Pituitary hemangioblastoma in a patient with von Hippel-Lindau disease

1975 ◽  
Vol 42 (2) ◽  
pp. 232-235 ◽  
Author(s):  
Noel G. Dan ◽  
Dermer E. Smith
Keyword(s):  
Content Type ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Fine Print

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

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
Keyword(s):  
Optic Nerve ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Dural Attachment ◽  
Microscopic Findings ◽  
Fine Print

✓ 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.

Symptomatic intrasellar hemangioblastoma in a child treated with subtotal resection and adjuvant radiosurgery

1996 ◽  
Vol 84 (6) ◽  
pp. 1046-1050 ◽  
Author(s):  
Paul D. Sawin ◽  
Kenneth A. Follett ◽  
B. Chen Wen ◽  
Edward R. Laws
Keyword(s):  
Residual Tumor ◽  
Subtotal Resection ◽  
Resonance Imaging ◽  
Subsequent Growth ◽  
Conventional Radiotherapy ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Adjuvant Therapies ◽  
Von Hippel Lindau Disease ◽  
Fine Print

✓ 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.

Surgical management of spinal cord hemangioblastomas in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 106-116 ◽  
Author(s):  
Russell R. Lonser ◽  
Robert J. Weil ◽  
John E. Wanebo ◽  
Hetty L. Devroom ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Surgical Management ◽  
Postoperative Outcome ◽  
Tumor Location ◽  
Neurological Dysfunction ◽  
Autosomal Dominant Disorder ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

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.

Distal anterior inferior cerebellar artery aneurysms

2002 ◽  
Vol 97 (3) ◽  
pp. 692-696 ◽  
Author(s):  
Eric L. Zager ◽  
Ellen G. Shaver ◽  
Robert W. Hurst ◽  
Eugene S. Flamm
Keyword(s):  
Artery Aneurysm ◽  
Distal Segment ◽  
Anterior Inferior Cerebellar Artery ◽  
Management Options ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Cerebellar Artery ◽  
Distal Branch ◽  
Acute Subarachnoid Hemorrhage ◽  
Fine Print

✓ 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.

The natural history of hemangioblastomas of the central nervous system in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 82-94 ◽  
Author(s):  
John E. Wanebo ◽  
Russell R. Lonser ◽  
Gladys M. Glenn ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Mass Effect ◽  
Content Type ◽  
Von Hippel Lindau ◽  
History Of ◽  
The Central Nervous System ◽  
Brainstem Tumors ◽  
Vhl Disease ◽  
Fine Print

Object. The goals of this study were to define the natural history and growth pattern of hemangioblastomas of the central nervous system (CNS) that are associated with von Hippel—Lindau (VHL) disease and to correlate features of hemangioblastomas that are associated with the development of symptoms and the need for treatment. Methods. The authors reviewed serial magnetic resonance images and clinical histories of 160 consecutive patients with VHL disease who harbored CNS hemangioblastomas and serially measured the volumes of tumors and associated cysts. Six hundred fifty-five hemangioblastomas were identified in the cerebellum (250 tumors), brainstem (64 tumors, all of which were located in the posterior medulla oblongata), spinal cord (331 tumors, 96% of which were located in the posterior half of spinal cord), and the supratentorial brain (10 tumors). The symptoms were related to a mass effect. A serial increase in hemangioblastoma size was observed in cerebellar, brainstem, and spinal cord tumors as patients progressed from being asymptomatic to symptomatic and requiring surgery (p < 0.0001). Twenty-one (72%) of 29 symptom-producing cerebellar tumors had an associated cyst, whereas only 28 (13%) of 221 nonsymptomatic cerebellar tumors had tumor-associated cysts (p < 0.0001). Nine (75%) of 12 symptomatic brainstem tumors had associated cysts, compared with only four (8%) of 52 nonsymptomatic brainstem lesions (p < 0.0001). By the time the symptoms appeared and surgery was required, the cyst was larger than the causative tumor; cerebellar and brainstem cysts measured 34 and 19 times the size of their associated tumors at surgery, respectively. Ninety-five percent of symptom-producing spinal hemangioblastomas were associated with syringomyelia. The clinical circumstance was dynamic. Among the 88 patients who had undergone serial imaging for 6 months or longer (median 32 months), 164 (44%) of 373 hemangioblastomas and 37 (67%) of 55 tumor-associated cysts enlarged. No tumors or cysts spontaneously diminished in size. Symptomatic cerebellar and brainstem tumors grew at rates six and nine times greater, respectively, than asymptomatic tumors in the same regions. Cysts enlarged seven (cerebellum) and 15 (brainstem) times faster than the hemangioblastomas causing them. Hemangioblastomas frequently demonstrated a pattern of growth in which they would enlarge for a period of time (growth phase) and then stabilize in a period of arrested growth (quiescent phase). Of 69 patients with documented tumor growth, 18 (26%) harbored tumors with at least two growth phases. Of 160 patients with hemangioblastomas, 34 patients (median follow up 51 months) were found to have 115 new hemangioblastomas and 15 patients new tumor-associated cysts. Conclusions. In this study the authors define the natural history of CNS hemangioblastomas associated with VHL disease. Not only were cysts commonly associated with cerebellar, brainstem, and spinal hemangioblastomas, the pace of enlargement was much faster for cysts than for hemangioblastomas. By the time symptoms appeared, the majority of mass effect—producing symptoms derived from the cyst, rather than from the tumor causing the cyst. These tumors often have multiple periods of tumor growth separated by periods of arrested growth, and many untreated tumors may remain the same size for several years. These characteristics must be considered when determining the optimal timing of screening for individual patients and for evaluating the timing and results of treatment.

