scholarly journals Sporadic Intradural Extramedullary Hemangioblastoma of Cauda Equina with Large Peritumoral Cyst—A Rare Presentation

2022 ◽  
Author(s):  
Pushpa B. Thippeswamy ◽  
Dilip C. R. Soundararajan ◽  
Ríshi M. Kanna ◽  
Venkata S. Kuna ◽  
Shanmuganathan Rajasekaran
Keyword(s):  
Cauda Equina ◽  
Surgical Excision ◽  
Vascular Tumor ◽  
Spinal Tumors ◽  
Rare Presentation ◽  
Von Hippel Lindau ◽  
Drop Metastasis ◽  
Neural Axis ◽  
Von Hippel Lindau Disease ◽  
Intradural Extramedullary

AbstractCauda equina intradural tumors commonly reported include ependymoma, schwannoma, neurofibroma, meningioma, and drop metastasis. Hemangioblastoma of the neural axis is a rare benign vascular tumor comprising only 1.6 to 6.4% of spinal tumors, and are usually associated with Von-Hippel Lindau disease. Sporadic intradural extramedullary hemangioblastoma involving cauda equina is very rare with only countable reports, and the presence of peritumoral cyst has been reported only once. We report one such case of hemangioblastoma with a large peritumoral cyst, which was diagnosed radiologically and confirmed by histopathology following surgical excision. Pertinent radiological characteristics, diagnostic clues, treatment, and surgical outcomes are discussed.

Cauda Equina Syndrome as First Manifestation of von Hippel-Lindau Disease

2019 ◽  
Vol 125 ◽  
pp. 316-319
Author(s):  
Xinji Chen ◽  
Guokang Xu ◽  
Qing Bi ◽  
Yazeng Huang ◽  
Haiyu Shao ◽  
...  
Keyword(s):  
Cauda Equina Syndrome ◽  
Cauda Equina ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

scholarly journals Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

2008 ◽  
Vol 109 (2) ◽  
pp. 313-317 ◽  
Author(s):  
Martin Baggenstos ◽  
Emily Chew ◽  
John A. Butman ◽  
Edward H. Oldfield ◽  
Russell R. Lonser
Keyword(s):  
Color Vision ◽  
Vision Loss ◽  
Tumor Resection ◽  
Vascular Tumor ◽  
Peritumoral Edema ◽  
Von Hippel Lindau ◽  
Extended Transsphenoidal Approach ◽  
Von Hippel Lindau Disease ◽  
Optic Fibers ◽  
Reversible Visual Loss

Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultrafiltrate through permeable neoplastic vessels. The authors report the clinical and imaging findings in a 62-year-old man with von Hippel–Lindau disease who presented with rapid (within 24 hours) loss of color vision and nearcomplete loss of left eye vision (acuity too poor to test). Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations. Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma. Postoperatively, the patient recovered color vision and had improvement in visual acuity (20/320). Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss. Tumor resection led to resolution of the edema and improvement in visual function.

scholarly journals Medullary hemangioblastoma in a child with von Hippel-Lindau disease: vascular tumor perfusion depicted by arterial spin labeling and dynamic contrast-enhanced imaging

2015 ◽  
Vol 16 (1) ◽  
pp. 50-53 ◽  
Author(s):  
Hyun Woo Goo ◽  
Young-Shin Ra
Keyword(s):  
Arterial Spin Labeling ◽  
Vascular Tumor ◽  
Spin Labeling ◽  
Diagnostic Value ◽  
Tumor Perfusion ◽  
Von Hippel Lindau ◽  
Dynamic Contrast Enhanced Imaging ◽  
Dynamic Contrast Enhanced ◽  
Contrast Enhanced ◽  
Von Hippel Lindau Disease

Medullary hemangioblastoma is very rare in children. Based on small nodular enhancement with peritumoral edema and without dilated feeding arteries on conventional MRI, hemangioblastoma, pilocytic astrocytoma, oligodendroglioma, and ganglioglioma were included in the differential diagnosis of the medullary tumor. In this case report, the authors emphasize the diagnostic value of arterial spin labeling and dynamic contrast-enhanced MRI in demonstrating vascular tumor perfusion of hemangioblastoma in a 12-year-old boy who was later found to have von Hippel-Lindau disease.

scholarly journals Surgical outcome of intradural extramedullary spinal cord tumors - Our experience at a tertiary health care center

2020 ◽  
Vol 1 (2) ◽  
pp. 103-109
Author(s):  
Gyanendra Joshi ◽  
Binod Bijukachhe ◽  
Javed Ahmad Khan
Keyword(s):  
Spinal Cord ◽  
Cauda Equina ◽  
Clinical Results ◽  
Spinal Tumors ◽  
Myxopapillary Ependymoma ◽  
Spinal Cord Tumors ◽  
Vas Score ◽  
Post Surgery ◽  
Intradural Extramedullary

