Intramedullary Hemangioblastoma Research Articles

Prognostic Factors of Spinal Intramedullary Hemangioblastoma : Analysis of Surgical Outcomes and Tumor Characteristics.

Spinal intramedullary hemangioblastoma is a rare and highly vascularized benign tumor. The characteristics of the tumor, its corresponding location, and surgical outcomes remain unknown. The purpose of this study was to identify risk factors and strategies for neurologic deterioration following hemangioblastoma surgery. A comprehensive retrospective analysis was undertaken to evaluate patients who underwent surgical intervention for intramedullary hemangioblastoma at our institution from 1993 to 2022. Patients with at least one year of follow-up data were included. The analysis covered patient demographics, pre- and post-operative Modified McCormick Scale (MMCS), tumor location, and tumor size. This study included 25 cases. One-year after surgery, neurological deterioration was observed in 5 (20.0%) cases, and neurological improvement was found in 9 (36.0%) cases. Five cases were ventrally located, and twelve cases were dorsally located. Ventrally located cases were larger in tumor axial size (p = 0.029) than dorsal location tumors, resulting in poorer follow-up MMCS and a higher prevalence of von Hippel-Lindau syndrome (VHL) (p = 0.042). Three of them were confirmed to be supplied by the anterior spinal artery. In the case of dorsally located cases, there was no neurologic deterioration. In intramedullary spinal cord hemangioblastomas, cases located ventrally had a higher incidence of neurological deterioration following surgery than those located dorsally or in intramedullary extramedullary cases. Ventrally located hemangioblastomas were larger than those in other locations. They were mainly supplied by the anterior spinal artery in VHL patients.

Resection of Intramedullary Hemangioblastoma: Timing of Surgery and Its Impact on Neurological Outcome and Quality of Life.

Background and Objectives: Spinal intramedullary hemangioblastomas (SIMH) are benign vascular lesions that are pathological hallmarks of von Hippel-Lindau disease (vHL) and constitute the third most common intramedullary neoplasm in adults. So far, maximal and safe resection is the first choice of treatment. However, as SIMH show no malignant transformation, it remains unclear whether surgical resection is beneficial for all patients. Materials and Methods: We retrospectively analyzed the surgical outcomes of 27 patients who were treated between 2014 and 2022 at our neurosurgical department and investigated potential risk factors that influence the surgical outcome. Pre- and postoperative neurological status were classified according to the McCormick scale. Furthermore, surgical quality indicators, such as length of hospital stay (LOS; days), 90-day readmissions, nosocomial infections, and potential risk factors that might influence the surgical outcome, such as tumor size and surgical approach, have been analyzed. In addition to that, patients were asked to fill out the EQ-5D-3L questionnaire to assess their quality of life after surgery. Results: Surgery on SIMH patients that display no or minor neurological deficits (McCormick scale I or II) is associated with a favorable postoperative outcome and overall higher quality of life compared to those patients that already suffer from severe neurological deficits (McCormick scale III or IV). Conclusion: Early surgical intervention prior to the development of severe neurological deficits may offer a better neurological outcome and quality of life.

Dorsal hemangioblastoma with holocord syringomyelia: case report

Introduction: Intramedullary hemangioblastomas are usually accompanied by syringomyelia. However, a holocord syringomyelia is rare. The most common cause of syringomyelia continues to be Chiari disease, and only 10 cases of hemangioblastomas with holocord syringomyelia reported so far. Case report: We present a case of a 35-year-old patient with a two-month history of cervicobrachialgia at the C7-C8 root level, previously preceded by pain at the D1-D2 level. Cervico-dorso-lumbar MRI revealed a medullar tumor with hyper-uptake mural nodule at the conus medullaris level accompanied by an extensive syringomyelic cavity from C5 to L1 compatible with medullary hemangioblastoma. The patient underwent surgery for tumor resection with subsequent resolution of her painful symptoms. Conclusion: It is important to note that the surgery is aimed at treating the origin of this syringomyelia and not the syringomyelia itself. Although the majority of patients with holocord syringomyelia have Chiari as its cause, the possibility of focal spinal intramedullary tumors as being responsible for syringomyelia should not be forgotten.

