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von Hippel-Lindau disease(VHL) is a hereditary cancer syndrome caused by germline mutations of the VHL tumor suppressor gene, and manifested by hemangioblastomas in central nervous system, retinal angioma, cysts in liver, pancreas or kidney. Sometimes other systemic malignancies, such as renal cell carcinoma or pheochromocytoma, are associated. Major causes of death in VHL disease are associated with cerebellar hemangioblastoma or renal cell carcinoma. We have experienced two cases of VHL disease in a family. A son had multiple scattered hemangioblastomas in cerebellum, spinal cord, retinal angiomas and renal cyst. And his mother had a renal cell carcinoma in addition to a solid type hemangioblastoma in cerebellum. With a review of the literature, the authors present two cases of VHL disease in a family.
Objectives:Numerous genetic alterations are related to tumorigenesis and influence the radiological features of malignant glial tumor. We performed a cytogenetic analysis from a fresh tumor specimen and analyzed its relationship with radiological features and prognosis. Materials and Methods:Among the 29 patients with malignant glial tumor involved in cytogenetic analysis, 19 cases showing chromosomal abnormality were analyzed. Cytogenetic analysis was performed with pathologically proven fresh surgical specimen. Both medical records and radiological findings were reviewed to define the tumor, to evaluate peritumoral edema and to assess treatment results. Results:The most frequent genetic abnormality was observed in chromosome 7 (57.8 %). Complex karyotype presenting three or more abnormalities was noted in 11 cases (57.8 %). Chromosomal abnormalities, noted in more than three cases, were whole loss of Y (7 cases), whole gain of 7 (6 cases), whole gain of 2, whole gain of 5, whole gain of 12, 19q gain, 6p loss, 9p loss, and whole gain of 13 (3 cases). We reported that loss of chromosome 7 related to MDR-1 (Multidrug resistant gene) were correlated with good response of chemotherapy. Conclusions:A chromosomal analysis of malignant glioma can be perfomed to discover tumorigenic factors and to evaluate radiological features. Results of karyotyping is closely related to the clinical outcome and may be utilized in developing new therapeutic modalities.
Objective:Delayed hyponatremia is a common complication after transsphenoidal surgery (TSS) for pituitary adenomas. We retrospectively evaluated our patients and tried to find possible associated risk factors of delayed hyponatremia. Methods:From 2006 until 2010, total 575 cases of pituitary adenomas were operated with TSS in a single institution. Thirty-one patients (5.3%) with delayed hyponatremia were compared to other 544 patients without delayed hyponatremia in the aspects of age, sex, Hardy classification, tumor size, hormonal type and activity on preoperative combined pituitary function test. Their postoperative outcomes were also evaluated in the aspects of tumor removal fashion and hormonal outcome on postoperative combined pituitary function test. Results:In 31 patients, delayed hyponatremia started 7.0 days after surgery (range : postoperative day 3-10). At the time of diagnosis of hyponatremia, serum level of sodium ranged from 111 mmol/L to 132 mmol/L (mean : 120.5 mmol/L) and mean duration of treatment was 4.l days (range : 2-9 days). Piecemeal tumor removal was more frequent in a group with delayed hyponatremia (p=0.035). The presence of postoperative hypopituitarism and aggravation of pituitary function were observed more frequently in a group with delayed hyponatremia (p=0.002 and p=0.017, respectively). Conclusion:Delayed hyponatremia is a common complication after TSS and potentially fatal. Piecemeal fashioned tumor removal, postoperative hypopituitarism and aggravation of pituitary function are associated with higher chance of delayed hyponatremia.
Objectives:The endoscopic endonasal transsphenoidal approach (EETSA) is increasingly being used to treat pituitary adenomas and other sellar/suprasellar pathologies. One important complication of the transsphenoidal approach is cerebrospinal fluid (CSF) leakage, which occurs in 0.4 to 9% of cases. We report our experience with EETSA and describe the me-thods that we use to prevent CSF leakage. Methods:A single surgeon performed EETSA on 170 consecutive patients between Feb 2004 and Apr 2011. Patients were divided into three groups based on their CSF leakage status as follows: no CSF leakage, minor CSF leakage, and major CSF leakage. Sellar floor reconstruction was performed according to this classification. In the no-CSF leakage group, sellar floor reconstruction was performed with oxidative cellulose (Surgicel®) only. In the second group (minor CSF leakage), sellar floor reconstruction was performed using autologous fat and bone grafts. Patients in the third group (major CSF leakage) underwent an additional reconstructive procedure of the skull base using the pedicled septal mucosal flap. Results:Of 170 patients, 30 (17.6%) showed CSF leakage in the operation field ; 24 (14.1%) were classified as having minor CSF leakage and 6 (3.5%) patients were classified as having major CSF leakage. Post-operative CSF leakage occurred in only two cases. Conclusion:CSF leakage, though relatively common in the operation field, can be prevented effectively with adequate reconstruction techniques. Assignment of patients to one of three groups based on their CSF leakage in the operation field and sellar floor reconstruction based on this classification resulted in favorable postoperative outcomes.
