Spinal Hemangioblastomas Research Articles

Spinal Haemangioblastomas: A Report of Two Cases

Background: Spinal haemangioblastomas are rare benign but potentially devastating vascular tumours.They may occur sporadically or as a component of Von Hippel-Lindau (VHL) disease. There is a dearth of information about these tumours among Nigerians. This study aims to evaluate the clinical profile and outcome of surgery as well as the challenges of management of these tumours in a resource-limited country.
 Methods: We retrospectively analysed data from the records of patients who had surgery for spinal haemangioblastoma at our center between January 2004 and December 2018.
 Results: We operated 2 cases of spinal haemangioblastomas during the study period (1 male, 1 female). The patients were aged 25 and 50years. Both patients presented with sensorimotor deficits but only one them had sphincteric dysfunction. The duration of symptoms was 2 weeks in one patient and 18 months in the other. The tumour was located in the thoracic region in both of them. One patient had extramedullary tumour while the had an intramedullary spinal tumour. Both patients were paraplegic at the time of surgery, for 3 days in one patient and more than 4 months in the second patient. Gross total tumour resection was achieved in both individual. The patient with short duration of symptoms and extramedullary tumour made a rapid post-operative neurological improvement while the one with long duration of symptoms and intramedullary tumour remained paraplegic until his demise 9 months after surgery.
 Conclusion: Spinal haemangioblastomas are rare tumours. Long duration of symptoms and intramedullary tumour location are predictors of unfavorable surgical outcome.

Minimally invasive resection of spinal hemangioblastoma: feasibility and clinical results in a series of 18 patients.

Hemangioblastomas are benign, highly vascularized tumors that can occur sporadically or as part of von Hippel-Lindau (VHL) disease. Traditionally, spinal hemangioblastomas have been surgically treated via an open approach. In recent years, however, minimally invasive techniques using tubular retractors have been increasingly applied in spine surgery. Such procedures involve less tissue trauma but are also particularly demanding for the surgeon, especially in cases of highly vascular tumors such as hemangioblastomas. The object of this study was to evaluate the safety and efficacy of minimally invasive resection of selected spinal hemangioblastomas. The authors conducted a retrospective single-center study of all patients who, between January 2010 and January 2018, had been operated on for spinal hemangioblastoma via a minimally invasive approach performed at the surgeon's discretion. The surgical technique is described and the pre- and postoperative neurological and imaging results were analyzed descriptively. The primary outcome was the postoperative compared to preoperative neurological condition (McCormick grade). The secondary outcomes were the extent of tumor resection and postoperative complications. Eighteen patients, 12 female and 6 male, harboring a total of 19 spinal hemangioblastomas underwent surgery in the study period. Seventeen patients had stable neurological findings with stable or improved McCormick grades (94.5%) at a mean of 4.3 months after surgery. One (5.5%) of the 18 patients developed progressive neurological symptoms with a worsened McCormick grade that did not improve in the long-term follow-up. Sixteen of the 18 patients had VHL disease, whereas 2 patients had sporadic spinal hemangioblastomas. In all patients, postoperative MRI showed complete resection of the tumors. No other surgery-related perioperative or postoperative complications were recorded. A minimally invasive approach for the resection of selected spinal hemangioblastomas is safe and allows complete tumor resection with good clinical results in experienced hands.

Spinal Hemangioblastomas: Clinical Presentation, Radiology, and Treatment

Spinal Hemangioblastomas: Clinical Presentation, Radiology, and Treatment

Spinal Hemangioblastomas: Clinical Presentation, Radiology, and Treatment

Sayyahmelli, Sima MD; Aycan, Abdurrahman MD; Erginoglu, Ufuk MD; Başkaya, Mustafa K. MD Author Information