Intramedullary hemangioblastoma. Case report

Hemangioblastomas are benign tumors that develop from the vessels of the central nervous system and can be a manifestation of autosomal dominant von Hippel-Lindau disease. Statistically, they account for 1.5‒2.5% of all intracranial tumors and 2‒15% of spinal cord tumor lesions. There are very few publications on the intramedullary localization of these neoplasms.The patient, 45 years old, a serviceman, presented with complaints of headache, slight unsteadiness of gait, as well as slight weakness in the right extremities, more pronounced in the right upper extremity, periodic numbness of the upper extremities, which progressed and made further service impossible. On neurological examination: pupils D=S, light reflexes were brisk, eye movement was fully preserved and horizontal nystagmus. BNI - PS - I, BNI - NS - I. HB - I. GR - I. Swallowing and phonation were fully preserved. There was a slight hemiparesis on the right. Hemihypesthesia on the right was more prominent in the upper extremity. Ataxia of mixed genesis. Pelvic organs function was preserved. Periodic constipation for up to 7 days. Magnetic resonance imaging revealed a multifocal brain lesion. Supratentorially, a cystic mass measuring 2.60×2.12×2.14 cm with a solid component up to 1.5 cm in the diameter was detected in the of the thickened corpus callosum. Intramedullary cystic-solid lesion of the medulla oblongata extending to the cervical spinal cord with conventional dimensions of the solid component 1.76×1.23×1.57 cm and the cystic component was 1.52×1.62×1.22 cm. Magnetic resonance imaging of the cervicothoracic region of the spinal cord revealed significant hydromyelitic expansion of the central canal from the C2 level to the Th3 level (up to 10mm in the diameter). Endovascular embolization of the neoplasm with a liquid embolic agent (Phil) and following microsurgical en bloc tumor resection were performed.Hemangioblastomas with an intramedullary location are extremely difficult and risky for the surgical removal. The presence of a cystic component in the hemangioblastoma structure or perifocally gives a chance to remove such a neoplasm avoiding risk of functional deterioration. Preoperative endovascular obliteration of hemangioblastoma vascularity is considered as an effective measure, although it is associated with a risk of cerebral ischemia in corresponding brain structures. Another crucial issues of intramedullary hemangioblastoma surgery includes multimodal intraoperative neuromonitoring and maximum possible intraoperative magnification to minimize injury of adjacent brain structures. Present practice shows that multiple intramedullary cysts in the spinal cord commonly regressing after neoplasm removal and leads to improvement of neurological deficit within a relatively short period of time.Conclusions. Hemangioblastomas with intramedullary growth and perifocal cyst can be surgically removed with a good functional result and comprehensive approach includes preoperative selective angiography with endovascular embolization, multimodal intraoperative neuromonitoring and appropriate microsurgical technique.

Surgical Treatment of Intramedullary Hemangioblastomas: Current State of Problem (Review).

Intramedullary hemangioblastomas (HAB) refer to very rare highly vascularized vascular spinal cord tumors associated with various neurological disorders. Effective HAB therapy to a greater extent depends on diagnostic accuracy and the absence of intra- and postoperative complications.The present study is a review of publications concerned with modern diagnostic and therapeutic techniques to control spinal HAB. The authors showed that perfusion computed tomography, computed tomographic angiography, and magnetic resonance angiography can be reasonably used for diagnosis and differentiation in a number of HAB due to their high vascularization. Preoperative embolization significantly reducing intraoperative bleeding risks is highly efficient. Some authors recommend this procedure in case of large lesions and high risks of intraoperative bleeding. The review also considered intraoperative imaging of a tumor and its feeding vessels using indocyanine green providing inspectability over the total tumor resection and clear imaging of tumor vascular architecture. The advantages and restrictions of the mentioned procedures were described.

