Optic nerve and chiasm hemangioblastomas in von Hippel-Lindau disease: report of 12 cases and review of the literature.

Introduction: Optic nerve and chiasm hemangioblastomas are rare tumors, occurring sporadically or in the context of von Hippel-Lindau (VHL) disease. They have only been portrayed in isolated case reports and small cohorts. Their natural history and therapeutic strategies are only scarcely described. To better characterize these rare tumors, we retrospectively analyzed an optic nerve and chiasm hemangioblastoma series of 12 VHL patients. By combining our own experience to a review of all known cases in literature, we intended to create treatment recommendations for optic nerve and chiasm hemangioblastomas in VHL patients. Methods: We reviewed two electronic databases in the hospitals of our senior authors, searching for VHL patients with optic nerve or chiasm hemangioblastomas. Clinical data were summarized. Tumor size and growth rate were measured on contrast enhanced MRI. Comparable data were collected by literature review of all available cases in VHL patients (Pubmed, Trip, Google and Google Scholar). Results: Of 269 VHL patients, 12 had optic nerve or chiasm hemangioblastomas. In 10 of 12 patients, tumors were diagnosed upon annual ophthalmoscopic/MRI screening. Of 8 patients who were asymptomatic at diagnosis, 7 showed absent or very slow annual progression, without developing significant vision impairment. One patient developed moderate vision impairment. Two symptomatic patients suffered from rapid tumor growth and progressive vision impairment. Both underwent late-stage surgery, resulting in incomplete resection and progressive vision impairment. One patient presented with acute vision field loss. A watchful-waiting approach was adopted because the hemangioblastoma was ineligible for vision-sparing surgery. One patient developed progressive vision impairment after watchful waiting. In the literature we found 45 patient cases with 48 hemangioblastomas. Discussion: When optic nerve and chiasm hemangioblastomas are diagnosed, we suggest annual MRI follow-up as long as patients do not develop vision impairment. If tumors grow fast, threaten the contralateral eye, or if patients develop progressive vision deficiency; surgical resection must be considered because neurological impairment is irreversible, and resection of large tumors carries a higher risk of further visual decline.

Radiosurgically Treated Recurrent Cerebellar Hemangioblastoma: A Case Report and Literature Review.

BACKGROUND: Cystic, sporadic hemangioblastomas (HBLs) represent a unique, therapeutically challenging subset of central nervous system tumors, mainly due to their unpredictable growth patterns and potential for symptomatic progression. This study aims to explore the complexities surrounding the diagnosis, treatment, and long-term management of these lesions. METHODS: A comprehensive literature review was performed, and a detailed case study of a 56-year-old patient with a cystic, sporadic cerebellar HBL was produced. RESULTS: The case highlights the multiphasic growth pattern typical of cystic, sporadic HBLs, characterized by periods of dormancy and subsequent rapid expansion. An initial surgical intervention offered temporary control. Tumor recurrence, mainly through cystic enlargement, was treated by SRS. A subsequent recurrence, again caused by cystic growth, eventually led to the patient's death. The intricacies of treatment modalities, focusing on the transition from surgical resection to stereotactic radiosurgery (SRS) upon recurrence, are discussed. Parameters indicating impending tumor growth, coupled with symptomatic advances, are also explored. CONCLUSIONS: The management of cystic, sporadic cerebellar HBLs requires a strategic approach that can be informed by radiological characteristics and tumoral behavior. This study underscores the importance of a proactive, individualized management plan and suggests guidelines that could inform clinical decision making.

Characterization of spinal hemangioblastomas in patients with and without von Hippel-Lindau, and YAP expression.

INTRODUCTION: Hemangioblastoma (HB) is a benign tumor of the central nervous system, associated with von Hippel-Lindau disease (VHL), or sporadic. The aim of this study was to compare and examine the clinical-pathological profile of patients with spinal hemangioblastoma and YAP expression. METHODS: A retrospective, descriptive, comparative study. All patients who underwent surgery for spinal HB between 2016 and 2023 were included. Clinical and radiological data were collected and analyzed. An immunohistochemistry panel including NeuN, neurofilaments (NF), and YAP-1, was performed. RESULTS: Nine patients were studied, six women and three men. Four patients had previously diagnosed VHL. The tumor location included: four cervical (44.44%), two thoracic (22.22%), two pontine with cervical extension (22.22%) and one patient with two lesions, one cervical and one thoracic (11.11%). Non-significant clinical differences were identified between VHL and sporadic patients. Imaging evidenced seven extramedullary and three intramedullary tumors. Histologically, intra-tumoral and perivascular axonal tracts were observed in all cases. One third of the tumors (two with VHL and one sporadic) presented extramedullary hematopoiesis. Seven cases (77.8%) expressed nuclear YAP (three with VHL and four sporadic HBs). The surgical outcome was good and only one patient with VHL undergoing subtotal resection had recurrence. CONCLUSIONS: Spinal HBs can be associated with VHL or be sporadic. To the best of our knowledge, this is the first study to describe YAP expression in HB. It is important to investigate the involvement of the Hippo pathway in HBs as a possible therapeutic target.