Clinical analysis of syringomyelia resulting from spinal hemangioblastoma in a single series of 38 consecutive patients

objectiveSyringomyelia was predominantly caused by Chiari malformation or intramedullary ependymoma. The goal of this study was to identify factors related to clinical outcomes and spinal hemangioblastoma (SH)-induced syringomyelia formation in a single series of patients. Patient and methodsThirty-eight patients with SH were treated with microsurgery from January 2013 to December 2018. Clinical features and related factors were retrospectively analyzed in SH patients with and without syringomyelia. ResultsOut of the total number of SH patients, 21 presented with remarkable syringomyelia, resulting in an incidence of 55.26% (21/38).Gross total resection was achieved in 36 cases (94.73%), and subtotal resection was obtained in 2 patients (5.27%). Neurological symptoms improved in 34 patients, remained stable in 2 patients and were aggravated in 2 cases during follow-up. In addition, there was a notable difference between the location of tumors and syringomyelia (P < 0.05). Syringomyelia occurred more frequently in the cervical segment than in any other spinal segment. Moreover, there was an association between symptom duration and clinical prognosis (P < 0.05). Ordinal regression analysis showed that the prognosis of middle duration groups (6–12 months) was better than early groups (0–6 months, p < 0.05, OR 20.21, 95%CI 2.34–336.97) and late groups (>12 months, p < 0.05, OR 11.54, 95%CI 1.30–102.21). Syringomyelia collapse or reduction occurred between two weeks and 15 months after surgery. An improvement of spinal function grade after surgery was more significant in syringomyelia reduction groups (p < 0.05). ConclusionsThe prevalence of syringomyelia due to SH is considerably high, and the initial clinical presentation of syringomyelia resulting from SH should be emphasized. Satisfactory outcomes were achieved by effective surgery in affected patients.

Dysphagia: an unusual presentation of Von Hippel-Lindau disease

A 25-year-old man presented with 2-week history of progressively worsening dysphagia to solids and liquids. There was no history of anorexia or significant weight loss. On examination, his blood pressure...

Microsurgical Resection of Spinal Cord Hemangioblastoma: 2-Dimensional Operative Video.

This video demonstrates microsurgical resection of spinal cord hemangioblastoma. Hemangioblastomas are rare, benign, highly vascularized tumors classified as grade I according to World Health Organization classification systems. About 3% of all intramedullary tumors are hemangioblastomas.1,2 Spinal cord hemangioblastomas are either sporadic3,4 or manifestations of von Hippel-Lindau (VHL) disease in 20% to 45% of patients.5,6 A 30-year-old male presented with sudden onset urinary incontinence. Magnetic resonance imaging showed contrast enhancing intramedullary tumor with adjacent cyst in T11, and syringomyelia extending to C1. Surgical resection followed rules that apply to resection of arteriovascular malformations: coagulation of arterial feeders precedes the coagulation of the draining vein, which is preserved until the end of surgery.2,4,5,7,8 First, posterior midline myelotomy was performed and the tumor cyst was drained in order to develop a dissection plane. Following this, we continuously separated dorsal nerve roots from the tumor nodule using microsurgical technique. The key step in tumor resection is devascularization of the tumor, achievable in 2 ways.2,7,9-13 The circumferential detachment of the normal pia from the tumor pia is crucial in developing a plane of dissection. The coagulation and division of arterial feeders while preserving the drainage vein further devascularizes the tumor. Once the tumor mural nodule was detached from the spinal cord, the drainage vein was coagulated last and the tumor was removed. The patient fully recovered from his incontinence and was neurologically intact. Screening for VHL disease was negative. Written consent was obtained directly from the patient.