Visual fluorescence combined with laser spectroscopy in surgery for intramedullary spinal cord tumors

Surgical treatment of intramedullary spinal cord tumors is aimed at total resection of tumor with maximum preservation of neurological and functional status. In some cases, intramedullary tumors have unclear dissection plane or gliosis zone. This area is not a tumor and does not require resection. However, it is difficult to distinguish visually intact spinal cord tissue and tumor at the last surgical stages. Thus, we evaluated the effectiveness of fluorescence combined with laser spectroscopy in surgical treatment of intramedullary spinal cord tumors. To determine the effectiveness of visual fluorescence combined with laser spectroscopy in surgery for intramedullary spinal cord tumors. There were 850 patients with intramedullary spinal cord tumors for the period 2001-2019. In 35 cases, intraoperative fluoroscopy with laser spectroscopy were used. All patients underwent a comprehensive pre- and postoperative clinical and instrumental examination (general and neurological status, McCormick grade, spinal cord MRI). Carl Zeiss OPMI Pentero microscope with a fluorescent module was used for intraoperative fluorescence diagnosis. A domestic preparation 5-ALA «ALASENS» (State Research Center NIOPIK, Moscow, Russia) was used for induction of visible fluorescence. Laser spectroscopy was carried out using a LESA-01-BIOSPEK spectrum analyzer. Morphological analysis of intramedullary spinal cord tumors was performed in the neuromorphology laboratory of the Burdenko Neurosurgery Center. Intramedullary anaplastic ependymoma and astrocytoma, as well as conventional ependymoma were characterized by the highest index of 5-ALA accumulation. Intramedullary hemangioblastoma and cavernoma do not accumulate 5-aminolevulinic acid due to morphological structure of these tumors. In particular, there are no cells capable of capturing and processing 5-ALA in these tumors. Sensitivity of visual fluorescence combined with laser spectroscopy varies from 0% to 100% depending on the histological type of tumor: hemangiogblastoma and cavernoma - 0%, low-grade astrocytoma - 70%, high-grade astrocytoma - 80%, ependymoma - 92%, anaplastic ependymoma 100%. Dissection plane is absent in anaplastic ependymoma, high-grade astrocytoma. We often observed gliosis during resection of ependymoma. This tissue is not a part of tumor. Intraoperative metabolic navigation with neurophysiological monitoring are advisable for total tumor resection in case of unclear dissection plane and peritumoral gliosis. Visual fluorescence combined with laser spectroscopy is a perspective method for intraoperative imaging of tumor remnants and total resection of intramedullary spinal cord tumors with minimum risk of neurological impairment.

Imaging Diagnosis of Von Hippel-Lindau Syndrome

Von Hippel-Lindau (VHL) syndrome is a group of rare autosomal dominant hereditary diseases that involve multiple organs. Due to its high recurrence rate and complex and diverse clinical manifestations, VHL is prone to being either misdiagnosed or missed entirely. Therefore, patients with VHL syndrome have a poor prognosis. This study reports the details of 2 patients, a 55-year-old male and a 37-year-old female, who were diagnosed as having VHL syndrome with a positive family history. The male patient presented with upper abdominal discomfort 2 years prior to the current study, and was diagnosed in another hospital as having a space-occupying lesion at the head of the pancreas. After undergoing hemangioblastoma resection of the right cerebellar hemisphere 1 month ago, he was admitted to the hospital for recent aggravation of upper abdominal discomfort to receive further diagnosis and treatment. The female patient previously underwent right ovarian cystectomy and T5-T6 intramedullary hemangioblastoma resection. She was diagnosed at another hospital as having pancreatic cancer, and was admitted to the hospital for recent aggravation of upper abdominal discomfort to receive further diagnosis and treatment. The diagnosis and treatment of VHL syndrome is currently relatively difficult. It poses a substantial threat to patients and their families. The early and timely diagnosis and treatment of VHL syndrome can improve patients' prognosis and rates of survival.