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel—Lindau disease

1997 ◽  
Vol 87 (3) ◽  
pp. 445-449 ◽  
Author(s):  
Jean-Christophe Ouallet ◽  
Kathlyn Marsot-Dupuch ◽  
Remy Van Effenterre ◽  
Michele Kujas ◽  
Jean-Michel Tubiana
Keyword(s):  
Temporal Bone ◽  
Imaging Techniques ◽  
Endolymphatic Sac ◽  
Cystic Tumor ◽  
Content Type ◽  
Von Hippel Lindau ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
Magnetic Resonance Imaging Techniques ◽  
Temporal Bone Tumor

✓ This report describes a patient with von Hippel—Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.

Analysis of von Hippel—Lindau mutations with comparative genomic hybridization in sporadic and hereditary hemangioblastomas: possible genetic heterogeneity

2002 ◽  
Vol 97 (4) ◽  
pp. 977-982 ◽  
Author(s):  
Johanna M. M. Gijtenbeek ◽  
Bram Jacobs ◽  
Sandra H. E. Sprenger ◽  
Marc J. Eleveld ◽  
Ad Geurts van Kessel ◽  
...  
Keyword(s):  
Comparative Genomic Hybridization ◽  
Molecular Mechanisms ◽  
Chromosome 3 ◽  
Comparative Genomic ◽  
Genomic Hybridization ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Chromosome 3P ◽  
Chromosomal Imbalances ◽  
Fine Print

Object. Hemangioblastomas (HBs) occur sporadically or as a manifestation of von Hippel—Lindau (VHL) disease. In the majority of VHL-related HBs, inactivation of the VHL tumor suppressor gene (TSG), which is located on chromosome 3p25–26, is found. The VHL gene is assumed to be involved also in the development of sporadic HBs. In a previous study of chromosomal aberrations of sporadic HBs, multiple chromosomal imbalances were found in the majority of tumors. The aim of this study was to analyze further both sporadic HBs and VHL-related HBs to determine if these histopathologically identical tumors have a different genetic background. Methods. Sixteen sporadic HBs and seven VHL-related HBs were identified by clinical criteria and analyzed. Comparative genomic hybridization was used to screen for chromosomal imbalances throughout the entire HB genome. Additionally, mutation analysis of the VHL gene was performed using direct sequencing. Loss of chromosome 3 and multiple other chromosomal imbalances were found in the sporadic HBs, although only one imbalance, a loss of chromosome 3, was detected in the seven VHL-related HBs. Somatic VHL gene mutations were found in one third of sporadic HBs, whereas a mutation of the VHL gene was detected in all VHL-related HBs. Conclusions. These results indicate that the molecular mechanisms underlying sporadic HBs and VHL-related HBs are different. Inactivation of the VHL gene is probably not the most important event in the tumorigenesis of sporadic HBs. Other mechanisms of inhibition of VHL protein function, or inactivation of other TSGs, on chromosome 3p or on other chromosomes, might be important in the development of sporadic HBs.

Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 117-124 ◽  
Author(s):  
Ryszard M. Pluta ◽  
Brian Iuliano ◽  
Hetty L. Devroom ◽  
Tung Nguyen ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Anterior Approach ◽  
Posterior Approach ◽  
Long Term Results ◽  
Surgical Approaches ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Ventral Spinal Cord ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant neoplastic syndrome with manifestations in multiple organs, which is evoked by the deletion or mutation of a tumor suppressor gene on chromosome 3p25. Spinal hemangioblastomas (40% of VHL disease—associated lesions of the central nervous system) arise predominantly in the posterior aspect of the spinal cord and are often associated with an intraspinal cyst. Rarely, the tumor develops in the anterior aspect of the spinal cord. Ventral spinal hemangioblastomas are a surgical challenge because of difficult access and because vessels feeding the tumor originate from the anterior spinal artery. The goal of this study was to clarify whether an anterior or posterior surgical approach is better for management of hemangioblastomas of the ventral spinal cord. Methods. The authors performed a retrospective analysis of clinical outcomes and findings on magnetic resonance (MR) imaging studies in eight patients (two women and six men with a mean age of 34 ± 15 years) who underwent resection of ventral spinal hemangioblastomas (nine tumors: five cervical and four thoracic). Two surgical approaches were used to resect these tumors. A posterior approach was selected to treat five patients (laminectomy and posterior myelotomy in four patients and the posterolateral approach in one patient); an anterior approach (corpectomy and arthrodesis) was selected to treat the remaining three patients. Immediately after surgery, the ability to ambulate remained unchanged in patients in whom an anterior approach had been performed, but deteriorated significantly in patients in whom a posterior approach had been used, because of motor weakness (four of five patients) and/or proprioceptive sensory loss (three of five patients). This difference in ambulation, despite significant improvements over time among patients in the posterior access group, remained significant 6 months after surgery. In all cases, MR images revealed complete resection of the tumor and in five patients significant or complete resolution of the intramedullary cyst was demonstrated (present in six of eight patients). Conclusions. The outcomes of these eight patients with hemangioblastomas of the ventral spinal cord indicate that both immediate and long-term results are better when an anterior approach is selected for resection.