Management and outcomes of pregnant patients with central nervous system hemangioblastoma

The study aims to assess the management and maternal and fetal outcomes of pregnancies complicated by central nervous system (CNS) hemangioblastoma. Twenty-four female patients with CNS hemangioblastoma, who were pregnant in a tumor-burden status, were identified. Their medical charts, treatments, and follow-up materials were carefully reviewed. Of the included 24 CNS patients with hemangioblastoma (14 intracranial and 10 spinal hemangioblastomas), 5 patients (20.8%) were diagnosed with Von Hippel-Lindau disease (VHL). The median age of these patients at admission was 27.5 years. Intracranial hypertension was a common presenting symptom for patients with intracranial hemangioblastoma and was observed in 85.7% (12/14) of cases; the other 10 patients with spinal hemangioblastomas all suffered from paresthesia. Overall, 66.7% (16/24) of patients with CNS hemangioblastoma went through the gestational course with conventional observation; 16.6% (4/24) of patients accepted a ventriculo-peritoneal shunt (VPS) to delay the tumor resection; and 16.7% (4/24) of patients needed urgent tumor resection even when symptomatic treatments were given. Variable symptom improvement was seen when patients had follow-up visits at a median of 32.5 months. No maternal death or tumor recurrence was identified. For the fetal prognoses, one (4.2%) pregnancy ended in a spontaneous miscarriage and for (16.7%) pregnancies were interrupted; the other 19 (79.2%) live births were in good status without any congenital malformations. Symptomatic treatment was the first choice for pregnant patients with CNS hemangioblastoma. When needed, urgent tumor resection could be safely achieved with careful maternal and fetal monitoring. Both maternal and fetal prognoses were favorable during follow-up.

Long-term outcome and prognostic factors of intramedullary spinal hemangioblastomas

Spinal hemangioblastomas constitute 1.6-5.8% of all spinal cord tumors. Microsurgical excision of these tumors is challenging. The purpose of this study is to analyze the neurological improvement and long-term functional outcome of spinal hemangioblastomas. This retrospective study included 15 patients who underwent surgery for intramedullary spinal hemangioblastoma at the Department of Neurosurgery of Sri Chitra Tirunal Institute for Medical Sciences and Technology from January 2001 to June 2014. Eight patients (53%) were diagnosed to have von Hippel-Lindau (vHL) disease. Eight (53%) of them were females, and seven were males (47%). Mean age was 33.8years (16-55years). Duration of illness ranged from 2weeks to 4years, and average duration was 10.5months. Most common symptom was motor weakness followed by sensory disturbances, pain, and bladder incontinence. Six (85.7%) sporadic spinal hemangioblastomas were in McCormick grade I; whereas, 7 (87.5%) of vHL spinal hemangioblastomas were in grade II or above. In the immediate postoperative period, three patients noticed improvement in their motor weakness. Six patients (40%) experienced deterioration of preoperative neurological status in the immediate postoperative period. Three of them were sporadic tumors, and others had vHL syndrome. Favorable long-term outcome was achieved in 80% of cases. Though neurological deterioration is common after surgical resection of spinal hemangioblastomas, majority of them are reversible. Long-term functional outcome is favorable with minimal postoperative morbidities. Both sporadic- and vHL-associated tumors share common clinical and radiological features, and neurological outcome is equally good in both.

Posterior approach for anteriorly located cervical spinal cord hemangioblastomas: technical note.

The surgical management of anteriorly located spinal cord hemangioblastomas remains a challenge. Different approaches have been published, of which the anterior approach seems to be the most obvious and commonly used. A posterior approach might be more suitable in certain patients, especially in cases of cystic hemangioblastomas. The authors present 3 cases of anterior spinal hemangioblastomas, which were all resected via a posterior approach. The authors discuss the rationale for choosing this approach and explain the technique in detail.

Sporadic hemangioblastoma of the film terminale with peritumoral cyst

Background:Spinal hemangioblastoma originating from the film terminale are rare tumors. Here, we present a film terminale hemangioblastoma and review the appropriate literature.Case Description:A 37-year-old female presented with bilateral lower extremity pain without a focal neurological deficit. The magnetic resonance (MR) image demonstrated an intradural spinal tumor at the L1 level, which was accompanied by peritumoral cysts. In addition, there were multiple surpentine flow voids (e.g., consistent with torturous and convoluted vessels), which is typical for hemangioblastoma. At surgery, a spinal hemangioblastoma originating from the film terminale with peritumoral cysts at the L1 level was fully excised without producing a focal postoperative neurological deficit. Histological examination revealed stromal cells with vacuolated cytoplasm and small nuclei in a rich capillary network accompanied by several enlarged vessels. These finding were compatible with a hemangioblastoma.Conclusions:We reported a rare case of a hemangioblastoma originating from the conus presenting at the L1 level. Complete surgical resection was accomplished without any motor deficit.

