Spinal Hemangioblastomas Research Articles

Spontaneous Subarachnoid Haemorrhage in Spinal Hemangioblastoma: Illustrative Case and Discussion of a Pathophysiological Hypothesis

Spontaneous non-aneurysmal subarachnoid haemorrhage (naSAH) is an unusual finding that could be burdened by significant mortality and morbidity rates. Rare pathologies and delayed diagnosis could be advocated as responsible of unfavourable outcomes. Herein, we describe an exceedingly rare giant lumbar spinal hemangioblastoma (80 × 23 mm) presenting as an intracranial naSAH. Based on our radiological and clinical findings a pathophysiological hypothesis linking intracranial naSAH to venous hypertension was discussed for the first time even among lumbar spinal tumors. Although rare, unusual causes should be investigated in presence of radiological atypical finding as a prompt evaluation and treatment could be needed.

Hypercalcemia in Pheochromocytoma: From MEN to VHL

Background: Co-occurrence of phaeochromocytoma and primary hyperparathyroidism is usually seen in patients of Multiple Endocrine neoplasia 2A(MEN2A) and is rare in Von Hippel Lindau disease (VHL). Parathyroid adenoma with pheochromocytoma in a genetically confirmed VHL has been reported only once till date (1). Clinical Case: A 30-year male was admitted for evaluation of hypertension and incidentally diagnosed adrenal mass on ultrasound. 9-years back, he was diagnosed as a case of VHL (right renal clear cell carcinoma, pancreatic cystadenoma, spinal hemangioblastoma and epididymal cysts). Right nephrectomy and pancreatic cyst excision had been done and past work-up for pheochromocytoma was normal. He also had been operated twice for recurrent renal calculi. Family history revealed surgery in mother for pancreatic mass. Current imaging revealed right adrenal mass (4.7*4.6 cm) with left renal cysts and calculi, pancreatic cysts and spinal- medullary hemangioblastoma and epididymal cysts. Fundus examination was normal. 24-hours urinary fractionated normetanephrines were elevated (2062 mcg/24 hours) and I131MIBG scan showed 4.7*4.6cm concentrating lesion in right renal fossa suggestive of right adrenal pheochromocytoma. However his biochemical evaluation revealed hypercalcemia (12.1 mg/dl), low phosphorus (3.2 mg/dl), low 25(OH) D (24.84 nmol/l), and raised PTH (121pg/ml). Ultrasound neck and Tc99m-Sestamibi localized left inferior parathyroid adenoma. DEXA scan showed severe osteoporosis. Genetic analysis confirmed VHL mutation in exon-1. Calcitonin and RET mutation were normal (ruled out MEN2A). Therapeutic approach was surgical excision of adrenal pheochromocytoma followed by parathyroidectomy. We report a case of pheochromocytoma with primary hyperparathyroidism (cause: left inferior parathyroid adenoma) in a patient of VHL (Renal clear cell carcinoma, pancreatic cystadenoma, epidydymal cysts and medullary and spinal hemangioblastoma). Hypercalcemia seen in patients of VHL is either due to bone metastasis/PTHrP/IL-6 secretion from RCC or due to PTHrP/PTH/calcitonin secretion from pheochromocytoma and rarely due to associated parathyroid adenoma. Literature search revealed four case reports of parathyroid adenoma with VHL. In only one of these, VHL had pheochromocytoma associated with parathyroid adenoma (1). Conclusion: Ours is the 2nd such case reported in literature of primary hyperparathyroidism in a genetically confirmed case of VHL with pheochromocytoma. This case highlights the overlap of tumorigenesis in two rare genetically divergent syndromes and importance of long-term follow-up for sequential development of new tumors. Reference: Arao T, Okada Yet al. A case of VHL disease with bilateral pheochromocytoma, renal cell carcinoma, pelvic tumor, spinal hemangioblastoma and primary hyperparathyroidism. Endocr J. 2002 Apr;49(2):181–8.