Surgical treatment of sporadic and von Hippel–Lindau syndrome-associated intramedullary hemangioblastomas

ObjectIntramedullary hemangioblastomas are rare lesions. They can be related to von Hippel–Lindau syndrome or they may be sporadic. This study describes surgical treatment for this infrequent tumor. MethodsTwelve consecutive patients received surgery to remove sporadic or von Hippel–Lindau syndrome-associated intramedullary hemangioblastomas. Patients were evaluated at four time points: before treatment, on postoperative day one, on the day of discharge, and at a follow-up examination. ResultsThe patients showed good preoperative neurological status. The cohort had a slight female predominance. All tumors spanned at least one spinal segment. In all cases, total tumor removal was achieved, and a good outcome was obtained. None of the following factors had a significant effect on outcome: age, sex, tumor size, the presence of a syrinx, or the presence of von Hippel–Lindau syndrome. ConclusionsThe surgical removal of intramedullary hemangioblastomas resulted in satisfactory long-term functional outcomes. The best results were obtained before neurological symptoms occurred. Thus, we suggest that surgery should be considered for managing asymptomatic, surgically accessible, space-occupying lesions in sIH group, and isolated, space-occupying lesions in vHLS-IH group.

Role of preoperative embolization of intramedullary hemangioblastoma

ObjectHemangiobastomas (HB) are rare lesions accounting for 1 to 5% of all spinal cord tumors. Due to their hypervascular nature, an angiography may be proposed preoperatively in order to identify tumoral vascular anatomy. Preoperative embolization may be indicated to reduce intraoperative bleeding, thus facilitating tumor resection and minimizing surgical risk. The aim of this paper is to report our experience of preoperative embolization in intramedullary hemangioblastomas. MethodsWe performed a retrospective analysis of all patients operated on for intramedullary hemangioblastomas between 1995 and 2014 who had undergone embolization before surgery. ResultsSeven patients were analyzed: there were 6 females and 1 male, mean age 43years, 6 patients had Von Hippel-Lindau disease. Four tumors were located in the cervical spine and three in the dorsal spine. The average maximum sagittal diameter was 19mm (range 8–32mm), while the average maximum axial diameter was 11.5mm (range 6–21mm). The embolic agent used was Histoacryl (NBCA). Endovascular embolization was routinely performed the day before surgery. One patient experienced a major preoperative complication with a vertebrobasilar infarctus with consequent unilateral cerebellar syndrome and gait instability. Minor extravasation of embolic agent was observed in two cases. In one of these two cases, there was also the penetration of the embolic agent in the tumor; the resection was impossible due to the hard consistency of the tumor. In the other 6 patients, the resection was total. Six patients had identical preoperative and postoperative McCormick score and one patient shifted to a better score at follow-up. ConclusionPreoperative endovascular embolization is an effective adjunct treatment. It is useful in reducing the surgical bleeding and thus the operative risks. The procedure is not always safe and complications could occur. We recommend preoperative embolization in selected cases.

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.

Les hémangioblastomes intramédullaires

Aim and backgroundIntramedullary hemangioblastomas are rare lesions representing 1 to 5% of spinal tumors. The aim of this study was to review our experience with the surgical management of intramedullary hemangioblastomas. Materials and methodsWe performed a retrospective analysis of all the patients with intramedullary hemangioblastomas operated on between 1993 and 2011 in our department. All the patients were screened for Von Hippel Lindau disease. The minimum follow-up was 3 years. The clinical presentation, radiological findings, surgical procedure and outcomes were recorded and analyzed. ResultsOur consecutive series included 59 patients with a total of 65 tumors. The mean age at diagnosis was 38 years. Forty-two patients (72.5%) had Von Hippel Lindau disease. The main symptom was pain (58% of cases). The most common location was cervical spinal cord. The average size was 15mm. The resection was complete in 95% cases resulting in clinical improvement in 12% cases, stability in more than 86% of cases and deterioration in less than 2% cases. ConclusionAll patients with intramedullary hemangioblastoma should have a screening for the Von Hippel Lindau disease and if the diagnosis is correct, close monitoring should be initiated. Surgical removal is strongly advised in cases of neurological deficits or radiological progression of the tumour.

Intramedullary hemangioblastoma: Microsurgical resection technique

Spinal hemangioblastomas are benign and highly vascular tumors accounting for 1–5% of intramedullary spinal tumors in surgical series. Surgery is curative in sporadic cases. We present the description of a surgical technique to safely resect an intramedullary hemangioblastoma. A dorsal midline myelotomy provides an excellent exposure of the tumor and identification of the feeding arteries. Interruption of these arteries and precise dissection of the tumor from the cord tissue followed by division of the venous drainage allow the in toto excision of the tumor. Closure of the dorsal myelotomy may be achieved with sequential fusion of the pial and arachnoid edges using a “welding” technique.