Introduction:Intraventricular meningiomas are uncommon subtype of intracranial meningiomas. Trigonal meningiomas consist of the majority of intraventricular meningiomas. The superior parieto-occipital transcortical approach is the most popular approach for removal of them. We analyzed our experience in removal of the trigonal meningiomas that were resected using the superior parieto-occipital transcortical approach. Patients and Methods:Twenty-four patients underwent surgical removal of trigonal meningioma from 1997 to May 2012. Demographics, clinical features, surgical outcomes, histopathologic results and prognosis were analyzed. Results:Study population had a mean age of 51.2 years and a female preponderance of 1 : 1.7. The most common presenting symptom was chronic headache, which was followed by memory impairment, visual disturbance and hemiparesis. Total removal of the tumor was achieved in all cases. Twenty-two (91.7%) tumors were World Health Organization (WHO) grade I while two cases were WHO grade III. There were two cases of intracerebral hemorrhage in the surgical corridor, one case of transient contralateral hemiparesis with cognitive decline, one case of transient contralateral homonymous hemianopsia, one case of aggravation of visual field defect, and one case of hydrocephalus which was successfully treated by ventriculo-peritoneal shunt. Conclusion:The superior parieto-occipital approach is suitable for most of the trigonal meningiomas and total removal of them can be achieved safely.
Multiple schwannomas without vestibular schwannomas (schwannomatosis) are new classification of neurofibromatoses. Understanding of this disease entity is emerging, recently. We experienced a case of schwannomatosis, and reviewed some literatures related to schwannomas. A 45-year-old woman visited our hospital with low back pain and radiating leg pain. The patient’s spine MR images revealed that she had multiple intradural extramedullary masses in lumbar spinal canal, extending to paravertebral space through neural foramen. On brain MR images, there were no vestibular schwannomas. We took a operation to resolve her symptoms. After we removed the intradural extramedullary masses in lumbar spinal canal, her symptoms were improved.
Gangliogliomas are rare tumors of the central nervous system, composed of neoplastic ganglion cells and neoplastic glial cells. They are characterized by slow growing, low incidence of malignancy, surgically curable, and usually occurred in children and young adults. We report a case of ganglioglioma occurred in 21 year-old male which radiological findings were compatible with the malignancy but histologically diagnosed as benign, with review of literatures.
Objective:The purpose of this study was to compare the dose planning between the Leksell Gamma Knife Perfexion (LGK PFX) and the Leksell Gamma Knife C (LGK C) using variable indices. Methods:A total of 100 cases, which were composed of 35 meningiomas, 20 vestibular schwannomas, 35 metastases, and 10 pituitary adenomas, were enrolled in this study. First, these cases were treated with the LGK PFX and then, were re-planned with the LGK C. We compared these two models in terms of the number of shots, the percentage of coverage, the conformity index (CI), Paddick’s conformity index (PCI), the gradient index (GI), and the beam on time. Results:The LGK PFX completely outperformed the LGK C in terms of GI and the LGK PFX tended to have a longer beam on time than that of the LGK C. However, in patients with schwannomas, the LGK PFX outperformed the LGK C in terms of the CI, PCI, and GI, and in patients with pituitary adenomas, the LGK PFX outperformed the LGK C in terms of the percentage of coverage, PCI, and GI with statistical significance. Conclusion:The LGK PFX is an entirely redesigned radiosurgery unit accompanied by the development of software. The LGK PFX is supposed to achieve highly conformal dose prescription consisting of many isocenters with a reasonable treatment time.
The cause of intracerebral hemorrhage (ICH) is not always able to be obviously detected on computed tomography (CT), whether with enhance or not. In our case, CT showed multi-lobulated appearance of ICH but contrast enhanced CT did not revealed any abnormal enhanced lesions. During operation we met by chance the abnormal cortical tissue, pathologic diagnosis of which was glioblastoma. Although hemorrhage seem to have no pathologic cause on contrast- enhanced CT, if appearance of hemorrhage is different from hypertensive ICH on non contrast CT, we should perform magnetic resonance imaging (MRI) if available and keep in mind histological examination of the lesions.
Ectopic meningioma is derived from presummed ectopic arachnoid cap cells in an extradural location and occurs in only 0.8-0.9% of all meningiomas. We report a very rare case of ectopic meningioma seemed to originate from the diploic space. A 46-year-old woman presented with a firm scalp mass on the left parietal region. Magnetic resonance images showed a huge heterogeneously enhancing mass both in the intracranial and in the extracranial portion. Intraoperative findings demonstrated the tumor mainly occupied the intradiploic space and through dural defect it expanded into both intracranial and extracranial space like a flying saucer. The tumor was excised subtotally and the pathological diagnosis was transitional meningioma. The postoperative clinical course was uneventful. With reviewing pertinent literatures, we enforce this case as intradiploic meningioma, which may be classified as a subgroup of primary intraosseous meningioma.