Spinal Hemangioblastomas and Neuropathic Pain

Spinal hemangioblastomas (SHs) are rare and benign tumors. Primary symptoms include pain, hypoesthesia, and neuropathic pain (NP). Clinical symptoms may be as a result of tumor mass effect, peritumoral effect, syrinx, or venous congestion. No studies have focused on NP in SHs. The objective of this study was to review the rate and causes of NP in patients with SHs. The present study comprises a retrospective analysis of 13 patients with spinal hemangioblastomas. For the retrospective analysis of the patients, we analyzed the absence or presence of NP in the pre- and postoperative periods and its relationship with the level, location, and size of the tumor, as well as the size and location of the syrinx. Postoperative NP was detected in 6 out of 13 patients. All 6 patients' tumors were located at the dorsal aspect of the spinal cord. There was a predominance of rostral syrinx location in patients with NP. Tumor size and level and syrinx size and level were not found to affect the occurrence of NP. The present study shows that NP is observable in both pre- and postoperative periods. Proximity of the tumor to the dorsal root entry zone, and especially the presence of rostral syrinx, are the main factors affecting postoperative NP symptomatology. It is concluded that the combination of these factors and iatrogenic injury of anatomic pathways of NP within the spinal cord are responsible for postoperative NP.

Spinal cord hemangioblastomas with a focus on clinical presentation, diagnosis, and treatment at a tertiary care hospital of Karachi, Pakistan: A retrospective chart review.

Background: Hemangioblastomas are benign neoplasms that consist of stromal cells and small blood vessels. They are highly vascular tumors and can arise throughout the central nervous system. This study aims to provide an overview of our experience with this rare tumor’s presentation, radiology, histopathology, and outcomes as literature regarding this pathology is sparse from our country.Methods: The study is a retrospective review of cases that were histopathology proven cases of spinal cord hemangioblastomas. The clinical characteristics of these patients were examined, and their presentation was recorded. The radiology was also reviewed to describe classic appearance on magnetic resonance imaging. A detailed review of immunohistochemistry was also performed and outcome was described.Results: A total of 25 cases of spinal hemangioblastomas were found in our records in the period of 2001–2019. There were 20 males (80%) and only 5 female patients (20%). Gross tumor fragments ranged in size from 0.24 cm2 to 10.5 cm2 (mean 3.28 ± 2.65). Histologically, tumor was composed of nests of large stromal cells with clear to vacuolated cytoplasm separated by thin-walled capillaries. Focal intratumoral hemorrhage was noted. No significant cytological atypia or mitotic figures were noted. Immunohistochemical stains were performed to confirm the diagnosis and exclude other tumors. Inhibin was tested in 20 cases and it was positive in 16 cases (80%). Neuron-specific enolase was positive in 6/8 cases. Cluster of differentiation (CD) CD68 was positive in 6/6 cases and vimentin in 4/4 cases. Glial fibrillary acidic protein (GFAP) and epithelial membrane antigen were performed in 14 and 8 cases, respectively, and all were negative. Cytokeratin AE1/AE3 was negative in 13/13 cases. CD34 highlighted vasculature in the 8 cases in which it was performed and was negative in tumor cells. Follow-up was available in 17 out of 25 cases and ranged from 12 months to 216 months (mean 61.8 ± 60.6 months). Recurrence occurred in 2 out of 17 (11.7%) patients for whom follow-up information was available.Conclusion: Our experience shows that spinal cord hemangioblastomas can be surgically removed in most cases with a low risk of recurrence. Most patients in our study were male and unlike other studies, none of our cases showed GFAP positivity.

Microsurgical Resection of an Intramedullary Spinal Cord Hemangioblastoma Through an Anterior Cervical Approach: 2-Dimensional Operative Video.

This video demonstrates the microsurgical removal of an intramedullary spinal cord hemangioblastoma through an anterior cervical approach. While most spinal hemangioblastomas arise from the dorsal or dorsolateral pial surface and can be safely resected through a posterior approach,1,2 ventral tumors can present a significant challenge to safe surgical removal.3-5 This patient presented with a progressively symptomatic ventral pial based hemangioblastoma at the C5-6 level with large polar cysts extending from C3 to T1. The tumor was approached through a standard anterior cervical exposure with a C5 and C6 corpectomy. Following midline durotomy, the tumor was identified and complete microsurgical resection was achieved. The principles and techniques of tumor resection are illustrated and described in the video. Following tumor resection and dural closure, a fibular allograft was inserted into the corpectomy defect and a C4-C7 fixation plate was placed. The patient was maintained in a supine position for 36 h. He was discharged home on postoperative day 3 in a cervical collar. The patient did well with near-complete recovery of neurological function. Postoperative magnetic resonance imaging at 6 wk showed a substantial resolution of the polar cysts and no evidence of residual tumor. The patient featured in this video consented to the procedure.

