Humans ; Hemangioblastoma/complications* ; Von Hippel-Lindau Tumor Suppressor Protein

Hemangioblastoma (HBL) in the suprasellar region is very rare and a few cases have been reported. Suprasellar HBL without von Hippel-Lindau disease is much rarer. A 76-year old male patient presented progressively deteriorating visual disturbance. MRI demonstrated solid suprasellar mass of 20 mm in diameter, broadly based to planum sphenoidale and diaphragm sella and dural tail sign after the administration of gadolinium diethylene triamine penta-acetic acid (Gd-DTPA). Preoperative diagnosis was meningioma. Total resection of the tumor was not accomplished because of massive hemorrhage, and the histopathologic examination revealed the tumor to be HBL. The visual disturbance of the patient was not improved. The authors reviewed the literature and considered a differential diagnosis of suprasellar tumors and treatment of suprasellar HBL.
Diagnosis ; Diagnosis, Differential ; Diaphragm ; Gadolinium ; Hemangioblastoma ; Hemorrhage ; Humans ; Magnetic Resonance Imaging ; Male ; Meningioma ; Tail ; Temazepam ; von Hippel-Lindau Disease

BACKGROUND: Only sporadic reports of fluorescence-guided surgery (FGS) have been published for non-glioma conditions. In this study, we focus on epidemiological data of fluorescence patterns and report the diverse experiences of FGS in non-gliomas. METHODS: During 8.5 years between July 2010 and January 2019, 900 FGS for brain tumor performed in Seoul National University Hospital. Among them, a total of 73 histologically proven non-glioma patients were analyzed. Indications for FGS have been the possibility of anaplastic tumor in intra-axial tumors in preoperative MRI and an attempt to reproduce known anecdotal experiences of 5-Aminolevulinic Acid (5-ALA) fluorescence. RESULTS: In cases of brain tumors except for gliomas, the most frequent cases were brain metastasis (23 cases) followed by lymphomas (9 cases) and meningeal tumors (8 cases). And there were embryonal tumors (6 cases), hemangioblastomas (4 cases), and solitary fibrous tumor/hemangiopericytomas (3 cases). Most brain metastases, meningiomas, primary central nervous system lymphomas, and treatment effect cases showed positive fluorescence. Moreover, some non-tumorous conditions also showed positive fluorescence. However, hemangioblastoma and germ cell tumor did not observe any fluorescence at all. CONCLUSION: 5-ALA induced fluorescence is not limited to glioma but is also evident in non-glioma and non-neoplastic conditions. This 5-ALA-induced fluorescence may be used as an intraoperative tool for various brain conditions.
Brain ; Brain Neoplasms ; Central Nervous System ; Fluorescence ; Glioma ; Hemangioblastoma ; Humans ; Lymphoma ; Magnetic Resonance Imaging ; Meningeal Neoplasms ; Meningioma ; Neoplasm Metastasis ; Neoplasms, Germ Cell and Embryonal ; Pathology ; Seoul

To investigate the value of diffusion-weighted imaging(DWI)and apparent diffusion coefficient(ADC)in the diagnosis and differential diagnosis of posterior fossa solid hemangioblastoma(PFSH). We retrospectively analyzed the clinical data of 15 PFSH patients and 58 patients with other hypervascular tumors in the posterior fossa(the latter included 23 cases of meningioma,5 cases of medulloblastoma,8 cases of acoustic neuroma,4 cases of hemangiopericytoma,5 cases of lymphoma,9 cases of metastatic tumor,3 cases of astrocytoma,and 1 case of choroid plexus papilloma)confirmed by operation and pathology.All patients underwent axial DWI scans,and the mean ADC value of solid part of the tumors and the adjacent normal cerebellar white matter were measured,and then the normalized ADC was calculated.In addition, test was used to compare the differences in mean ADC and normalized ADC between these two groups,and receiver operating characteristic(ROC)curve was applied to analyze the diagnostic performance of normalized ADC. Of all the 15 PFSH patients,DWI appeared hypointense in 12 patients and isointense in 3 patients;the signals on ADC maps were isointense or hyperintense;the mean ADC value of PFSHs was(1.881±0.445)×10 mm /s and the normalized ADC was 2.70±0.62.In contrast,in 58 patients with other tumors in the posterior fossa,DWI appeared hyperintense in 51 cases,isointense in 3 cases,and hypointense in 4 cases;the mean ADC value was(0.771±0.202)×10 mm /s,and the normalized ADC was 1.17±0.33.Thus,the ADC value and normalized ADC value were significantly higher in PFSH than in other tumors in the posterior fossa(=9.419,<0.001;=9.184,<0.001).The cut-off value of the normalized ADC for the diagnosis of solid hemangioblastoma was 1.89,with the sensitivity and specificity being 100%and 96.6%respectively,and the area under the ROC curve was 0.989. ADC and normalized ADC are valuable in the differential diagnosis of PFSH from other tumors with abundant blood supply.
Diagnosis, Differential ; Diffusion Magnetic Resonance Imaging ; Hemangioblastoma ; diagnosis ; Humans ; ROC Curve ; Retrospective Studies