Late-Onset Manifestations of Von Hippel-Lindau Syndrome: A Case Report.

Von Hippel-Lindau (VHL) syndrome is characterized by a range of tumors including phaeochromocytomas, pancreatic adenomas, cerebellar haemangioblastomas, and renal cell carcinomas. A 50-year-old male presented with a three-week history of headache. Additionally, the patient exhibited signs of hypertension. Ultrasonography (USG) abdomen and pelvis showed a solid mass lesion in the left adrenal gland, iso-echoic to the renal cortex. On contrast-enhanced computed tomography (CECT) of the brain, a well-defined solid cystic lesion was seen in the left posterior cerebellar hemisphere. Small nodular enhancing lesions were seen in the right cerebellar hemisphere. On further imaging with MRI brain contrast, the lesions in the cerebellum were diagnosed as multifocal hemangioblastomas. Laboratory investigations revealed elevated urinary metanephrines and normetanephrine, suggesting pheochromocytoma. Based on radiological and biochemical investigations, with the features of cerebellar haemangioblastomas and pheochromocytoma, a diagnosis of VHL syndrome was made.

Cauda equina myxopapillary ependymoma in von Hippel-Lindau disease: A case report.

Background: Patients affected by Von Hippel-Lindau (VHL) are prone to develop central nervous system neoplasms such as hemangioblastomas (HBs). Myxopapillary ependymoma (MPE) is not commonly associated with VHL disease. Case Description: We present the first case of a VHL patient affected by simultaneous silent cauda equina MPE and a symptomatic conus medullaris HB. The patient was first operated for systemic tumors and followed for asymptomatic HBs. Simple surveillance was maintained until neurological symptoms appeared. Regular follow-up demonstrated objective growth of the cystic conus medullaris tumor while the cauda equina lesion remained stable. Surgery was performed to avoid further neurological worsening. Histopathological examination showed conus medullaris HB and a nearby cauda equina MPE. Conclusion: Simultaneous spinal HBs and isolated MPE may exceptionally occur in VHL patients.

Oral Hypoxia-Inducible Factor 2α Inhibitor Belzutifan in Ocular von Hippel-Lindau Disease: Subgroup Analysis of the Single-Arm Phase 2 LITESPARK-004 Study.

PURPOSE: To report the efficacy of the oral hypoxia-inducible factor 2α inhibitor belzutifan in participants with von Hippel-Lindau disease-associated retinal hemangioblastomas in the LITESPARK-004 study. DESIGN: Subgroup analysis of the phase 2, single-arm, open-label LITESPARK-004 study. PARTICIPANTS: Adults with 1 or more von Hippel-Lindau disease-associated measurable renal cell carcinoma tumors not requiring immediate surgical intervention were eligible. METHODS: Participants received oral belzutifan 120 mg once daily until disease progression or unacceptable treatment-related toxicity. MAIN OUTCOME MEASURES: Efficacy of belzutifan in retinal hemangioblastomas was a secondary end point, measured as response (improved, stable, or progressed) by independent reading center-certified graders based on color fundus imaging performed every 12 weeks using the investigator's preferred imaging standards. Additional assessments, where available, included OCT and ultra-widefield fluorescein angiography. RESULTS: Among 61 participants in LITESPARK-004, 12 had 1 or more evaluable active retinal hemangioblastomas in 16 eyes at baseline per independent reading center. As of April 1, 2022, the median follow-up for participants with ocular von Hippel-Lindau disease at baseline was 37.3 months. All 16 eyes were graded as improved, with a response rate of 100.0% (95% confidence interval, 79.4%-100%). No new retinal hemangioblastomas or ocular disease progression were reported as of data cutoff date. Eight participants underwent additional multimodal eye assessments performed at the National Institutes of Health study site. Among this subgroup, 10 of 24 hemangioblastomas in 8 eyes of 6 participants measured 500 µm or more in greatest linear dimension at baseline and were analyzed further. All 10 hemangioblastomas had a mean area reduction of 15% or more by month 12 and of 30% or more by month 24. CONCLUSIONS: Belzutifan showed promising activity against ocular von Hippel-Lindau disease, including capacity to control retinal hemangioblastomas, with effects sustained for more than 2 years while treatment is ongoing. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Cerebellar Hemangioblastoma Mimicking Arteriovenous Malformation: A Case Report.

