness, and a regression in developmental milestones. A computerized tomography (CT) scan of the head initially revealed ventriculomegaly and multiple cystic cerebellar lesions. In addition, magnetic resonance (MR) imaging revealed a cystic intramedullary lesion involving the cervical spinal cord. A CT scan of the head and an MR image obtained 3 years later demonstrated diffuse small cysts on the surface of the brainstem, cerebellum, medial temporal and inferior frontal cortices, subcortical white matter, and corpus callosum suggestive of leptomeningeal tumor spread. Analysis of pathological specimens obtained at surgery showed neoplastic glial cells with small, uniform nuclei and perinuclear clear zones. The cells appeared to migrate along the subpial space but no tumor cells were present in the subarachnoid space. These findings were compatible with a diagnosis of oligodendrogliomatosis cerebri. Despite having a complicated course, chemotherapy with carboplatin has provided the patient with long-term palliation and a high quality of life. This case may represent the fifth report in the literature of oligodendrogliomatosis occurring in a child but only the third occurring with a spinal primary tumor.- - - - - - - - - - ranking = 1.5keywords = spinal (Clic here for more details about this article) |
5/151. Multiple hemangiomas (hemangiomatosis) of the cauda equina and spinal cord. Case report.A case of multiple hemangiomas of the cauda equina nerve roots, conus medullaris, and lower spinal cord is described. The 74-year-old male patient presented with a 9-month history of progressive bilateral leg weakness. He had a history of lymphoma at the age of 39 years and renal cell carcinoma in his early 40s. Neither disease was evident at the time of this presentation. A magnetic resonance image revealed multiple enhancing nodules in the cauda equina region as well as on the pial surface of the lower thoracic spinal cord and conus medullaris. The patient underwent an L2-3 laminectomy. cauda equina nerve roots were found to be studded with numerous purple nodules, the largest measuring 6 to 8 mm. The nodules were adherent to nerve roots from which they could not be resected. Two lesions were histologically examined and found to be capillary hemangiomas. Twelve months into an uneventful postoperative course, the patient is neurologically unchanged. This unique case might represent a distinct form of hemangiomatosis confined to the cauda equina nerve roots and spinal cord.- - - - - - - - - - ranking = 3.5keywords = spinal (Clic here for more details about this article) |
6/151. Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report.The authors report the rare case of a patient with neurocutaneous vascular hamartomas mimicking Cobb syndrome. An 8-year-old boy was admitted to the authors' hospital with progressive urinary disturbance and upper back pain. Multiple skin nevi had been noted at the child's birth. Radiological examination revealed multiple cavernous angiomas in the spinal cord in the same metamere in which the skin nevi had been observed and also in the left cerebral hemisphere. His symptoms gradually improved without surgical intervention. Four years later he was readmitted because of a cerebral hemorrhage involving the left cerebral peduncle. Nonsurgical treatment was chosen because his symptoms promptly improved. To the best of the authors' knowledge, this is the first case of multiple cavernous angiomas in the brain and spinal cord associated with skin nevi. The authors discuss this clinical entity and the significance of the disease.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
7/151. Oncocytoma in melanocytoma of the spinal cord: case report.OBJECTIVE AND IMPORTANCE: Oncocytoma in the central nervous system is extremely unusual. The first reported example of oncocytoma in a melanocytoma of the spinal cord was successfully excised, and its pathological appearance is described. CLINICAL PRESENTATION: A 71-year-old woman presented with a 25-year history of back pain and myelographic evidence of a lumbar spinal cord mass. After declining surgical treatment for two decades, she elected eventually to have the mass excised. Preoperative magnetic resonance imaging revealed a large intraspinal mass that spanned spinal levels L3 through S1. TECHNIQUE: The mass was excised en bloc through posterior laminectomies, and histopathological analysis revealed a benign neoplasm composed predominantly of monotonous sheets of plump oncocytes. Electron microscopy confirmed that the cytoplasm of the oncocytes was packed full of mitochondria. Focal areas of the tumor contained spindle cells, with abundant intracytoplasmic granular deposits of brown melanin pigment that contained melanosomes. Positive Fontana-Masson, HMB-45, and S-100 staining confirmed the final diagnosis of melanocytoma, oncocytic variant. CONCLUSION: The first reported case of oncocytoma arising in spinal melanocytoma is described. After surgical excision, the patient recovered completely and has remained free of symptoms for 4 years.- - - - - - - - - - ranking = 4.5keywords = spinal (Clic here for more details about this article) |
8/151. Clinicopathological characterization of an hiv-2-infected individual with two clonally unrelated primary lymphomas.Human immunodeficiency virus 2 (hiv-2) is endemic in West africa and is a causative agent of the acquired immunodeficiency syndrome. Only a small number of hiv-2-infected patients have been described in detail. Non-Hodgkin's lymphoma (NHL) is the second most common neoplasm occurring in hiv-1-infected patients, but its incidence seems to be lower in hiv-2-infected individuals. We report an hiv-2-infected patient from cape verde (West africa) with separate and distinct systemic and primary central nervous system large B-cell lymphomas and review the findings of cases of hiv-2-associated lymphomas reported in the literature. Different clonal rearrangements of the immunoglobulin heavy chain gene could be detected in the two lymphomas of our patient by polymerase chain reaction and sequence analysis. These data indicate the presence of two clonally unrelated large B-cell lymphomas in the same patient, which is an unusual finding. Neither Epstein-Barr virus nor human herpesvirus 8 could be detected in the tumor tissues or the cerebrospinal fluid. hiv-2 infection should be considered in patients with NHL, especially in those from West africa.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
9/151. Intramedullary spinal cord metastases from a hypernephroma 11 years following the diagnosis and treatment of the primary lesion.Intramedullary spinal cord metastases are rare. An intramedullary spinal cord metastasis of renal origin, presenting 11 years after the diagnosis of the primary lesion, is reported.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
10/151. Double ependymoma of the filum terminale.Myxopapillary ependymoma of the filum terminale and conus medullaris are relatively common spinal intradural neoplasms in adults, only 8-14% affecting children. We describe a case of a 15-year-old girl with two myxopapillary ependymomas of the filum terminale. Although cases of two such tumours have been described in adults, we have not found any similar paediatric cases in the literature.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
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