Семейный случай болезни Гиппеля-Линдау, ассоциированной с гемангиобластомой спинного мозга и сирингомиелией: хирургическое лечение и динамическое наблюдение

Семейный случай болезни Гиппеля-Линдау, ассоциированной с гемангиобластомой спинного мозга и сирингомиелией: хирургическое лечение и динамическое наблюдение

STMO-02 Efficacy of preoperative embolization for hemangioblastoma

Introduction: Preoperative transarterial embolization (TAE) for hemangioblastoma carries a risk of cerebral infarction and hemorrhagic complications, and its safety and efficacy are controversial.Method: Twenty-two cases of hemangioblastoma (cerebellar: 18 cases, medulla oblongata: 3 cases, spinal cord: 1 case) treated via direct surgery in our hospital from 2007 to 2020 were enrolled.Results: Preoperative TAE was performed in 6 cases of cerebellar hemangioblastoma (1 bilateral case) and 1 case of spinal hemangioblastoma. The cerebellar hemangioblastoma feeders were only superior cerebellar artery (SCA) in 3 cases, SCA/anterior inferior cerebellar artery (AICA)/posterior inferior cerebellar artery (PICA) in 2 cases, AICA/PICA in 1 case, and single drainer in 5 cases. Tumors were ≥30 mm in all cases (25 mm on 1 side in bilateral cases), and solid or nodular lesions were located on the upper surface of the cerebellum. Cerebellar edema was severe in five cases with hydrocephalus. TAE was performed under local anesthesia in all cases, using a coil alone in two cases and liquid or particle embolization material in five cases. The day before direct surgery, TAE was performed in four cases, one of which underwent emergency decompression due to severe cerebellar edema. Three cases were intentionally embolized on the day of direct surgery. The median blood loss during direct surgery was 100 ml. Although cerebral infarction was observed in all cases, there were no cases of brain stem infarction or hemorrhagic complications. The Modified Rankin Scale at discharge was 0 in 2 cases, 1 in 3 cases, 3 in 1 case, and 4 in 1 case.Discussion/Conclusion: Preoperative TAE for hemangioblastoma reduced the blood loss for direct surgery. Same-day TAE avoided neurological deficit due to cerebral infarction and cerebellar edema. To prevent severe infarction, guiding the microcatheter to the vicinity of the tumor bed is important.

Management of intramedullary spinal cord tumors: A single-center experience of 247 patients.

Intramedullary spinal cord tumors (ISCT) are rare tumors requiring multidisciplinary care in an expert center. Here, we report a single-center experience including 247 patients with ISCT: 134 ependymomas, 54 astrocytomas and 59 hemangioblastomas. Preoperative evaluation and surgical considerations are discussed to share our principles in managing these patients. Ependymomas are largely benign lesions (95% of WHO grade II) for which total resection (91% rate of gross total resection (GTR)) without neurological impairment (79% of patients remained stable or improved) is the goal in order to ensure long-term oncological control (94.5% overall survival at 5 years, excluding anaplastic ependymomas). On the other hand, astrocytomas are more frequently high-grade tumors (30% of WHO grade III or IV) for which partial resection (60% of cases) is often necessary to preserve neurological status (48% of patients deteriorate at one year), leading to higher rates of recurrence (60% recurrence rate at 5 years for grade III astrocytomas). Lastly, spinal hemangioblastomas require a specific microsurgical resection with particular attention to the vasculature in order to ensure en bloc resection (95% GTR), allowing excellent neurological results (12% of patients improved, 86% remained stable). Altogether, these results demonstrate that ISCT can be managed surgically with good functional outcome, while oncological results will mostly depend on the histopathological grading.