Relationship between Spinal Hemangioblastoma Location and Age.

Study DesignRetrospective case series.PurposeTo investigate the relationship between tumor location and clinical characteristics.Overview of LiteratureHemangioblastoma is a rare disease that develops in the central nervous system. Magnetic resonance imaging (MRI) is useful to evaluate hemangioblastomas. Hemangioblastoma's location is designated as intramedullary, intramedullary+extramedullary, or extramedullary by MRI.MethodsWe analyzed 11 patients who underwent surgery for spinal hemangioblastoma. Using T1 contrast axial MRI data, the cases were divided into three groups (intramedullary, intramedullary+extramedullary, and extramedullary). Patient demographics, MRI findings, and preoperative neurological status were analyzed and compared for each group.ResultsThe average age of patients with intramedullary, intramedullary+extramedullary, and extramedullary hemangioblastoma was 34.0, 64.4, and 67.5 years, respectively. Patients in the intramedullary hemangioblastoma group were younger than the other groups. Extramedullary cases had a smaller syrinx compared to the other groups.ConclusionsAge may play an important role in the hemangioblastoma tumor location and the subsequent diagnosis by an MRI.

Aim and indications of spinal angiography for spine and spinal cord surgery: Based on a retrospective series of 70 cases

AimFor spinal surgery, computerized tomography (CT scan) and magnetic resonance imaging (MRI) have clear indications and are easily accessible. In contrast, the indications and the use of spinal angiograms (SA) remain unclear, and many centres performing spinal surgery do not have an access to SA. Based on a retrospective study, the role of SA in spinal surgery is assessed and their indications are discussed. Material and methodsA retrospective series of 72 SA in 70 patients is presented. No procedural accident occurred. SA was performed under general anaesthesia in 57 cases (82%). In 61 patients, locating the radiculomedullary arteries (RMA) was obligatory and performed in all cases: for 14 patients (21%), RMA were identified using the forecasted surgical approach (4 patients with degenerative disc disease out of 10 in the entire series were included), and modified. No ischaemic complications were observed in the series. Thirty-nine patients were treated for a tumour that was considered hypervascular (based on a histological hypothesis or the MRI data): 20 of them (51%) were preoperatively embolised and in only 8 cases was the operation considered “haemorrhagic” by the surgeon (among which, 3 intramedullary hemangioblastomas were included). No accident was observed during the embolisations. Thirteen patients presented with a vascular or haemorrhagic lesion (4 arteriovenous malformations, 6 dural arteriovenous fistulas, 3 intramedullary cavernomas): in all these cases, the SA was indispensable for the diagnosis and the decision-making process. Seven patients were treated by embolisation. In the last 8 cases, SA was considered for the diagnosis of a clinical worsening myelopathy with a non-contributive MRI, but it was not useful in providing a positive diagnosis. ConclusionsSAs were performed with different goals: (i) localization of RMA when a surgical approach between T4 and L2 involved the intervertebral foramen, or when an anterior approach was considered in order to avoid severe ischaemic complications (40% of the degenerative disc patients and 33% of the extramedullary tumour patients in this series); (ii) preoperative embolisation in cases of extramedullary tumours probably considered hypervascular (51% of the cases in the series) or in cases of arteriovenous shunt lesions (7 of 13 patients were treated by embolisation); (iii) as a diagnostic tool, SA is indispensable when MRI can reveal vascular abnormalities; it also provides information about the vascularisation as well as the endovascular possibilities in extramedullary tumours. In contrast, SA was not useful for intramedullary tumours because the RMA preoperative localisation is not mandatory (posterior approach), and embolisation seemed ineffective. SA was also not useful for the diagnosis of myelopathy with normal MRI. In the future, angiograms and MR angiography of the spinal cord may be useful in order to avoid general anaesthesia for a diagnostic procedure, but not practical to obtain access for endovascular treatment.