Objective To analyze the clinical features of Von-Hippel-Lindau(VHL)syndrome and explore the diagnostic value of abdominal ultrasound for this disease.Methods The clinical features including age at first diagnosis,symptoms,signs,affected organs,number of operations,and diagnostic examinations of 35 patients with VHL syndrome admitted to our center from January 1994 to December 2017 were retrospectively analyzed.The diagnostic value of abdominal ultrasound for VHL syndrome was analyzed.Results Pheochromocytoma(=14)and nervous system hemangioblastoma(=13)were the common firstly-identified tumors.Nervous system hemangioblastoma(=21),pheochromocytoma(=19),renal carcinoma(=17),and pancreatic mass(=15)were common tumors.The main surgical reasons were nervous system hemangioblastoma(=22),pheochromocytoma(=23)and renal carcinoma(=13).Abdominal organ involvements were found in 33 patients,which were first detected by abdominal ultrasound in 20 patients and were found accidently during routine health checkups in 6 patients.The ultrasound results were accurate in 27 of 33 adrenal gland scans,13 of 16 pancreas scans,and 8 of 19 kidney scans.Conclusions When multiple tumors are detected in the kidney,adrenal gland,and pancreas by ultrasound,the possibility of VHL syndrome should be considered.When the clinical findings suggest the possibility of VHL syndrome,ultrasound can discover and diagnose the abdominal tumors and can also be used for the long-term follow-up of the tumors.Therefore,ultrasound is an important method in the screening and follow-up of patients with VHL syndrome.
Abdomen ; diagnostic imaging ; Adrenal Gland Neoplasms ; diagnostic imaging ; Hemangioblastoma ; diagnostic imaging ; Humans ; Pheochromocytoma ; diagnostic imaging ; Retrospective Studies ; Ultrasonography ; von Hippel-Lindau Disease ; complications ; diagnostic imaging

BACKGROUND: Tumors with cysts often correlate with gliomas, metastatic tumors, or hemangioblastomas, which require differentiation. METHODS: Thirty-eight cases of cyst associated-meningioma based on preoperative radiologic studies and histologic confirmations were reviewed from November 1998 to July 2017. RESULTS: A total of 395 cases of meningioma were observed in the 20 years, and surgical treatment of intracranial meningioma was performed in 120 cases. Thirty-eight (9.6%) cases of cyst associated meningiomas were analyzed. Nauta type I was the most common type of cyst (39.5%) and the most frequent histopathological subtype was meningothelial type (36.8%). CONCLUSION: Statistically there were no significant associations between meningioma histopathological type and associated cysts; however, the rate of World Health Organization grade II was higher in cyst associated meningiomas than in unrelated meningiomas. This correlation was weak, in accordance with the meningioma grade.
Glioma ; Hemangioblastoma ; Meningioma* ; Neuropathology ; World Health Organization

We reported a case of supratentorial extra-axial hemangioblastoma diagnosed by magnetic resonance imaging (MRI). As a simple solid mass,this tumor has its unique MRI features due to its tissue structures.
Hemangioblastoma ; diagnostic imaging ; Humans ; Magnetic Resonance Imaging ; Supratentorial Neoplasms ; diagnostic imaging

This report presents a case of fourth ventricle neurenteric cyst (NE cyst) mimicking hemangioblastoma, which developed in a 50-year-old woman. A tiny enhancing mural portion of the fourth ventricle in MRI suggested that the cyst was hemangioblastoma, but pathological evidence showed that the cyst was in fact NE cyst in the fourth ventricle. In order to make proper decision on to what extent of surgical resection should be done, considering every possibility in differential diagnosis might be helpful. This case reports an unusual pathology in 4th ventricle, considering the patient's age, and demonstrates that a rarer disease may share radiological features of a common disease.
Diagnosis, Differential ; Female ; Fourth Ventricle* ; Hemangioblastoma* ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neural Tube Defects* ; Pathology

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

von Hippel-Lindau (VHL) disease is an autosomal dominant inherited tumor syndrome associated with mutations of the VHL tumor suppressor gene located on chromosome 3p25. The loss of functional VHL protein contributes to tumorigenesis. This condition is characterized by development of benign and malignant tumors in the central nervous system (CNS) and the internal organs, including kidney, adrenal gland, and pancreas. We herein describe the case of a 74-year-old man carrying the VHL gene mutation who was affected by simultaneous colorectal adenocarcinoma, renal clear cell carcinoma, and hemangioblastomas of CNS.
Adenocarcinoma* ; Adrenal Glands ; Aged ; Carcinogenesis ; Carcinoma, Renal Cell* ; Central Nervous System ; Colorectal Neoplasms ; Genes, Tumor Suppressor ; Hemangioblastoma* ; Humans ; Kidney ; Pancreas ; von Hippel-Lindau Disease*