Hemangioblastoma (HBM) is a tumor distinguished by the presence of stromal cells and small vessels. These stromal cells represent stem cells, which, due to the influence of the neoplasm, proliferate and differentiate into "vasoformative elements" that create new blood vessels. Hemangioblastomas resemble arteriovenous malformation (AVM) in imaging features, characterized by an apparent vascular blush, the presence of multiple feeding vessels, and evident draining veins observed on digital subtraction angiography (DSA). Our study presents a case of HBM in the right cerebellar hemisphere mimicking AVM. The patient had been diagnosed with AVM in the same location two years ago and managed with endovascular embolization. One month prior, the patient experienced severe headaches, imbalance, nausea, left ear fullness, blurry vision, and high blood pressure. The imaging feature suggests HBM rather than AVM. The patient next underwent sub-occipital craniotomy and tumor resection with external ventricular drainage (EVD) insertion. The histopathological report of the excised mass confirmed HBM. In conclusion, AVM and HBM rarely occur together. Recent research indicates that HBM and AVM have exact embryologic origins and need later genetic alterations to develop into symptomatic lesions. Further research is required to clarify the uncommon combination of these lesions.

Preoperative embolization and microsurgical resection of a cerebellar hemangioblastoma: embolization goals and technical nuances of the approaches. Illustrative case.

BACKGROUND: Resection remains the optimal treatment for hemangioblastomas, highly vascular tumors commonly located in the cerebellum or spinal cord. Preoperative embolization can be used with caution to reduce intraoperative blood loss and promote gross-total resection while reducing neurological morbidity. OBSERVATIONS: The authors report a case of a 44-year-old male who presented with worsening dizziness, gait imbalance, and diplopia. Imaging revealed a large vascular cerebellar mass with brainstem compression and hydrocephalus, concerning for hemangioblastoma. The patient underwent preoperative embolization of the main arterial supply from the right superior cerebellar artery, which devascularized the tumor and reduced the early venous shunting, followed by gross-total resection of the tumor. Preoperative embolization helped to facilitate safe microsurgical resection because this main arterial supply was deep to the planned surgical approach, and the Onyx cast served as a landmark of the dorsal midbrain and remaining arterial supply. This resulted in minimal intraoperative blood loss. At 2-year follow-up, the patient's diplopia and vertigo had resolved, and his gait continues to improve. LESSONS: Despite controversy regarding the use of preoperative embolization for hemangioblastoma resection, the authors emphasize its safety and utility during resection of a large cerebellar hemangioblastoma. Onyx embolization provided benefit in both reducing the tumor blood supply and serving as intraoperative visual guidance.

Diagnostic Role of Oral Fluorescein Angiography in Pediatric Ambulatory Clinics.

Oral ingestion of fluorescein can be done in ambulatory pediatric clinics. We show that oral ultra-widefield fluorescein angiography is a non-invasive approach to rapidly diagnose and manage a diverse set of pediatric retinal vascular diseases.

Multimodal management of giant solid hemangioblastomas in two patients with preoperative embolization.

Background: Hemangioblastomas are benign vascular neoplasms, World Health Organization grade I, with the most frequent location in the cerebellum. Complete microsurgical resection can be a challenge due to excessive bleeding, which is why preoperative embolization takes importance. Case Description: Two clinical cases are presented, a 25-year-old woman and a 75-year-old man, who presented with intracranial hypertension symptoms due to obstructive hydrocephalus; a ventriculoperitoneal shunt was placed in both cases; in addition, they presented with cerebellar signs. Both underwent embolization with ethylene vinyl alcohol copolymer, with blood flow reduction. After that, they underwent microsurgical resection within the 1st-week post embolization, obtaining, in both cases, gross total resection without hemodynamic complications, with clinical improvement and good surgical outcome. It is worth mentioning that surgical management is the gold standard that allows a suitable surgical approach, like in our patients, for which a lateral suboccipital craniotomy was performed. Conclusion: Solid hemangioblastomas are less frequent than their cystic counterparts. The treatment is the surgical resection, which is a challenge and always has to be considered as an arteriovenous malformation in the surgical planning, including preoperative embolization to reduce perioperative morbidity and mortality and get good outcomes.