Incidence and Prognosis of Spinal Hemangioblastoma: A Surveillance Epidemiology and End Results Study

Objective: Intradural spinal hemangioblastoma are infrequent, vascular, pathologically benign tumors occurring either sporadically or in association with von Hippel-Lindau disease along the neural axis. Described in fewer than 1,000 cases, literature is variable with respect to epidemiological factors associated with spinal hemangioblastoma and their treatment. The objective of this study was to evaluate the epidemiology of intradural spinal hemangioblastoma with the Surveillance, Epidemiology and End Results (SEER) database while also presenting an illustrative case. Methods: The SEER database was queried for cases of spinal hemangioblastoma between 2000 and 2010 with the use of SEER*Stat software. Incidence was evaluated as a function of age, sex and race. Survival was evaluated with the Cox proportionate hazards ratio using IBM SPSS software evaluating age, sex, location, treatment modality, pathology and number of primaries (p = 0.05). Descriptive statistics of the same factors were also calculated. The case of a 43-year-old patient with a surgical upper cervical intramedullary hemangioblastoma is also presented. Results: In the data set between 2000 and 2010, there were 133 cases with an age-adjusted incidence of 0.014 (0.012-0.017) per 100,000 to the standard USA population. Hemangioblastoma was the tenth most common intradural spinal tumor type representing 2.1% (133 of 6,156) of all spinal tumors. There was no difference in incidence between men and women with an female:male rate ratio of 1.05 (0.73-1.50) with p = 0.86. The average age of patients was 48.0 (45.2-50.9) years, and a lower incidence was noted in patients <15 years compared to all other age groups (p < 0.05). There was no difference in incidence amongst the different races. Treatment included surgical resection in 106 (79.7%) cases, radiation with surgery in 7 (5.3%) cases, and radiation alone was used in only 1 (0.8%) case, and no treatment was performed in 17 (12.8%) cases. Mortality was noted in 12 (9%) cases, and median survival of 27.5 months (range 1-66 months) over the 10-year period. Mortality was attributable to the malignancy in 3 (2%) cases. There was no statistically significant different in Cox hazard ratios for mortality for sex, race, treatment modality, pathology or number of primaries. Conclusions: Spinal hemangioblastoma represent a small fraction of primary intradural spinal tumors, and this study did not identify any difference in incidence between genders. Surgical treatment alone was the most common treatment modality. Overall prognosis is good, with 9% observed mortality over the 10-year period, with 2% mortality attributable to the malignancy.

Intramedullary hemangioblastomas: surgical results in 16 patients.

OBJECT Hemangioblastomas are rare, benign, highly vascularized tumors that can be found throughout the neuraxis but are mainly located in the cerebellum and in the spinal cord. Spinal hemangioblastomas can present with motor and sensory deficits, whose severity varies according to the size and location of the tumor. Resection is the best treatment option to avoid neurological deterioration. The authors report surgical results in the treatment of intramedullary hemangioblastomas and discuss the technical nuances important to achieving total resection without adding new deficits. METHODS A consecutive series of patients with intramedullary hemangioblastomas operated on between 2000 and 2014 by the senior author (H.T.) is presented. The functional scale proposed by McCormick was used to evaluate the patients' neurological status before and after surgery. RESULTS Sixteen patients were included in the study and underwent 17 surgeries. Follow-up was at least 6 months. Age at presentation varied from 13 to 58 years (mean 33.8 years). Ten patients (62.5%) were males and 6 patients (37.5%) were females. Seven (43.75%) of the 16 patients had associated von Hippel-Lindau syndrome, with hemangioblastomas also presenting in other locations. Three patients had multiple tumors in the same segment in the spinal cord, and 10 patients (62.5%) presented with cysts. According to the site of presentation, 11 tumors (68.75%) were localized at the cervical region (including the cervicomedullary junction) and 5 tumors (31.25%) at the thoracic level. Total resection was achieved in all cases, evidenced by postoperative MRI. Four patients had some functional worsening immediately after surgery. After 6 months, 1 patient had functional worsening compared with preoperative status, and 2 patients had clinical improvement. The majority of the patients remained clinically stable postoperatively. CONCLUSIONS Adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors, with minimal morbidity and maximum functional recovery. Outcome seems to be directly related to the neurological status before surgery.