A rare condition: Spontaneous subarachnoid haemorrhage due to spinal hemangioblastoma: Report of 2 cases and review of the literature

IntroductionSubarachnoid haemorrhage (SAH), secondary to spinal hemangioblastoma (HBL), is extremely rare, with only a few case reports to date. We report the experience of our reference centre for spinal tumours and Von Hippel–Lindau (VHL) disease in patients with spinal HBL presenting with SAH. We further performed a systematic review of the literature. MethodsWe report two cases. A systematic search was performed using the PubMed, Embase and Cochrane databases, with no limit for publication date. Inclusion criteria were: patients with HBL presenting with SAH, with or without VHL. The systematic review retrieved only 10 studies, including 16 patients. ResultsIn our centre, the first case concerned radicular HBL at D12 level, presenting with spinal and brain SAH. The patient underwent uneventful microsurgical en bloc resection. Postoperative course was normal. The second case concerned HBL with SAH at the cervico-medullary junction, with rapidly fatal course. The systematic review revealed female predominance, at a median age of 40 years, with HBL predominantly located at cervical level, common preoperative symptoms being headache and signs of meningeal irritation. ConclusionsIn conclusion, spinal HBL is an extremely rare cause of SAH. The systematic review found putative risk factors: female gender, age 40–50 years, cervical location, and median size 2cm. Diagnosis can be difficult when presentation mimics intracerebral SAH. We advocate early surgical removal. The risk of rapidly fatal course, in case of major haemorrhage, needs to be borne in mind.

Surgical management and outcome of adult posterior cranial fossa and spinal hemangioblastoma: a 6-case series and literature review

ABSTRACT Objective Hemangioblastomas of the posterior cranial fossa and spinal cord in adults are excessively vascularized, well-differentiated, and scarce tumors with no metastatic potential. This paper discusses its surgical management and outcome, pointing out their morphological, radiological, and histopathological aspects. This report based on a personal series of six patients and on a literature review. Methods A single-institution personal 6-case series of adult patients diagnosed and operated on by a senior neurosurgeon (KIA) due to posterior cranial fossa or spinal cord hemangioblastoma was analyzed. For easier understanding of hemangioblastoma, we have classified them into four different types. Results The tumors, which were all surgically treated, were located in the posterior cranial fossa in five patients (4 cerebellar, 1 brain stem) and intramedullary in the thoracic spinal cord in one patient. All patients successfully recovered neurologically after a complete tumor resection, having no post-operative neurological deficit or other complications. Conclusion Surgical management of cerebellar and spinal cord hemangioblastoma in adults is highly dependent on its morphological features, as well as on microsurgical technique applied. Since huge differences exist between the cystic/nodular tumor type (Type 1) and the solid type and its two additional variants (Types 2–4), morphology is the most important consideration when deciding surgical approach. Despite significant morphological differences among different subtypes of hemangioblastomas, their histology appears to be relatively similar. Nonetheless, a meticulous and refined surgical technique has to be utilized to achieve a successful outcome.

Spinal hemangioblastoma: surgical procedures, outcomes and review of the literature.

total removal of spinal hemangioblastomas with satisfactory clinical outcomes remains a challenge. We aimed to evaluate the surgical outcomes of our spinal hemangioblastomas patients, summarize our experiences with this condition and review-related literature. records of 18 spinal hemangioblastoma patients who underwent microsurgical resection were analyzed retrospectively. Clinical features, surgical procedures and outcomes were reviewed to assess the prognosis of their spinal hemangioblastomas. The McCormick classification method was used to evaluate spinal function and MR scans used to assess location and features of the tumor pre-surgically, tumor recurrence and syringomyelia status post-surgically. total resection of 37 tumors was achieved in all 18 cases. Of those patients, two (11%) were accompanied with von Hippel-Lindau (VHL). Of the 37 tumors, 3 (8.1%) were completely intramedullary, 16 (43.2%) intramedullary-extramedullary and 18 (48.6%) primarily extramedullary. Tumors accompanied with syringomyelia were present in 17 (94.4%) patients. Clinical symptoms such as pain were reduced within 48h after surgery in 16 patients. Post-operative neurological functions improved in all cases at 3months post-surgery. Over the subsequent 3-18month follow-up period, pre-operative symptoms improved in all 18 patients and no tumor recurrence was present. Syringomyelia was reduced or absent within 3-6months. our results indicated that a complete microscopic resection was effective in eliminating symptoms in these spinal hemangioblastoma patients, who showed a good prognosis after surgery. These improvements in clinical symptoms may be associated with the blood flow changes, but not with reductions and/or eliminations of syrinx after surgery.