Patients With Hemangioblastoma: Mood Disorders and Sleep Quality.

BACKGROUND: Sleep has confirmed physical, psychological, and behavioral benefits, and disruptions can result in disturbances in these states. Moreover, it can be linked bidirectionally with susceptibility to and the subsequent status of brain tumors. The current study examined mood disorders and sleep quality before and after surgery for hemangioblastoma brain tumors. METHODS: Thirty-two patients diagnosed with hemangioblastoma brain tumors between 2017 and 2023 underwent surgical treatment. The Karnofsky Performance Status and ECOG performance status scales, the Brunel Mood Scale, the Morningness-Eveningness Questionnaire, and the Mini-Sleep Questionnaire were employed to assess the patients. RESULTS: The findings indicate that after surgery, sleep quality and mood disorders, including tension, vigor, and depression, did not exhibit significant differences in these patients (p>0.05). However, tension, vigor, depression, and sleep quality did have a significant impact on their functional status post-surgery (p<0.05). CONCLUSION: Depression is the significant mood factor in patients with brain tumors that impact their functional status. In this context, it is recommended that psychological therapies be considered for them, alongside conducting more comprehensive and in-depth studies on psychological disorders in patients with brain tumors.

Case Report of a Hemangioblastoma Originating from the T1 Spinal Nerve Treated with in Toto Resection, Hemicorporectomy and Pedicle Screw Stabilization.

Introduction: Previously, only 40 cases with extradural hemangioblastoma at the spinal nerve (none at the T1 nerve root) have been described in the medical literature. In toto, resection of this hypervascular tumor is essential to avoid bleeding complications. Surgery for hemangioblastoma at the cervicothoracic junction is complex and nerve resection of the T1 results in specific neurodeficits of the hand muscles which are not well known. Case Report: A 34-year-old woman was diagnosed with a slowly growing tumor located at the left foramen T1/T2. Pressure from the tumor resulted in Horner's syndrome and pain and paresthesia in the upper extremity. The tumor was resected in toto through a posterior midline approach and rib resection and transection of the left T1 and T2 spinal nerves. T2 hemicorporectomy and spinal stabilization were performed to gain access to and mobilize the tumor ventrally. Ptosis decreased after surgery and no neurodeficit was observed except the expected deficit (no deficit was present preoperatively) caused by the T1 resection specifically a small decrease in strength of the abductor and flexor pollicis brevis and opponens pollicis and the lateral two lumbricals. Histological examination of the tumor demonstrated a hemangioblastoma. von Hippel-Lindau disease was ruled out by genetic testing of the patient's blood. Eight-month postoperatively, all pre-operative symptoms had decreased considerably and the radiographic examination shoved unchanged pedicle screw/rod stabilization of the cervicothoracic junction. Conclusion: Hemangioblastoma is a rare hypervascular tumor very rarely located at the spinal nerve. The tumor should be resected in toto to avoid recurrence and bleeding. In the current case, the location was at the T1 root necessitating complex surgery with laminectomies and hemicorporectomy of T2 and a posterior rib resection/thoracotomy. Spinal stabilization is mandatory. Preferably embolization should be performed preoperatively. T1 transection results in a specific neurodeficit which should be explained to the patient preoperatively. The patients should undergo genetic testing for Hippel-Lindau disease.

Case report: A cystic capillary hemangioma located at the conus medullaris mimicking hemangioblastoma.

Capillary hemangiomas, usually found in skin and mucosal tissues, are rarely encountered within the spinal cord, presenting a significant diagnostic challenge. We report a rare case of intradural extramedullary capillary hemangioma at the conus medullaris in a 66-year-old female patient. Our initial diagnosis leaned towards a cystic hemangioblastoma based on MRI findings due to the presence of cystic formation with an enhanced mural nodule. However, surgical exploration and subsequent pathological examination revealed the lesion as a capillary hemangioma. To the authors' knowledge, this case may represent the first documented instance of a spinal capillary hemangioma that mimics a cystic hemangioblastoma.