Introduction: To report the treatment results of 19 patients who underwent excision of intradural extramedullary (IDEM) spinal tumors. Materials & Methods:  This is a retrospective study. Patients’ records were retrieved from the electronic database of Grande International Hospital. There were 19 IDEM spinal tumors excised over a period of 6 years between January 2013 and August 2019 by a single surgeon. There were 11 (57.9%) males and 8 (42.1%) females with an average age of 48.37±21.87 years (range, 5-79 years). The mean postoperative follow-up period was 12.87±14.88 months (range, 15 days - 60 months). The histopathological findings, locations of the tumors, and clinical results were analyzed. Neurological findings were evaluated using ASIA grading system and pain was evaluated using VAS score. Results:  Histopathologically the tumors were: 8 meningioma (42.1%), 4 schwannoma (21.1%), 4 neurofibroma (21.1%), 1 dermoid cyst (5.3%), 1 lipoma (5.3%), and 1 myxopapillary ependymoma (5.3%). Tumor locations were: dorsal in 10 (52.6%), lumbar in 5 (26.3%), lumbosacral in 2 (10.5%), dorsolumbar in 1 (5.3%), and cervical in 1 (5.3%) patient. Neurologic status of 7 patients was normal and 12 had neurologic involvement with 3 patients having Cauda Equina Syndrome (CES) preoperatively. At the final follow-up, 3-grade, 2-grade and 1-grade improvement in ASIA score was observed in 1, 6 and 1 cases, respectively. There was no change in the ASIA grade in 1 case. All 3 cases of CES achieved full neural recovery. Preoperative VAS score was 9.21±1.08 and improved significantly to 0.74±1.62 after surgery. Neurological improvement was seen in 91.67% with complete recovery in 75% of the cases and not a single case deteriorated neurologically post-surgery. Conclusion:  Most intradural-extramedullary spinal cord tumors are mostly benign and good clinical results can be obtained when adequately treated with surgery.

Adenomas of the Temporal Bone

1988 ◽  
Vol 97 (6) ◽  
pp. 605-612 ◽  
Author(s):  
Thomas L. Eby ◽  
Miro S. Makek ◽  
Ugo Fisch
Keyword(s):  
Tumor Invasion ◽  
Bone Destruction ◽  
Complete Removal ◽  
Vascular Tumor ◽  
Petrous Bone ◽  
Von Hippel Lindau ◽  
Subtotal Petrosectomy ◽  
Benign Process ◽  
Von Hippel Lindau Disease ◽  
Ear Symptoms

Three cases of primary adenomas of the petrous bone are presented and compared to an adenoma arising from the external ear canal. In contrast to other adenomatous tumors, these caused slowly progressive inner ear symptoms without other signs, hence making differentiation from other benign lesions difficult. In one case, association with von Hippel-Lindau disease is suspected because of a strong family history and recent discovery of a similar lesion in the opposite ear. Bone destruction was found radiographically and clinically with vascular tumor tissue and cysts permeating the bone, causing adjacent bone destruction and remodeling. The histopathologic examination demonstrated this to be a benign process, however, with no direct tumor invasion or other signs of malignancy. Subtotal petrosectomy is a proven approach for complete removal of these tumors. Review of the literature revealed no previous reports of benign adenomas originating in the petrous bone.

Leptomeningeal Hemangioblastomatosis in a Case of von Hippel-Lindau Disease: Case Report

Neurosurgery ◽  
2003 ◽  
Vol 52 (5) ◽  
pp. 1212-1216
Author(s):  
Nicolas Reyns ◽  
Richard Assaker ◽  
Etienne Louis ◽  
Jean-Paul Lejeune
Keyword(s):  
Case Report ◽  
Cauda Equina ◽  
Lower Limbs ◽  
Pathological Examination ◽  
Pia Mater ◽  
Von Hippel Lindau ◽  
Leptomeningeal Spread ◽  
Shunt Procedure ◽  
Disease Case ◽  
Von Hippel Lindau Disease

Abstract OBJECTIVE AND IMPORTANCE We report a unique case of extended leptomeningeal hemangioblastomatosis in a patient presenting with clinical von Hippel-Lindau disease. CLINICAL PRESENTATION A 50-year-old male patient had a history of three surgical procedures for the removal of a cerebellar hemangioblastoma, initially considered to be a recurrence of a sporadic form at the same location. Seven years after the last operation, he developed chronic hydrocephalus. Despite a ventriculoperitoneal shunt procedure, he experienced progressive worsening of gait disturbances, associated with touch numbness of the lower limbs and Parinaud's syndrome. Magnetic resonance imaging of the brain and spine showed evidence of leptomeningeal contrast enhancement around the brainstem, spinal cord, and cauda equina and enlarged tortuous vessels around the mesencephalon. INTERVENTION A lumbar laminectomy allowed a leptomeningeal biopsy. Pathological examination revealed leptomeningeal spread of the hemangioblastoma. It is assumed that the tumor arose in the pia mater and that its direction of growth was purely extramedullary, invading all subarachnoid spaces. The patient had a poor outcome as a result of progressive tetraplegia and died 6 months after diagnosis as a result of respiratory failure. CONCLUSION To the best of our knowledge, the clinical course of our patient, consistent with a thick leptomeningeal spread of hemangioblastoma from the posterior fossa to the sacrum, is unique. Nevertheless, the short life expectancy of our patient is usual in von Hippel-Lindau disease. This case report illustrates the crucial challenge to develop a specific drug therapy related to angiogenesis in von Hippel-Lindau disease.