Von Hippel-Lindau Disease (VHL): A Rare Radiological Case Report

Von Hippel-Lindau disease (VHL) is a rare autosomal dominant syndrome caused by mutation in the VHL tumor suppression gene located on chromosome 3. The presented case was a 13 years male patient who initially presented to our hospital with chief complaints of Weakness in b/l lower limbs, Low backache, Right-sided flank pain. On Physical examination there was a lump in right lumbar region– which was firm on palpation. On imaging and histopathology examination the patient was found to have multiple simple pancreatic cysts, malignant renal lesion, retialangioma and spinal hemangioblastoma. So a diagnosis of VHL was made. Regular follow-up with imaging (ultrasound, CT, MRI) are necessary to follow the previous lesions and detect any newly-developed VHL-associate tumors. The Importance of screening is emphasized because the lesions in VHL disease are treatable.

Microsurgical treatment of cervical spinal hemangioblastoma

BackgroundHemangioblastoma is a rare intracranial vascular tumor, mostly found in the cerebellum. Cervical spinal hemangioblastoma (CSHB) has rarely been reported, and microsurgical treatment even less. ObjectiveTo present our experience with CSHB and conduct a literature review, in order to provide a systematic approach to diagnosis and microsurgical treatment. MethodsThe study included 11 patients with CSHB treated by total tumor resection from January 2005 to December 2015. We analyzed clinical and radiological data, surgical outcome, and pathological examination results. ResultsAll patients were treated by total tumor resection. All preoperative nerve root pain resolved completely. Four patients had associated numbness, which improved in 2 and persisted in 2. All patients were in good postoperative health. There were no recurrences during follow-up of 6–48 months. ConclusionPreoperative magnetic resonance imaging (MRI) and computed tomography angiography (CTA) can be used for diagnosis of CSHB. Finding and blocking the nutrient arteries during surgery is necessary reduce bleeding. Total tumor resection requires accurately identifying and separating the proliferative area around the tumor.

Spinal Haemangioblastomas in Nigerians: 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 on these tumours among Nigerians. This study aims to evaluate the clinical profile and outcome of surgery as well as the challenges of the 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 centre 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 50 years. The onset of symptoms was 2 weeks in one patient and 18 months in the other. The tumour was located in the thoracic region in the 2 patients. One patient had extramedullary tumour. Both individuals were paraplegic at the time of surgery, which was 3 days in one patient and more than 4 months in the other. Whereas gross total tumour resection was achieved patient with shorter duration of symptoms and While both patients had thoracic lesions and presented with sensorimotor deficits, only one had sphincteric dysfunction. Patient had an intramedullary tumour location both instances, the extramedullary tumour made a rapid-post operative neurological improvement, while the one with the longer duration of symptoms and intramedullary tumour remained paraplegic until his death nine months after surgery.

Spinal extradural hemangioblastoma: A systematic review of characteristics and outcomes.

Extradural spinal nerve root hemangioblastoma is a rare entity with very few cases reported in the literature. A comprehensive picture of the treatments and outcomes of the same is thus not available. A systematic search was done according to PRISMA guidelines. Search criteria included terms: spinal extradural hemangioblastoma, extradural hemangioblastoma, and spinal root hemangioblastoma. The parameters considered were treatment, motor, and sensory outcome, association with von-Hippel-Lindau (VHL) syndrome. Twenty-two studies (19 full text articles) were available for the review. A total of 39 cases of extradural spinal nerve root hemangioblastoma have been reported. These cases had a median age of 44 years with male predominance (2:1) and up to 48% occur in the thoracic level, similar to our case. Thirty-six percent of patients were associated with VHL syndrome. Surgical resection was the primary modality of treatment with embolization used in selected cases (20%). They had mean follow-up of 23 (±11) months. The prognosis was better than the intradural counterpart with no motor deficit and sensory deficit in only 9%. Preoperative identification of the extradural nature of this pathology and complete excision at the first surgery offers excellent outcomes compared to intradural lesion. Targeted embolization may be used in cases anticipated with high blood loss.