Discrete or diffuse intramedullary tumor? Contrast-enhanced intraoperative ultrasound in a case of intramedullary cervicothoracic hemangioblastomas mimicking a diffuse infiltrative glioma: technical note and case report

Hemangioblastomas are benign, highly vascularized intramedullary lesions that may also extend into the intradural space. Surgery represents the standard therapy, with the goal of obtaining complete resection even at the risk of neurological morbidity. MRI is the gold standard for diagnosis and assessment of intramedullary tumors. Nevertheless, sometimes MRI may not accurately differentiate between different types of intramedullary tumors, in particular if they are associated with syringes or intra- and peritumoral cysts. This could subsequently affect surgical strategies. Intraoperative ultrasound (ioUS) has become in the last few years a very useful tool for use during neurosurgical procedures. Various ioUS modalities such as B-mode and Doppler have been applied during neurosurgical procedures. On the other hand, the use of contrast-enhanced ultrasound (CEUS) is not yet well defined and standardized in this field. We report a case of a young patient harboring a cervicothoracic intramedullary tumor, for which the preoperative neuroradiologi-cal diagnosis was in favor of a diffuse astrocytoma with nodular components whereas ioUS demonstrated 3 distinct intramedullary nodules. CEUS showed highly vascularized lesions, compatible with hemangioblastomas. These findings, particularly those obtained with CEUS, allowed better definition of the lesions for diagnosis, enhanced understanding of the physiopathological aspects, and permitted the localization of all 3 nodules, thus limiting spinal cord manipulation and allowing complete resection of the lesions, with an uneventful postoperative neurological course. To the best of our knowledge, this is the first report of the use of intraoperative CEUS in a case of intramedullary hemangioblastoma.

An unusual association of von Hippel–Lindau disease and posterior nutcracker syndrome: Management of neurological and urological aspects in a young male

Von Hippel–Lindau disease is a complex and systemic entity that can be discovered by neurological complications. Its association with other serious malformative disease like Nutcracker syndrome was not described, and its complex presentation must not make some other serious problem easily missed or neglected. Case Report: We present the case of 27 year-old male patient with 3 intramedullary hemangioblastoma of 6 months of insidious evolution complicated by medullary compression. The lesions were completely removed with excellent neurological postoperatory outcome. Thinking of von Hippel-Lindau (VHL) disease, our investigations were expanded and revealed multiple vermian and cerebellar hemangioblastomas, renal masses, and multiple pancreatic cysts. In addition, an incidentally found of posterior NCS during the abdominal computed tomography (CT) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal carcinomas) and for the missed symptomatic posterior Nutcracker syndrome were discussed. Conclusion: The complex presentation of the VHL disease must not makesome other serious problems easily missed or neglected like renal carcinoma or Nutcracker syndrome as seen in our case

Epidemiology of pediatric intramedullary spinal cord tumors

Intramedullary spinal cord tumors (IMSCTs) are a rare neurosurgical disease. There are a lot of controversies in their epidemiology. Most references were published in the 80-90ss. The authors describe and analyze the largest contemporary clinical series of IMSCTs treated by one surgeon (YK). 201 patients (aged 2 months - 18 years) with IMSCT operated on during the past 12 years. A slight predominance of male patients was discovered. Astrocytomas account for 74% of all pediatric IMSCTs. Classic ependymomas are rare (5,5%). Pediatric spinal cord hemangioblastomas (HABs) are highly suspicious for von Hippel-Lindau disease. Benign histological variants of astrocytomas are the most common type of pediatric IMCTs. No gender or age differences in the incidence of most tumors (except for HABs) have been observed. Patients with intramedullary HAB should be routinely screened for von Hippel-Lindau disease. There are still a lot of uncertainties in the published data on epidemiology of IMSCTs.

Intraoperative Indocyanine Green Video Angiography for Resection of Spinal Cord Hemangioblastomas

Background: The use of intraoperative video indocyanine green (ICG) to aid resection of spinal cord hemangioblastomas has previously been suggested as a potential adjunct in these cases. Intramedullary hemangioblastomas are not always easily identifiable on the spinal cord surface, making myelotomy placement difficult and knowledge of complete resection dependant on postoperative imaging. ICG videoangiography is established as an invaluable tool for intracranial vascular neurosurgery.