scholarly journals Extradural thoracic nerve root hemangioblastoma approached by a combined posterior thoracic spine and video-assisted thoracoscopic surgery: A case report

2022 ◽  
Vol 13 ◽  
pp. 10
Author(s):  
Diogo Roque ◽  
Daniel Cabral ◽  
Cristina Rodrigues ◽  
Nuno Simas
Keyword(s):  
Nerve Root ◽  
Thoracoscopic Surgery ◽  
Arterial Embolization ◽  
Surgical Excision ◽  
Cord Compression ◽  
Spinal Root ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Video Assisted Thoracoscopic Surgery ◽  
Complete Tumor

Background: Hemangioblastomas commonly occur in the posterior fossa and are typically attributed to sporadic or familial Von Hippel–Lindau disease. Spinal hemangioblastomas, found in 7–10% of patients, are usually located within the cord (i.e., intramedullary). Here, a 58-year-old male presented with a purely extradural hemangioblastoma involving a spinal root that was surgically excised. Case Description: A 58-year-old male was admitted with a progressive paraparesis and incomplete sensory deficit. The magnetic resonance imaging documented a solid dumbbell-shaped lesion that extended through the left T3-T4 foramen resulting in nerve root and spinal cord compression. Following arterial embolization and lesion excision by both neurosurgeons and thoracic surgeons, the patient’s deficits improved. The postoperative computed tomography scan documented complete tumor removal, and the neuropathology revealed a hemangioblastoma. Conclusion: Here, we describe a 58-year-old male with a purely extradural thoracic foraminal T3-T4 dumbbell-shaped hemangioblastoma successfully treated by both embolization and surgical excision.

Multiple intradural-extramedullary ependymomas: proven dissemination by genetic analysis

2010 ◽  
Vol 12 (5) ◽  
pp. 467-473 ◽  
Author(s):  
Murat Vural ◽  
Ali Arslantas ◽  
Evrim Ciftci ◽  
Sevilhan Artan ◽  
Baki Adapinar
Keyword(s):  
Epidermal Growth Factor Receptor ◽  
Genetic Analysis ◽  
Histopathological Examination ◽  
Growth Factor Receptor ◽  
Spinal Tumors ◽  
Neural Axis ◽  
Epidermal Growth ◽  
Intradural Extramedullary ◽  
Conventional Cytogenetics

This 45-year-old woman was admitted with neck and back pain and difficulty in ambulation that had been progressively worsening for 2 years. Admission MR imaging revealed a cervicomedullary junction tumor and 2 intradural-extramedullary spinal tumors located separately at the levels of T5–6 and T8–9. All masses were successfully resected in a 2-stage operation. Histopathological examination of the surgical specimens revealed that all the lesions were ependymomas. Genetic analysis was performed to determine if the tumors were related. Conventional cytogenetics, multiplex fluorescence in situ hybridization (M-FISH), interphase-FISH specific to 22q11, and epidermal growth factor receptor loci analyses of the tumor samples revealed that the lesions originated from the same primary tumor. Although 3 simultaneous tumors in different compartments of the neural axis were diagnosed as ependymoma by histopathological examination, it was not possible to be sure if their multiplicity was due to spread of tumor cells via CSF or if it was due to multicentric foci. Thus, genetic analysis of the tumor samples is essential to confirm the exact mechanism of development of multiple ependymomas.

scholarly journals Intradural extramedullary hemangioblastoma of the thoracic cord: A case report

2021 ◽  
Vol 12 ◽  
pp. 126
Author(s):  
Masahiro Kawanishi ◽  
Hidekazu Tanaka ◽  
Yutaka Itoh ◽  
Kunio Yokoyama ◽  
Makoto Yamada ◽  
...  
Keyword(s):  
Rare Case ◽  
Spinal Cord Tumors ◽  
Lower Extremity Weakness ◽  
Tumor Excision ◽  
Von Hippel Lindau ◽  
Left Lower Extremity ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
Intradural Extramedullary ◽  
Thoracic Cord

Background: Spinal hemangioblastomas account for 1–3% of all spinal cord tumors and are mostly intramedullary in location. Here, we report an intradural extramedullary hemangioblastoma of the thoracic spine, occurring in in a patient without von Hippel-Lindau disease. Case Description: A 58-year-old female had a 5-year history of progressive left lower extremity weakness. When the MR demonstrated an intradural/extramedullary lesion with a syrinx at the T2-3 level, she successfully underwent gross total tumor excision following which she neurologically improved. Conclusion: Here, we report a rare case of an intradural/extramedullary thoracic hemangioblastoma successfully excised at the T 2-3 level in a patient without von Hippel-Lindau disease.

Sign in / Sign up

Export Citation Format

Share Document