Predictors of Progression in a Series of 81 Adult Patients Surgically Managed for an Intracranial Hemangioblastoma: Implications for the Postoperative Follow-Up.

The aim was to identify predictors of progression in a series of patients managed for an intracranial hemangioblastoma, in order to guide the postoperative follow-up modalities. The characteristics of 81 patients managed for an intracranial hemangioblastoma between January 2000 and October 2022 were retrospectively analyzed. The mean age at diagnosis was of 48 ± 16 years. Eleven (14%) patients had von Hippel-Lindau disease. The most frequent tumor location was the cerebellar hemispheres (n = 51, 65%) and 11 (14%) patients had multicentric hemangioblastomas. A gross total resection was achieved in 75 (93%) patients. Eighteen (22%) patients had a local progression, with a median progression-free survival of 56 months 95% CI [1;240]. Eleven (14%) patients had a distant progression (new hemangioblastoma and/or growth of an already known hemangioblastoma). Local progression was more frequent in younger patients (39 ± 14 years vs. 51 ± 16 years; p = 0.005), and those with von Hippel-Lindau disease (n = 8, 44% vs. n = 3, 5%, p < 0.0001), multiple cerebral locations (n = 3, 17% vs. n = 2, 3%, p = 0.02), and partial tumoral resection (n = 4, 18% vs. n = 1, 2%, p = 0.0006). Therefore, it is advisable to propose a postoperative follow-up for at least 10 years, and longer if at least one predictor of progression is present.

Pre-operative Embolization of a Cerebellar Hemangioblastoma Using Polyvinyl Alcohol (PVA) and Target Tetra 360 Detachable Coil.

Due to its hypervascularity, hemangioblastoma, a rare primary central nervous system intracranial tumor, has been treated with pre-operative embolization prior to surgical resection. Here, we describe a case treated as such. A 37-year-old male presented with worsening chronic headache and right ear tinnitus was found to have a hypervascular, heterogeneous right cerebellar lesion suspicious for arteriovenous malformation or hemangioblastoma. He underwent polyvinyl alcohol (PVA) and Target Tetra 360 (Fremont, CA: Stryker Neurovascular) detachable coil embolization followed by complete tumor resection. Pathology was consistent with hemangioblastoma. He presented with complete resolution of his symptoms immediately post-operatively and at a two-week follow-up. Our case highlighted the importance of pre-operative embolization to help achieve complete tumor resection which is considered curative in the treatment of hypervascular hemangioblastoma. The Target Tetra 360 detachable coil embolization is another material that can be considered.

Screening and surveillance recommendations for central nervous system hemangioblastomas in pediatric patients with Von Hippel-Lindau disease.

PURPOSE: Von Hippel-Lindau (VHL) disease is an autosomal-dominantly inherited tumor predisposition syndrome. One of the most common tumors are central nervous system (CNS) hemangioblastomas. Recommendations on the initiation and continuation of the screening and surveillance program for CNS tumors in pediatric VHL patients are based on small case series and thus low evidence level. To derive more robust screening recommendations, we report on the largest monocentric pediatric cohort of VHL patients. METHODS: We performed a retrospective analysis on a pediatric cohort of 99 VHL patients consulted at our VHL center from 1992 to 2023. Clinical, surgical, genetic, and imaging data were collected and statistically analyzed. RESULTS: 42 patients (50% male) developed CNS hemangioblastomas, of whom 18 patients (56% male) underwent hemangioblastoma surgery (mean age at first surgery: 14.9 ± 1.9 years; range 10.2-17). The first asymptomatic patient was operated on at the age of 13.2 years due to tumor progress. Truncating VHL mutation carriers had a significantly higher manifestation rate (HR = 3.7, 95% CI: 1.9-7.4, p < 0.0001) and surgery rate (HR = 3.3, 95% CI: 1.2-8.9, p = 0.02) compared with missense mutation carriers. CONCLUSION: We recommend starting MRI imaging at the age of 12 years with examination intervals every (1-) 2 years depending on CNS involvement. Special attention should be paid to patients with truncating variants. Affected families should be educated regularly on potential tumor-associated symptoms to enable timely MRI imaging and eventually intervention, as CNS hemangioblastoma may develop before screening begins. GERMAN CLINICAL TRIALS REGISTER REGISTRATION NUMBER: DRKS00029553, date of registration 08/16/2022, retrospectively registered.