Fibrous meningioma in a patient with von Hippel—Lindau disease: a genetic analysis

2001 ◽  
Vol 95 (6) ◽  
pp. 1045-1049 ◽  
Author(s):  
Lance S. Governale ◽  
Alexander O. Vortmeyer ◽  
Zhengping Zhuang ◽  
Edward H. Oldfield
Keyword(s):  
Gene Locus ◽  
Neurofibromatosis Type ◽  
Magnetic Resonance Images ◽  
Polymorphism Analysis ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Single Stranded Conformational Polymorphism ◽  
Von Hippel Lindau Disease ◽  
Unaffected Parent ◽  
Vhl Disease

✓ Meningioma has been included in the constellation of tumors associated with von Hippel—Lindau (VHL) disease in previously published reports. It is unclear whether these tumors are an uncommon component of VHL disease or are more readily detected in these patients because of the frequency with which they undergo central nervous system imaging as part of the routine management of VHL disease. The authors report the case of a patient with VHL disease in whom a progressively enlarging supratentorial mass developed and was diagnosed as a hemangioblastoma because of its appearance on serial magnetic resonance images. At surgery the tumor displayed the typical features of a meningioma and was given the histological diagnosis of fibrous meningioma. Single-stranded conformational polymorphism analysis of the tumor DNA revealed a loss of heterozygosity at the neurofibromatosis Type 2 gene locus, known to be associated with sporadically occurring meningiomas. Despite this finding, the VHL gene locus on the allele from the patient's unaffected parent was normal. Thus it is unlikely that the occurrence of this patient's fibrous meningioma was associated with underlying VHL disease. Given the high frequency of neuroimaging sessions in patients with VHL disease, some supratentorial lesions that have been given radiological diagnoses of hemangioblastomas may be incidental meningiomas.

Gamma knife surgery for hemangioblastomas

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 171-174 ◽  
Author(s):  
Masao Tago ◽  
Atsuro Terahara ◽  
Masahiro Shin ◽  
Keisuke Maruyama ◽  
Hiroki Kurita ◽  
...  
Keyword(s):  
Contrast Enhancement ◽  
Gamma Knife ◽  
Survival Rates ◽  
Gamma Knife Surgery ◽  
Tumor Control ◽  
Repeated Treatment ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Fine Print

Object. The authors reviewed their 14-year experience using stereotactic radiosurgery for the treatment of hemangioblastomas and define the role and the proper strategy for radiosurgery of this condition. Methods. This is a retrospective study of 38 hemangioblastomas in 13 patients. Seven patients had von Hippel—Lindau disease. All patients have undergone at least one follow-up visit. The median and mean tumor volumes were 0.23 cm3 and 0.72 cm3 respectively (range 0.004:4.84 cm3). Twenty-eight tumors received 20 Gy to the margin, and the remainder received 18 Gy. The median clinical follow-up period was 36 months (range 3–159 months). No patient died. The survival rate was 84.6% (11 of 13 patients). The actuarial 5- and 10-year survival rates were both 80.8%. The median radiological follow-up period was 35 months (range 7–147 months). Only one tumor increased in volume 24 months after treatment in association with an intratumoral hemorrhage. The tumor control rate was 97.4% (37 of 38 tumors). Actuarial 5- and 10-year control rates were both 96.2%. New lesions and/or those increasing in size outside the irradiated area were discovered in five patients (38.5%). Nine tumors revealed peritumoral contrast enhancement which was seen more frequently in larger tumors with a volume greater than 0.5 cm3 (p = 0.0034). Conclusions. Gamma knife surgery is a safe and effective method to control hemangioblastomas for as many as 10 years. Higher doses and smaller tumors probably contribute to good outcomes. Recurrence outside the original irradiated area is common. Peritumoral contrast enhancement may be seen in larger tumors. The authors recommend regular imaging follow up and early repeated treatment in the face of new or growing tumors.

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