Spinal Cord Hemangioblastoma Research Articles

Microsurgical treatment of intramedullary hemangioblastoma of spinal cord

Objective To investigate the therapeutic strategies of spinal cord hemangioblastoma.Method Clinical data of 40 patients with intramedually hemangioblastoma were analyzed.The function of spinal cord was evaluated before and after operation with McCormick scale.Results The diagnosis of hemangioblastoma was confirmed by postoperative pathology.All the 40 patients underwent complete excision under microscope.The postoperative neurological status was improved in 30 patients,remained unchanged in 8,and aggravated in 2.There was no other surgical complication.Conclusions Spinal cord hemangioblastoma can be safely and effectively removed via posterior approach.Postoperatively,clinical outcome is excellent in the majority of cases.Patients with symptoms should be operated actively. Key words: Spinal cord neoplasms; Hemangioblastoma; Microsurgery

Intraoperative Use of Indocyanine Green Fluorescence Videography for Resection of a Spinal Cord Hemangioblastoma

Indocyanine green (ICG) fluorescence videography has been recently applied to the neurosurgical field, mostly in the management of cerebral aneurysms, but has had limited description in the subspecialty of spine or oncological neurosurgery. We describe a novel application of this previously defined surgical tool to assist in the resection of a residual spinal cord hemangioblastoma. Our patient is a 49-year-old woman with a residual symptomatic cervical hemangioblastoma that was previously embolized and resected at another institution. After initial symptomatic improvement, she returned with progressive symptoms, increasing radiographic spinal cord edema, and a residual lesion at the level of C1. We resected the remaining tumor with the adjuvant use of ICG fluorescence videography. Intraoperative injection of ICG clearly identified a component of the tumor underlying adhesive, opaque arachnoid that was not visualized by direct microscopy. Immediate postresection ICG videography suggested a complete resection was achieved which was later corroborated by postoperative magnetic resonance imaging. The adjuvant use of ICG videography is a useful surgical tool that permits greater visualization of the complete extent of the lesion, particularly in managing recurrent or residual lesions obscured by adhesions.

Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease

Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas. Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed. One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 +/- 5.0 years (range 0.5-20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54-96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07-55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients). Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.

Tolerance of the Spinal Cord to Stereotactic Radiosurgery: Insights from Hemangioblastomas

Due to a paucity of reported radiation myelopathy, the radiation tolerance of the spinal cord to stereotactic radiosurgery (SRS) is poorly understood. A variety of spinal cord dose constraint parameters exist. Without a clear consensus, the general recommended spinal cord maximum dose (Dmax) constraints range from 10–14 Gy. In order to provide insight on spinal cord dose–volume effects, particularly for relatively small volumes of spinal cord receiving doses higher than the general dose constraints, we present a retrospective review of patients treated with SRS for spinal cord hemangioblastomas, a histology traditionally treated aggressively at our institution.

Monotherapy with thalidomide for treatment of spinal cord hemangioblastomas in a patient with von Hippel–Lindau disease

Von Hippel-Lindau (VHL) disease is a cancer-prone syndrome characterized by abnormalities in vascular proliferation and the development of both the visceral and CNS tumors. Complications from hemangioblastoma are among the principal causes of death from this syndrome. Antiangiogenic therapy has been used with different modalities in patients suffering from such complications. Here, we describe an adolescent with VHL complicated by progressive, multifocal spinal hemangioblastomas. Treatment with single-agent thalidomide over the course of 3 years was associated with an unexpected stabilization of the disease. The antiangiogenic effect of thalidomide may be associated with the control of progressive hemangioblastoma.

Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups

To analyze the surgical outcome of a consecutive and single center series of medulla oblongata (MO) and spinal cord hemangioblastomas (HB). We retrospectively reviewed the medical charts of all MO and spinal HB patients operated on in our institution between 1985 and 2002. All patients had pre- and at least one post-operative MRI. McCormick classification was used to assess neurological status and functional outcome. Forty surgical procedures have been performed on 34 patients (19 females and 15 males, mean age of 41 years). Twenty-five (73%) patients had Von Hippel Lindau (VHL) disease confirmed by genetic screening, and nine patients had sporadic disease. Complete clinical, radiological, and genetic studies were done in all cases. The most frequent clinical symptom was pain (28 patient, 85%) followed by motor (42%) and sensitive deficits (42%). Fourteen lesions (19%) were located in the MO, 28 (38%) in the cervical spine, 25 (34%) in the thoracic spine, 4 (5%) in the lumbar spine and 3 (4%) in the Cauda Equina. In the VHL group, 15 patients (60%) presented multiple lesions and 10 a single neurological lesion (40%). A cyst was present in 23% of VHL patients and in 55% of the non-VHL group. A complete removal was achieved in 85% of all cases. No deaths related to surgery occurred. At the end of the follow-up period (mean 60 months) 50% of patients were stabilized, while the condition of 32.35% was improved and of 17.65% worsened. Comparing the clinical evolution considering the presence or not of VHL we have seen that there are no differences in terms of functional outcome between VHL and non-VHL groups. Our results confirmed that surgery remains a safe and effective treatment for medulla oblongata and spinal hemangioblastoma. Only symptomatic lesion required surgical treatment. In other cases, especially in VHL patients, a close and regular follow-up (clinical and MRI) is necessary.

A case of von Hippel-Lindau disease with exudative maculopathy

Von Hippel-Lindau (VHL) disease is a rare multisystem familial tumor syndrome of autosomal dominant inheritance. Hallmark lesions include retinal, cerebellum and spinal cord hemangioblastomas, renal cell carcinomas, adrenal pheochromocytomas, angiomatous or cystic lesions of the kidneys, pancreas, and epididymis. We report a case of VHL disease in a 26-year-old patient who presented with exudative macular edema. Ocular and systemic studies revealed the presence of retinal and central nervous system hemangioblastomas, adrenal pheochromocytoma, multiple pancreatic, and kidney cysts. The retinal angiomas were successfully treated with argon laser photocoagulation and cryotherapy.

Spinal cord hemangioblastomas in von Hippel–Lindau disease

Retrospective data analysis. To clarify the clinical features and surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease (VHL). Clinical VHL Research Group in Japan, Japan. Forty-eight out of 66 patients with associated spinal cord hemangioblastoma among 142 VHL patients were retrospectively examined with respect to clinical features, accompanying lesions and outcome of surgical treatment. Among these 48 patients, 46 of them (95.8%) also had a central nervous system (CNS) hemangioblastoma at another site: 42 (87.5%) with cerebellar hemangioblastoma and 11 (22.9%) with brain stem hemangioblastoma. Twenty-three patients (47.9%) had more than one spinal cord hemangioblastoma. The 48 patients with spinal cord hemangioblastomas collectively had a total of 74 tumors. The tumor was accompanied with a syrinx in 64 and without it in 10 patients. Forty of the 48 patients underwent surgical treatment for their spinal cord hemangioblastomas, and 7 of these 40 underwent surgical treatment twice. When functional changes in the patients after these 47 operations were examined by postoperative evaluation by McCormick's classification, 39 of these operations (83.0%) resulted in improvement/no change and 8 (17.0%) in aggravation of symptoms. Von Hippel-Lindau disease patients bearing spinal cord hemangioblastomas mostly had a CNS hemangioblastoma at another site. These tumors can be removed in the majority of VHL patients without aggravation. In these patients, when the timing of treatment for spinal cord hemangioblastoma is determined, the probability of occurrence and treatment of other lesions should be considered.

