1/51. Successful removal of a hemangioblastoma from the medulla oblongata: case report.Hemangioblastomas are histologically benign tumors that occur exclusively within the neuraxis, most commonly in the posterior fossa. They are typically cystic tumors located in the cerebellum. Excision of the vascular mural nodules leads to cure. brain stem lesions are rarely reported. Surgical extirpation of a solid brain stem hemangioblastoma is relatively risky and requires precise microsurgical techniques. We present a woman with a hemangioblastoma embedded in the medulla oblongata. This 33-year-old woman presented with occipital headaches and sensory ataxia. Complete and detailed preoperative imaging studies were followed by successful microsurgical excision of the lesion. The patient recovered completely within 2 weeks after the operation except for mild paresthesia of the legs. Preoperative magnetic resonance imaging and cerebral angiography provided important information regarding the nature, location, and blood supply of this lesion, which facilitated its total removal. The importance of intraoperative identification and control of the feeding artery of the tumor is emphasized.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/51. The occurrence of cerebellar hemangioblastoma in numerous first degree relatives with von hippel-lindau disease.von hippel-lindau disease is an autosomal dominant disorder caused by a mutation of VHL gene. The incidence of the disease is one in 36,000 and its clinical manifestation is a familial occurrence of hemangioblastoma of the central nervous system and retina, renal cell cancer and pheochromocytoma. Cerebellar hemangioblastoma is the most frequent or sometimes the only abnormality observed in this syndrome. We present a family with von hippel-lindau disease in which four first degree relatives had a cerebellar hemangioblastoma. This neoplasm caused the death of two brothers aged 27 and 24 years old, respectively and their mother aged 62. The third son of this family was affected ten years ago, at the age of 30. The healthy family members are counselled in Oncological Genetic Outpatient Unit in Gdansk.- - - - - - - - - - ranking = 46.390279235107keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/51. Somatic mosaicism in von hippel-lindau disease.von Hippel-Lindau (VHL) disease is an autosomal dominant familial cancer syndrome predisposing to the development of retinal and central nervous system haemangioblastomas, pheochromocytomas, renal and pancreatic cancer. In the course of a molecular analysis conducted to detect germline mutations of this gene in von Hippel-Lindau patients and individuals affected by sporadic tumors, we have identified a case of somatic mosaicism in the asymptomatic mother of a VHL patient who was subsequently diagnosed with pheochromocytoma. This is the first report providing molecular evidence of somatic mosaicism in von hippel-lindau disease. mosaicism could provide some genetic explanation for the clinical heterogeneity and variable severity of the VHL phenotype, and should be considered, as a possible event when evaluating sporadic cases of VHL or patients with isolated VHL-related tumors. Hum Mutat 15:114, 2000.- - - - - - - - - - ranking = 46.390279235107keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/51. Hemangioblastomas with blood supply from the dural arteries--two case reports.Hemangioblastomas are benign vascular tumors that often occur in the cerebellum, and are located near the pia mater. The blood supply is usually received through the pia mater, and rarely through the external carotid artery. The present cases of hemangioblastoma received blood supply from the external carotid artery (occipital artery) and a branch of the internal carotid artery (carotico-tympanic artery or artery of Bernasconi Cassinari) through the dural branches. The dural arteries were not the main feeders in either case, but preoperative embolization of the occipital artery contributed to minimum bleeding during the operation in one case. Incomplete resection of hemangioblastoma is related to multicentricity of the tumors, small mural nodules, or brain stem involvement. Angiography is valuable for demonstrating arterial supply to small or multiple mural nodules. Conventional angiography is necessary for investigation of the external carotid artery branches.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
5/51. Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.- - - - - - - - - - ranking = 56.950075581186keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/51. Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases.Generally accepted contraindications to using a transsphenoidal approach for resection of tumors that arise in or extend into the suprasellar region include a normal-sized sella turcica, normal pituitary function, and adherence of tumor to vital intracranial structures. Thus, the transsphenoidal approach has traditionally been restricted to the removal of tumors involving the pituitary fossa and, occasionally, to suprasellar extensions of such tumors if the sella is enlarged. However, conventional transcranial approaches to the suprasellar region require significant brain retraction and offer limited visualization of contralateral tumor extension and the interface between the tumor and adjacent structures, such as the hypothalamus, third ventricle, optic apparatus, and major arteries. In this paper the authors describe successful removal of suprasellar tumors by using a modified transsphenoidal approach that circumvents some of the traditional contraindications to transsphenoidal surgery, while avoiding some of the disadvantages of transcranial surgery. Four patients harbored tumors (two craniopharyngiomas and two hemangioblastomas) that arose in the suprasellar region and were located either entirely (three patients) or primarily (one patient) within the suprasellar space. All patients had a normal-sized sella turcica. Preoperatively, three of the four patients had significant endocrinological deficits signifying involvement of the hypothalamus, pituitary stalk, or pituitary gland. Two patients exhibited preoperative visual field defects. For tumor excision, a recently described modification of the traditional transsphenoidal approach was used. Using this modification, one removes the posterior portion of the planum sphenoidale, allowing access to the suprasellar region. Total resection of tumor was achieved (including absence of residual tumor on follow-up imaging) in three of the four patients. In the remaining patient, total removal was not possible because of adherence of tumor to the hypothalamus and midbrain. One postoperative cerebrospinal fluid leak occurred. Postoperative endocrinological function was worse than preoperative function in one patient. No other new postoperative endocrinological or neurological deficits were encountered. This study demonstrates the feasibility of using a modified transsphenoidal approach for resection of certain suprasellar, nonpituitary tumors.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
7/51. MKM-guided resection of diffuse brainstem neoplasms.OBJECTIVES: Some primary brainstem tumors, when extensive, are considered inoperable. We wanted to assess the value of robotic image-guided microscopic surgery in the resection of these tumors and to improve survival and quality of life for these patients. methods: Two patients with extensive brainstem tumors were evaluated at our center. They previously underwent several biopsies, attempted partial resections, radiotherapy and shunting. They presented with progressive neurological deterioration, 'coma vigil' for several months, and required life-supporting measures prior to surgery. Both patients underwent frameless stereotactic craniotomy using a MKM robotic microscope, intraoperative neurophysiological monitoring, and extensive resection of their recurrent brainstem tumors. RESULTS: In the immediate weeks after surgery, both patients became interactive and regained major motor and cranial nerve deficits present prior to surgery. Nine months after surgery, 1 patient succumbed to pneumonia. At 2 years after the operation, 1 patient has maintained his neurological status and showed no recurrence on imaging studies. CONCLUSIONS: Image-guided surgery with an MKM microscope allows surgical outlines to be injected in the microscope viewer and facilitates resection of extensive brainstem tumors previously considered inoperable.- - - - - - - - - - ranking = 8keywords = brain (Clic here for more details about this article) |
8/51. Multiple cerebellar haemangioblastomas symptomatic during pregnancy.A patient with von hippel-lindau disease presented during her seventh week of pregnancy with obstructive hydrocephalus and brainstem compression caused by multiple cerebellar haemangioblastomas. Surgery was performed to relieve the hydrocephalus and remove the right cerebellar tumour. The patient's subsequent course supports the hypothesis of vascular engorgement of cerebellar haemangioblastomas during pregnancy.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/51. central nervous system complications of von hippel-lindau disease and pregnancy; perinatal considerations: case report and literature review.A 30-year-old woman with von hippel-lindau disease presented at 30 weeks' gestation with a symptomatic cerebellar hemangioblastoma. She underwent a craniotomy for complete removal of the tumor. The postoperative period and the remaining of the pregnancy were uneventful. She delivered under epidural anesthesia after induction for postterm.- - - - - - - - - - ranking = 42.239185384315keywords = nervous system (Clic here for more details about this article) |
10/51. Haemangioblastoma of a cervical sensory nerve root in Von Hippel-Lindau syndrome.Spinal haemangioblastomas are rare, accounting for only about 7% of all central nervous system cases. The case of a 40-year-old woman with a haemangioblastoma arising solely from a cervical sensory nerve root is presented. At operation via a cervical laminectomy, it was possible to resect the tumour en masse with the sensory ramus, by extending the laminectomy through the exit foramen for C6. Haemangioblastomas are commonly intramedullary, and have only been reported in this location on one previous occasion. The patient has Von Hippel-Lindau syndrome and a history of multiple solid tumours. The possible role of the Von Hippel-Lindau tumour suppressor gene in the pathogenesis of these neoplasms is discussed.- - - - - - - - - - ranking = 46.390279235107keywords = central nervous system, nervous system (Clic here for more details about this article) |
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