Longitudinal Pulse-Synchronous Bouncing During Catheter Angiography-A Phenomenon Specific to Spinal Hemangioblastomas.

BACKGROUND: Spinal hemangioblastomas are often evaluated with catheter angiography for both workup and treatment planning. We report a unique longitudinal pulse-synchronous bouncing phenomenon observed during their angiographic evaluation and consider the association of pulse-synchronous bouncing with syringomyelia, another pathologic feature associated with hemangioblastomas. METHODS: Preoperative spinal angiograms and associated magnetic resonance imagings (MRIs) obtained over a 16-year period at a single institution were retrospectively evaluated. Magnetic resonance imaging (MRI) parameters included lesion and syrinx location and size. Angiograms were evaluated for bouncing phenomena. Student's t-test and Chi square test compared characteristics between groups. Linear regression analyses evaluated maximum amplitude of dynamic motion and any associated syrinx. RESULTS: Nineteen hemangioblastoma patients had preoperative angiograms available for review. Eight exhibited bouncing behavior. Between the dynamic and nondynamic cohorts, there was no difference in presence or volume of syrinxes. Lesions in the dynamic cohort trended towards a cervical location (75% vs. 36.3%, P = 0.10). No significant correlation was found between bouncing amplitude and syrinx size (R2 = 0.023). Dural contact may be related to this dynamic behavior since other high-flow lesions like AVMs do not demonstrate this phenomenon, and AVMs are pial-based and more likely to contact stationary dura. Here, there were fewer lesions abutting the thecal sac in the dynamic cohort (50% vs. 81.8%, P = 0.14). CONCLUSIONS: Though no significant relationship was established between this bouncing behavior and syrinx formation, noted trends included a greater range of motion for cervical lesions and limited motion in tumors abutting the thecal sac.

Time course of tumorigenesis of a newly developed sporadic hemangioblastoma in an elderly patient: illustrative case.

BACKGROUND: von Hippel-Lindau disease-associated hemangioblastomas (HBs) account for 20%-30% of all HB cases, with the appearance of new lesions often observed in the natural course of the disease. By comparison, the development of new lesions is rare in patients with sporadic HB. OBSERVATIONS: A 65-year-old man underwent clipping for an unruptured aneurysm of the anterior communicating artery. Fourteen years later, follow-up magnetic resonance imaging (MRI) revealed a strongly enhanced mass in the right cerebellar hemisphere, diagnosed as a sporadic HB. A retrospective review of MRI studies obtained over the follow-up period revealed the gradual development of peritumoral edema and vascularization before mass formation. LESSONS: Newly appearing high-intensity T2 lesions in the cerebellum may represent a preliminary stage of tumorigenesis. Careful monitoring of these patients would be indicated, which could provide options for early treatment to improve patient outcomes.

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

Objective: 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. Methods: 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. Results: 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. Conclusion: 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.

Resolution of epiretinal membrane after anti-VEGF and photodynamic therapy of retinal hemangioblastoma.

Purpose: To describe the clinical and multimodal imaging features in a case of resolution of pre-macular fibrosis after photodynamic therapy (PDT) of retinal hemangioblastoma (RH) not related to von-Hippel-Lindau (VHL). Observations: A 25-year-old man presenting with blurred vision and central metamorphopsia in his left eye (LE) due to macular epiretinal membrane secondary to a peripheral RH. The patient had a comprehensive ophthalmic examination, including best corrected visual acuity (BCVA), wide-field fundus photography and autofluorescence (FAF), wide-field optical coherence tomography (OCT) and OCT angiography (OCTA), fluorescein angiography (FA) and indocyanine green angiography (ICGA) and ocular ultrasound. Baseline BCVA was 20/200 in his LE, and it improved to 20/40 nineteen months after anti-VEGF and photodynamic therapy were performed. The treatment led to a progressive shrinkage of the tumor and associated intraretinal exudation, and to a progressive resolution of the epiretinal membrane. Conclusions and importance: Treatments performed on the peripheral retina may result in changes at the level of the vitreo-retinal interface in the macular region. In our patient, after an intravitreal anti-VEGF associated with PDT, a posterior vitreous detachment (PVD) was induced resulting in auto-peeling of the macular epiretinal membrane secondary to a peripheral RH. An accurate assessment of the macular area by OCT is highly recommended in the first days following treatments.