Treatment of spinal cord hemangioblastoma by microoperations combined with embolization

To investigate the effects of microoperations combined with embolization in treatment of spinal cord hemangioblastoma. Thirty-six patients with spinal cord hemangioblastoma, 21 males and 15 females, aged 24.2 (12-48), underwent MRI and digital abstraction angiography. Twelve of them underwent pure microsurgical treatment and 24 of them underwent operation combined with remobilization. MRI showed clear boundary mass, and DSA showed round or oval high density images with clear boundary and definite supplying artery. 30 cases had their tumors totally removed; and 6 cases had their tumors subtotally removed. Complete functional recovery was seen in 27 of the 36 patients (67%). Four patients resumed their self-care ability,and 4 still needed someone's help. Embolization lowers the blood supply of the spinal cord hemangioblastoma, elevates the cure rate, and enlarges the indication of spinal cord hemangioblastoma operation.

Embolization of Hemangioblastomas

Object. In this study the authors compare the clinical outcomes after particle embolization of hemangioblastomas in the cerebellum and spinal cord. They also review the literature of similar cases. Methods. Seven patients with hemangioblastomas in the spinal cord (four patients) and cerebellum (three patients) underwent preoperative embolization at the authors' center. Magnetic resonance imaging and selective angiography studies as well as histological diagnoses were available in all patients. Embosphere particles (trisacryl gelatin microspheres) were used in all cases. The smallest particle diameter ranged from 100 to 300 μm at the beginning of embolization in all patients. The outcome of embolization was favorable in patients with spinal cord hemangioblastomas, but it was unfavorable for those with cerebellar hemangioblastomas; acute tumor bleeding and death occurred in all of the latter cases. The outcomes following embolization are very different for these two locations possibly because of the different capillary sizes. Conclusions. The authors no longer use particle embolization to treat cerebellar hemangioblastomas.

Intradural Extramedullary Leptomeningeal Hemangioblastomatosis and Paraneoplastic Limbic Encephalitis Diagnosed at Autopsy: An Unlikely Pair

Typical cerebellar or spinal cord hemangioblastoma is often solitary and has a benign clinical course, whereas disseminated hemangioblastomatosis is extremely rare. We report a 75-year-old man with disseminated "leptomeningeal" hemangioblastomatosis and concurrent paraneoplastic limbic encephalitis who died of this disease. The patient presented with gait problems and cognitive deficits, and was diagnosed with a cervical spinal tumor. Surgical resection achieved an apparent gross total resection, but later the patient began experiencing severe cognitive decline. His condition progressively deteriorated during the next few months, and he died 15 months after his initial surgery. Autopsy revealed disseminated extramedullary intradural hemangioblastomatosis with extensive leptomeningeal nodules involving the entire spinal cord, medulla, pons, and midbrain, as well as histologic features of limbic encephalitis. There was no evidence of von Hippel-Lindau disease, and no specific etiology was identified for the dementia. To the best of our knowledge, this is the first case of limbic encephalitis occurring in the setting of disseminated hemangioblastomatosis.

Intradural extramedullary leptomeningeal hemangioblastomatosis and paraneoplastic limbic encephalitis diagnosed at autopsy: an unlikely pair.

Typical cerebellar or spinal cord hemangioblastoma is often solitary and has a benign clinical course, whereas disseminated hemangioblastomatosis is extremely rare. We report a 75-year-old man with disseminated hemangioblastomatosis and concurrent paraneoplastic limbic encephalitis who died of this disease. The patient presented with gait problems and cognitive deficits, and was diagnosed with a cervical spinal tumor. Surgical resection achieved an apparent gross total resection, but later the patient began experiencing severe cognitive decline. His condition progressively deteriorated during the next few months, and he died 15 months after his initial surgery. Autopsy revealed disseminated extramedullary intradural hemangioblastomatosis with extensive leptomeningeal nodules involving the entire spinal cord, medulla, pons, and midbrain, as well as histologic features of limbic encephalitis. There was no evidence of von Hippel-Lindau disease, and no specific etiology was identified for the dementia. To the best of our knowledge, this is the first case of limbic encephalitis occurring in the setting of disseminated hemangioblastomatosis.