Von Hippel–Lindau disease with renal cell carcinoma and multiple cerebellar and spinal hemangioblastomas without retinal manifestations: A case report

A 35-year-old gentleman presented with headache in the occipital region, neck pain, slurring of speech, and vomiting. There was reported history of Von Hippel–Lindau disease (VHL) in his mother, who was operated for a cerebellar hemangioblastoma. On investigations, magnetic resonance imaging brain showed bilateral cerebellar hemangioblastomas and a tiny spinal hemangioblastoma. Contrast-enhanced computed tomography abdomen showed multiple small well-defined, non-enhancing hypodense lesions with fluid attenuation in the left kidney and pancreas, suggestive of simple cysts. There was history of right partial nephrectomy in the past for renal cell carcinoma. Complete excision of bilateral cerebellar hemangioblastomas was performed. Histopathological examination and immunohistochemical workup confirmed the clinical diagnosis of hemangioblastoma.

Spinal haemangioblastomas in Nigerians: 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 on these tumours among Nigerians. This study aims to evaluate the clinical profile and outcome of surgery as well as the challenges of the 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 centre 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 50 years. The onset of symptoms was 2 weeks in one patient and 18 months in the other. The tumour was located in the thoracic region in the 2 patients. One patient had extramedullary tumour patient had an intramedullar tumour location. Both individuals were paraplegic at the time of surgery, which was 3 days in one patient and more than 4 months in the other. Whereas gross total tumour resection was achieved both instances, the patient with shorter duration of symptoms and While both patients had thoracic lesions and presented with sensorimotor deficits, only one had sphincteric dysfunction extramedullary tumour made a rapid-post operative neurological improvement, while the one with the longer duration of symptoms and intramedullary tumour remained paraplegic until his death nine months after surgery. Conclusion: Spinal haemangioblastomas are rare tumours. Long duration of symptoms and intramedullary tumour location may be predictors of unfavorable surgical outcome. Keywords: Haemangioblastoma, Spinal cord, Nigerian, Surgery, Outcome

Spinal Haemangioblastomas in Nigerians: 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 on these tumours among Nigerians. This study aims to evaluate the clinical profile and outcome of surgery as well as the challenges of the 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 centre 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 50 years. The onset of symptoms was 2 weeks in one patient and 18 months in the other. The tumour was located in the thoracic region in the 2 patients. One patient had extramedullary tumour. Both individuals were paraplegic at the time of surgery, which was 3 days in one patient and more than 4 months in the other. Whereas gross total tumour resection was achieved patient with shorter duration of symptoms and While both patients had thoracic lesions and presented with sensorimotor deficits, only one had sphincteric dysfunction. Patient had an intramedullary tumour location both instances, the extramedullary tumour made a rapid-post operative neurological improvement, while the one with the longer duration of symptoms and intramedullary tumour remained paraplegic until his death nine months after surgery.
 Conclusion: Spinal haemangioblastomas are rare tumours. Long duration of symptoms and intramedullary tumour location may be predictors of unfavorable surgical outcome.

Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report

Spinal and cerebellar hemangioblastomas are common in von Hippel-Lindau disease (vHLD) and usually treated surgically. Multifocal presence and surgically not amenable locations are issues that require a combined microsurgical and radiosurgical approach to control complex cases.We would like to present the case of a 37-year-old male patient who was diagnosed vHLD with multiple spinal and one infratentorial hemangioblastomas and holocord syrinx formation of the whole spinal cord. Combined microsurgical approaches to two spinal lesions and the cerebellar lesion followed by external beam radiotherapy of the posterior fossa and the whole spinal axis stabilized tumor growth of the asymptomatic lesions, while no recurrent tumors were detected at the site of surgery. A clinical deterioration connected to early postoperative deficits stabilized to a moderate gait ataxia. The follow-up after radiotherapy covered 60 months.A combination of microsurgery and radiosurgery for the surgically not amenable lesions is an adequate treatment regimen to stabilize tumor growth and clinical symptoms of multifocal spinal hemangioblastomas in vHLD, though the therapy should be limited to symptomatic or growing lesions.

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 50 years. 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