Severe paralysis after spinal hemangioblastoma resection: A case report

Severe paralysis after spinal hemangioblastoma resection: A case report

Microsurgical Treatment of Sporadic and von Hippel-Lindau Disease Associated Spinal Hemangioblastomas: A Single-Institution Experience.

Study DesignRetrospective cohort study.PurposeTo examine the clinical profile and surgical complications in patients with spinal hemangioblastomas and to evaluate the long-term outcome in them.Overview of LiteratureAlthough considered to be histologically benign, hemangioblastomas may cause significant neurological deficits. The proportion of spinal hemangioblastomas associated with von Hippel–Lindau (VHL) disease has been estimated be 13%–59%. Preoperative neurological function correlates with postoperative neurological status. Studies have shown no difference in outcomes between sporadic and VHL-associated spinal hemangioblastomas.MethodsThis retrospective study included 14 consecutive patients treated for spinal hemangioblastomas at our institute between January 2000 and June 2013. The mean follow-up period was 5 years. Magnetic resonance imaging of the complete neuraxis was performed in all cases, and preoperative embolization was performed in two cases.ResultsIn total, 14 patients underwent 18 surgeries, of which 15 were for spinal hemangioblastomas. Of all the patients, 86% had motor weakness and 79% presented with sensory disturbances. Preoperative McCormick functional grades were grade I in 7 (50%), grade II in 3 (21%), and grade III in 4 (29%) patients; 50% patients were diagnosed with VHL disease. All patients underwent complete resection of the tumor. Eight patients experienced deterioration in their neurological status in the immediate postoperative period; among them, five had gradual improvement. At 5-year follow-up, 11 (78.57%) patients showed good functional outcomes.ConclusionsMicrosurgical excision of spinal hemangioblastomas can cause postoperative morbidity, mainly in the form of neurological deterioration. Almost half of our patients had deterioration in the McCormick grade in the immediate postoperative period. However, a complete microsurgical excision can result in good long-term functional outcomes, as most of the immediate postoperative neurological deterioration in our patients was reversible. There was no difference in the long-term functional outcomes between sporadic and VHL-associated spinal hemangioblastomas.

Cystadenoma of the Rete Testis in a Patient With von Hippel-Lindau Disease

von Hippel-Lindau disease is a rare, inherited multicystic disorder that is characterized by several benign and malignant neoplasms (Odrzywolski, 2010). Classically, the disease manifests itself in a broad spectrum, including renal cell carcinomas, intracranial and spinal hemangioblastomas, endolymphatic sac tumors, renal and pancreatic cysts, and pheochromocytomas. Another important, but commonly forgotten manifestation is the cystadenoma of the rete testis.

Rapid Worsening of Symptoms and High Cell Proliferative Activity in Intra- and Extramedullary Spinal Hemangioblastoma