Hemorrhage after particle embolization of hemangioblastomas: comparison of outcomes in spinal and cerebellar lesions

In this study the authors compare the clinical outcomes after particle embolization of hemangioblastomas in the cerebellum and spinal cord. They also review the literature of similar cases. Seven patients with hemangioblastomas in the spinal cord (four patients) and cerebellum (three patients) underwent preoperative embolization at the authors' center. Magnetic resonance imaging and selective angiography studies as well as histological diagnoses were available in all patients. Embosphere particles (trisacryl gelatin micro-spheres) were used in all cases. The smallest particle diameter ranged from 100 to 300 microm at the beginning of embolization in all patients. The outcome of embolization was favorable in patients with spinal cord hemangioblastomas, but it was unfavorable for those with cerebellar hemangioblastomas; acute tumor bleeding and death occurred in all of the latter cases. The outcomes following embolization are very different for these two locations possibly because of the different capillary sizes. The authors no longer use particle embolization to treat cerebellar hemangioblastomas.

Diagnosis and treatment of spinal cord hemangioblastoma

To study the diagnosis and treatment of spinal cord hemangioblastoma. The clinical data of 42 patients with spinal cord hemangioblastoma who were operated on between 1997 and 2005 were analyzed. Spinal cord hemangioblastoma mostly showed space occupying lesions with clear boundary and Dd-DTPA homogenous enhancement by MRI. All the 42 patients underwent complete excision. 27 patients showed improvement of their symptoms, the 6 patients failed to show any change, and neurological deficits were aggravated in the 5 patients. MRI and DSA are helpful in qualitative and localized diagnoses of the spinal cord hemangioblastoma. Surgical outcomes are favorable.

Spinal Cord Hemangioblastoma : Diagnosis and Clinical Outcome after Surgical Treatment

Spinal cord hemangioblastoma is an uncommon vascular neoplasm with a benign nature and is associated with von Hippel-Lindau (VHL) disease in 20-30% of patients. Total removal of these tumors without significant neurological deficit remains a great challenge. The purpose of this study was to investigate the efficacy of VHL mutation analysis and to evaluate surgical outcome of patients with spinal cord hemangioblastomas. This study included nine patients treated for spinal cord hemangioblastomas at our institute between December 1994 and March 2006. There were four male and five female patients. Mean age was 37.8 years. The mean follow-up period was 22.4 months. Magnetic resonance imaging (MRI) of the complete neuraxis was done in all cases and VHL mutation analysis was performed in three cases for a definite diagnosis. Six patients had intramedullary tumor, and the remaining patients had intradural extramedullary lesions. Five patients were associated with VHL disease. The von Hippel-Lindau mutation analysis was done in three patients and two of them showed VHL gene abnormality. Tumors were located in the cervical cord in five cases and in the thoracic cord in four cases. All patients underwent surgical intervention, and total removal was achieved in six cases. All patients showed improvement or, at least, clinically stationary state. Surgical complications did not develop in any cases. Spinal hemangioblastoma in this series has been safely and effectively removed via a posterior approach. Postoperatively, clinical outcome was excellent in the majority of cases. The VHL mutation analysis was useful in patients with family history and in those with multiple hemangioblastomas.

Microsurgical Resection of Spinal Cord Hemangioblastomas

Hemangioblastomas are not uncommon intramedullary spinal cord neoplasms. They are highly vascular, benign tumors that occur either sporadically or in the presence of von Hippel-Lindau disease. Despite their exceptionally vascular nature, these lesions can consistently be resected completely and safely with minimal blood loss. We describe a microsurgical method for removal of these tumors from the spinal cord.

