Intraorbital optic nerve hemangioblastoma with von Hippel-Lindau disease

1982 ◽  
Vol 56 (3) ◽  
pp. 426-429 ◽  
Author(s):  
Seiich In ◽  
Jun Miyagi ◽  
Nobuto Kojho ◽  
Shinken Kuramoto ◽  
Masaki Uehara

✓ An intraorbital hemangioblastoma of the optic nerve is reported in a 23-year-old woman with von Hippel-Lindau disease. The absence of dural attachment and the microscopic findings were characteristic of hemangioblastoma. Four years later the patient developed a cystic hemangioblastoma in the left cerebellum which was successfully treated.

1975 ◽  
Vol 42 (2) ◽  
pp. 232-235 ◽  
Author(s):  
Noel G. Dan ◽  
Dermer E. Smith

✓ A case of von Hippel-Lindau disease is described in which a pituitary hemangioblastoma was present as well as a cerebrovascular anomaly.


1996 ◽  
Vol 84 (6) ◽  
pp. 1046-1050 ◽  
Author(s):  
Paul D. Sawin ◽  
Kenneth A. Follett ◽  
B. Chen Wen ◽  
Edward R. Laws

✓ The first documented case of a symptomatic intrasellar hemangioblastoma is described, occurring in an 11-year-old girl with stigmata of von Hippel—Lindau disease who presented with headaches, progressive bitemporal hemianopsia, and adenohypophysial dysfunction. A subtotal resection of the lesion was achieved with two separate surgical procedures: a transsphenoidal approach and a subfrontal craniotomy. Subsequent growth of residual tumor was treated with combined conventional radiotherapy and stereotactic radiosurgery. Two years following completion of these adjuvant therapies, no residual tumor was evident on magnetic resonance imaging. Previous experience with hemangioblastoma in this region, as well as the rationale for radiotherapy in the treatment of incompletely resected lesions, is reviewed.


2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 143-146 ◽  
Author(s):  
Yang Kwon ◽  
Jun Seok Bae ◽  
Jae Myung Kim ◽  
Do Hee Lee ◽  
Soon Young Kim ◽  
...  

✓ Tumors involving the optic nerve (optic glioma, optic nerve sheath meningioma) are benign but difficult to treat. Gamma knife surgery (GKS) may be a useful treatment. The authors present data obtained in three such cases and record the effects of GKS.


2003 ◽  
Vol 98 (1) ◽  
pp. 106-116 ◽  
Author(s):  
Russell R. Lonser ◽  
Robert J. Weil ◽  
John E. Wanebo ◽  
Hetty L. Devroom ◽  
Edward H. Oldfield

Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients. Methods. Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20–58 years) at the National Institutes of Health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. Patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered. Conclusions. Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.


1987 ◽  
Vol 67 (1) ◽  
pp. 137-139 ◽  
Author(s):  
Alberto Isla ◽  
José Palacios ◽  
José M. Roda ◽  
Manuel Gutierrez ◽  
Cesáreo González ◽  
...  

✓ A 34-year-old woman presented with progressive loss of vision in her left eye of 2 months' evolution. Computerized tomography showed a hypodense lesion in the suprasellar region. At surgery a cystic lesion was found inside the optic nerve. Histological study proved it to be a neuroepithelial cyst. The pathogenesis of a neuroepithelial cyst in such an exceptional site is discussed.


2002 ◽  
Vol 97 (3) ◽  
pp. 692-696 ◽  
Author(s):  
Eric L. Zager ◽  
Ellen G. Shaver ◽  
Robert W. Hurst ◽  
Eugene S. Flamm

✓ Aneurysms of the distal anterior inferior cerebellar artery (AICA) are rare; fewer than 100 cases have been reported. The authors detail their experience with four cases and present endovascular as well as microsurgical management options. The medical records and neuroimaging studies obtained in four patients who were treated at a single institution were reviewed. Clinical presentations, neuroimaging and intraoperative findings, and clinical outcomes were analyzed. There were three men and one woman; their mean age was 43 years. Two patients presented with acute subarachnoid hemorrhage (SAH), and two presented with ataxia and vertigo (one with tinnitus, the other with hearing loss). Angiographic studies demonstrated aneurysms of the distal segment of the AICA. In one patient with von Hippel—Lindau syndrome and multiple cerebellar hemangioblastomas, a feeding artery aneurysm was found on a distal branch of the AICA. Three of the patients underwent successful surgical obliteration of their aneurysms, one by clipping, one by trapping, and one by resection along with the tumor. The fourth patient underwent coil embolization of the distal AICA and the aneurysm. All patients made an excellent neurological recovery. Patients with aneurysms in this location may present with typical features of an acute SAH or with symptoms referable to the cerebellopontine angle. Evaluation with computerized tomography, magnetic resonance (MR) imaging, MR angiography, and digital subtraction angiography should be performed. For lesions distal to branches coursing to the brainstem, trapping and aneurysm resection are viable options that do not require bypass. Endovascular obliteration is also a reasonable option, although the possibility of retrograde thrombosis of the AICA is a concern.


1971 ◽  
Vol 34 (1) ◽  
pp. 107-113 ◽  
Author(s):  
Albert W. Cook

✓ An operative technique for total removal of large global meningiomas at the medial aspect of the sphenoid ridge is described, and experience with 11 patients reported. The technique involves extradural liberation of the dural and tumor attachments to the underlying bone, and extradural occlusion of the blood supply through bone and middle meningeal artery. Subsequent procedures are carried out sequentially in the parasellar area to free the optic nerve and carotid, in the subtemporal tentorial region to release tumor from neighboring structures, and in the Sylvian fissure to isolate the middle cerebral artery.


1997 ◽  
Vol 7 (1) ◽  
pp. 48-50 ◽  
Author(s):  
Frank A. Raila ◽  
James Zimmerman ◽  
Phillip Azordegan ◽  
Jonathan Fratkin ◽  
Andrew D. Parent

1998 ◽  
Vol 88 (6) ◽  
pp. 1104-1106 ◽  
Author(s):  
Alister J. Hart ◽  
James Allibone ◽  
Adrian T. H. Casey ◽  
David G. T. Thomas

✓ Meningiomas, thought to arise from arachnoid cap cells, are usually attached to the dura. Malignancy is present in approximately 1% of these tumors. The authors report the case of a patient with a malignant meningioma arising from the oculomotor nerve with no dural attachment. The patient presented with a 7-month history of left-sided ptosis and diplopia. Magnetic resonance imaging demonstrated an extrinsic mass compressing the root of the oculomotor nerve at its exit from the midbrain. During surgery, a left-sided subtemporal approach revealed the tumor to be arising from the oculomotor nerve. Histological investigation showed a malignant spindle cell lesion with an immunohistochemical profile that was consistent with malignant meningioma. To the authors' knowledge, this is the first documented case of a malignant meningioma arising from the oculomotor nerve.


1993 ◽  
Vol 78 (6) ◽  
pp. 979-982 ◽  
Author(s):  
William Y. Lu ◽  
Marc Goldman ◽  
Byron Young ◽  
Daron G. Davis

✓ Gangliogliomas of the optic nerve are extremely rare. The case is reported of a 38-year-old man who presented with a visual field deficit and was discovered to have an optic nerve ganglioglioma. The possible embryological origins of this neoplasm, its histological and immunohistochemical features, and its appearance on magnetic resonance imaging are examined. The prognoses of optic nerve glioma and of gangliogliomas occurring elsewhere in the nervous system are compared.


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