Study DesignA retrospective analysis of a prospective database.ObjectiveTo compare preoperative symptoms, ambulatory ability, intraoperative spinal cord monitoring, and pathologic cell proliferation activity between intramedullary only and intramedullary plus extramedullary hemangioblastomas, with the goal of determining the optimal timing for surgery.MethodsThe subjects were 28 patients (intramedullary only in 23 cases [group I] and intramedullary plus extramedullary in 5 cases [group IE]) who underwent surgery for spinal hemangioblastoma. Preoperative symptoms, ambulatory ability on the McCormick scale, intraoperative spinal cord monitoring, and pathologic findings using Ki67 were compared between the groups.ResultsIn group IE, preoperative motor paralysis was significantly higher (100 versus 26%, p < 0.005), the mean period from initial symptoms to motor paralysis was significantly shorter (3.5 versus 11.9 months, p < 0.05), and intraoperative spinal cord monitoring aggravation was higher (65 versus 6%, p < 0.05). All 5 patients without total resection in group I underwent reoperation. Ki67 activity was higher in group IE (15% versus 1%, p < 0.05). Preoperative ambulatory ability was significantly poorer in group IE (p < 0.05), but all cases in this group improved after surgery, and postoperative ambulatory ability did not differ significantly between the two groups.ConclusionsIntramedullary plus extramedullary spinal hemangioblastoma is characterized by rapid preoperative progression of symptoms over a short period, severe spinal cord damage including preoperative motor paralysis, and poor gait ability compared with an intramedullary tumor only. Earlier surgery with intraoperative spinal cord monitoring is recommended for total resection and good surgical outcome especially for an IE tumor compared with an intramedullary tumor.

Image-guided stereotactic radiosurgery for treatment of spinal hemangioblastoma.

OBJECTIVE Stereotactic radiosurgery (SRS) has been an attractive treatment option for hemangioblastomas, especially for lesions that are surgically inaccessible and in patients with von Hippel-Lindau (VHL) disease and multiple lesions. Although there has been a multitude of studies examining the utility of SRS in intracranial hemangioblastomas, SRS has only recently been used for spinal hemangioblastomas due to technical limitations. The purpose of this study is to provide a long-term evaluation of the effectiveness of image-guided radiosurgery in halting tumor progression and providing symptomatic relief for spinal hemangioblastomas. METHODS Between 2001 and 2011, 46 spinal hemangioblastomas in 28 patients were treated using the CyberKnife image-guided radiosurgery system at the authors' institution. Fourteen of these patients also had VHL disease. The median age at treatment was 43.5 years (range 19-85 years). The mean prescription radiation dose to the tumor periphery was 21.6 Gy (range 15-35 Gy). The median tumor volume was 0.264 cm3 (range 0.025-70.9 cm3). Tumor response was evaluated on serial, contrast-enhanced CT and MR images. Clinical response was evaluated by clinical and imaging evaluation. RESULTS The mean follow-up for the cohort was 54.3 months. Radiographic follow-up was available for 19 patients with 34 tumors; 32 (94.1%) tumors were radiographically stable or displayed signs of regression. Actuarial control rates at 1, 3, and 5 years were 96.1%, 92.3%, and 92.3%, respectively. Clinical evaluation on follow-up was available for 13 patients with 16 tumors; 13 (81.2%) tumors in 10 patients had symptomatic improvement. No patient developed any complications related to radiosurgery. CONCLUSIONS Image-guided SRS is safe and effective for the primary treatment of spinal hemangioblastomas and is an attractive alternative to resection, especially for those with VHL disease.

Spinal cord hemangioblastomas: significance of intraoperative neurophysiological monitoring for resection and long-term outcome.