Minimally invasive surgery of the spinal cord hemangioblastoma

To clarify and discuss the diagnosis and minimally invasive surgery of spinal cord hemangioblastoma. The clinical data of 16 patients with spinal cord hemangioblastoma who were operated on between 1992 and 2001 were analyzed retrospectively with a review of the literature. Twelve patients were subjected to MRI examination, nine to spinal cord angiography, and six to preoperative embolization. Thirteen patients underwent complete excision, and three underwent partial excision due to massive bleeding and anterior location of the tumor in spinal cord. Eleven patients showed improvement of their symptoms, four no change at all, and tumor recurrence was seen in one patient with aggravation. MRI and spinal angiography are essential for preoperative diagnosis and surgical planning. Early diagnosis and microsurgical resection greatly preserve the neurological function of the patients. Preoperative embolization sometimes is helpful in surgery.

Spinal Cord Hemangioblastoma with Extensive Syringomyelia

We present the case of a 20-year-old male with intermittent right upper extremity numbness for 3 months. His pain perception and temperature sensation were severely disturbed. An incidental magnetic resonance imaging (MRI) finding of one small intramedullary enhancing nodule at spinal cord level T10-11 with long-segment syrinx formation suggested the diagnosis of spinal hemangioblastoma with syringomyelia. Surgical removal of the tumor and decompression of the spinal cord with opening of the syrinx were performed smoothly, and the pathology confirmed the diagnosis of spinal hemangioblastoma. Reviewing the literature, MRI is the examination of choice for spinal hemangioblastomas, and is helpful in preoperative planning and the differential diagnosis of spinal cord neoplasms and vascular lesions.

Surgical management of hemangioblastomas of the spinal cord.

Intramedullary hemangioblastomas are relatively rare intraspinal tumors. Total removal of these tumors without causing significant neurological deficit remains a great challenge. This study analyzed the preoperative characteristics, management, and outcome in patients with successful total removal of spinal intramedullary hemangioblastomas. Data from the medical records of patients with intraspinal hemangioblastomas treated from 1993 to 2003 were reviewed. The neurological function of these patients was graded and the preoperative data were recorded and correlated with clinical outcome. Ten patients with spinal intramedullary hemangioblastomas underwent microsurgical resection by the same neurosurgeon during the 10-year study period. They included 3 men and 7 women with age ranging from 20 to 49 years, with a mean age of 33 years. Five of the patients met the diagnostic criteria for von Hippel-Lindau disease. Preoperative neurological function was grade I in 6 patients, grade II in 3 patients and grade III in 1 patient. Immediate postoperative neurological function was worse in 3 cases, but all of these patients recovered their preoperative function within 3 weeks. At 3 months' follow-up, 9 patients had achieved functional grade I, and 1 patient with preoperative grade III function had improved to grade II. Detailed preoperative evaluation and skillful microsurgery are mandatory in successful total removal of intramedullary hemangioblastomas. The management of spinal hemangioblastomas associated with von Hippel-Lindau disease requires a cautious observation of clinical course and timely surgical intervention of symptomatic lesions to avoid possible neurological deficit.

Surgical strategy in spinal cord hemangioblastomas

The complete removal of spinal cord hemangioblastomas must be the neurosurgeon's ultimate goal. Over about 18 years, we have operated on 25 patients with spinal cord hemangioblastomas. This article reports our experience, which has improved with the advent of radiological techniques, primarily magnetic resonance imaging (MRI). At present, MRI is the best and, in most cases, the only examination to perform. Preoperative neurophysiology and improvements related to our progressive surgical experience have helped us to find surgical solutions for lesions in difficult locations. Surgical approach primarily depends on the anatomical location of a lesion. We achieved complete surgical resection in all cases but one patient who had undergone two surgeries elsewhere and had also received radiation therapy. The surgical strategy for spinal cord hemangioblastoma is specific: complete “en bloc” removal of the tumor, avoiding bleeding during dissection, and maintaining all vessels coursing around the tumor but not feeding it. Associated cysts are cured by tumor removal, and shunting even the huge intramedullary cavities is unnecessary.