OBJECTIVE Spinal cord hemangioblastomas are rare benign tumors developing either sporadically or as part of von Hippel-Lindau (VHL) disease. Generally, resection is the treatment of choice. However, the significance of intraoperative neurophysiological monitoring (IONM) for resection and postoperative outcome is still controversial. The authors analyzed the surgical and clinical courses of patients who had undergone resection of spinal cord hemangioblastoma, with special attention to preoperative imaging, the use of IONM, and short- and long-term outcomes. METHODS A series of 24 patients (male/female 1:1, lesion sporadic/associated with VHL 2.4:1) who had undergone 26 operations for the resection of 27 spinal cord hemangioblastomas was analyzed. All patients had undergone pre- and postoperative contrast-enhanced MRI. In all cases, microsurgical tumor removal had been performed under continuous IONM of both somatosensory and transcranial motor evoked potentials as well as electromyographic recording. Clinical characteristics, imaging findings, and operative records were retrospectively analyzed. Outcome parameters included short- and long-term status as regards sensorimotor deficits and a questionnaire on general performance, patient satisfaction, and Oswestry Disability Index (ODI) at the end of the follow-up period. The impact of IONM findings on postoperative deficits and outcome parameters as well as risk factors affecting functional prognosis was statistically assessed. RESULTS Preoperative symptoms (mean duration 16.2 ± 22.0 months) included sensory changes (100.0%), pain (66.7%), spinal ataxia (66.7%), motor deficit (41.7%), and bladder/bowel dysfunction (12.5%). Average age at the first operation was 36.8 ± 12.8 years. Most tumors (21 intramedullary, 6 intra- and/or extramedullary) were located dorsally (92.6%) and cervically (77.8%) and were accompanied by peritumoral edema and/or syringomyelia (81.5%). Tumor resection was achieved via laminectomy for 15 tumors, hemilaminectomy for 5, laminoplasty for 6, and interlaminar approach for 1. Gross-total resection was accomplished for 26 tumors (96.3%) with no local tumor recurrence during follow-up. Intraoperative neurophysiological monitoring was nonpathological in 11 operations (42.3%) and pathological in 15 (57.7%). Patients with nonpathological IONM had significantly fewer new sensorimotor deficits (p = 0.005). Long-term follow-up evaluation (mean 7.9 ± 4.0 years postoperatively, 7 patients lost to follow-up) revealed a stable or improved McCormick myelopathy grade in 88.2% of the patients, and 88.2% reported a stable or improved overall outcome according to Odom's criteria. Long-term general performance was excellent with 88.2% having a WHO/Eastern Cooperative Oncology Group (ECOG) Performance Status grade ≤ 1, 76.5% a Karnofsky Performance Scale score ≥ 80, and 70.6% a Barthel Index (BI) of 100. The mean ODI (11.4% ± 12.5%) indicated only minimal disability. There was a significant correlation between pathological IONM findings and a worse long-term status according to the BI and ODI (p = 0.011 and 0.024, respectively). Additionally, VHL disease was a risk factor affecting functional prognosis (p = 0.044). CONCLUSIONS Microsurgical removal of spinal cord hemangioblastomas with IONM facilitates a satisfying long-term outcome for patients. Nonpathological IONM findings are associated with a lower risk of new sensorimotor deficits and correlate with a better overall long-term outcome. von Hippel-Lindau disease is a risk factor for a worse long-term prognosis.

Potential role of (68)Ga-DOTATOC PET/CT in screening for pancreatic neuroendocrine tumour in patients with von Hippel-Lindau disease.

Neuroendocrine tumours of the pancreas (pNET) are observed in 8-17% of patients with von Hippel-Lindau disease (vHLD), and 11-20% of these patients develop metastatic disease. MRI and CT have a very high resolution; however, their sensitivity and specificity for the detection of pNET amongst cystic lesions in the pancreas of vHLD patients are generally considered insufficient. In contrast, (68)Ga-DOTATOC PET/CT demonstrates a high sensitivity for the diagnosis and staging of neuroendocrine tumours. In this study we investigated the potential role of (68)Ga-DOTATOC PET/CT in screening of patients with vHLD. (68)Ga-DOTATOC PET/three-phase contrast-enhanced CT was performed according to guidelines in all consecutive vHLD patients between January 2012 and November 2015. All patients underwent additional MRI imaging of the abdomen, spine, and head. Chromogranin A (CgA) was determined at the time of the PET/CT examination. A lesion seen on (68)Ga-DOTATOC PET in the pancreas was defined as positive if the uptake was visually higher than in the surrounding tissues. Lesions were quantified using maximum SUV. Overall, 20 patients (8 men, 12 women; mean age 44.7 ± 11.1years) were prospectively examined. Genetically, 12 patients had type 1 vHLD and 8 had type 2 vHLD. (68)Ga-DOTATOC PET/CT detected more pNET than morphological imaging (CT or MRI): 11 patients (55%; 8 type 1, 3 type 2) vs. 9 patients (45%; 6 type 1, 3 type 2). The concentration of CgA was mildly elevated in 2 of 11 patients with pNET. The mean SUVmax of the pancreatic lesions was 18.9 ± 21.9 (range 5.0-65.6). Four patients (36.4%) had multiple pNETs. The mean size of the lesions on CT and/or MRI was 10.4 ± 8.3mm (range 4-38mm), and 41.1% were larger than 10mm. In addition, somatostatin receptor-positive cerebellar and spinal haemangioblastomas were detected in three patients (SUVmax 2.1-10.1). One patient presented with a solitary somatostatin receptor-positive lymph node metastasis. pNETs were observed more frequently in vHLD type 1 than type 2 (66.7% vs. 37.5%, p = 0.089). None of the patients showed progressive disease during follow-up. In this study, (68)Ga-DOTATOC PET detected pNETs in a higher proportion of patients with vHLD than found in previous studies with (111)In-octreoscan, the imaging method recommended by the NCCN. We therefore suggest (68)Ga-DOTATOC PET/CT as the more sensible screening tool.

Sporadic intramedullary hemangioblastoma of the spine: a single institutional review of 21 cases

Introduction: Sporadic intramedullary spinal cord hemangioblastomas are rare, vascular tumours that can present with significant neurological dysfunction. Surgical resection is the standard treatment for these lesions, and we report our institution's experience with patients who underwent surgery for sporadic intramedullary spinal cord hemangioblastomas.Methods: We retrospectively reviewed 21 patients (14 male, 7 female) who underwent a total of 23 surgeries for sporadic intramedullary spinal cord hemangioblastoma between 1996 and 2014.We recorded demographic, operative and postoperative factors.Results: The median age was 45 years (IQR: 28). All tumours were present in the cervical (13 of 21, 62%) or thoracic (nine of 21, 43%) spine (one patient had tumours in both locations). Thirteen patients (62%) also had concurrent syringomyelia spanning a median of 11 vertebral levels (IQR: 5). Total resection was achieved in all but one surgery, and no cases involved intraoperative complications. However, postoperative complications occurred after five cases. Median follow-up was 12 months (IQR: 38). Twelve patients (57%) experienced long-term dysfunction after surgery, and two patients experienced recurrence requiring a second surgery.Conclusions: Maximal safe surgical resection should be considered as a primary treatment modality for individuals with intramedullary spinal cord hemangioblastomas.

Diagnosis and microsurgical treatment of spinal hemangioblastoma.

Spinal cord hemangioblastomas are rare benign tumors, with difficult surgical management and poor prognosis due to high vascularization. We aim to evaluate the diagnostic methods and microsurgical treatment of spinal cord hemangioblastoma. This retrospective study assessed 25 patients treated for spinal hemangioblastoma using microsurgery at Beijing Tiantan Hospital and Department of Neurosurgery, The General Hospital of Chinese People's Armed Police Forces, between October 2008 and October 2013. Clinical, imaging, and treatment data were collected. Meanwhile, efficacy was assessed with the McCormick grading system for spinal cord function. The symptoms lasted 17.0±15.1months. Sixteen (64%) patients were suffering from von Hippel-Lindau disease; magnetic resonance imaging revealed the lesions in all patients. Intraoperative fluorescence angiography was helpful in identifying the feeding arteries and draining veins. Total tumor removal was achieved in all subjects. Patients were followed up for 21.3±8.5months. One week after surgery, neurological symptoms were improved in 22 patients, remained stable in 2 patients, and were aggravated in 1. The latter patient began to recover 7-10days after surgery and was completely recovered within a month. At the last follow-up, all patients were alive, and all showed a McCormick grade ≤II. Microsurgery seems effective in the treatment of spinal cord hemangioblastoma. Intraoperative fluorescence angiography is helpful in defining the resection scope, to reduce intraoperative bleeding and prevent spinal swelling, which results in improved success rate.