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.

Endolymphatic sac tumours

2003 ◽  
Vol 117 (8) ◽  
pp. 666-669 ◽  
Author(s):  
P. S. Richards ◽  
A. G. Clifton
Keyword(s):  
Papillary Thyroid Carcinoma ◽  
Thyroid Carcinoma ◽  
Local Excision ◽  
Petrous Bone ◽  
Endovascular Embolization ◽  
Endolymphatic Sac ◽  
Papillary Thyroid ◽  
Von Hippel Lindau ◽  
Papillary Tumour ◽  
Von Hippel Lindau Disease

We present a case of a papillary tumour of the petrous bone. The established terminology for this rare neoplasm is endolymphatic sac tumour (ELST) but the true origin remains controversial. ELSTs are associated with von Hippel-Lindau disease. They are locally invasive, highly vascular and often require endovascular embolization prior to surgery. Both radiologically and histologically ELSTs are easily mistaken for other more common tumours such as paragangliomas and renal or papillary thyroid carcinoma metastases. This is important because local excision is curative.

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 The vestibular aqueduct: site of origin of endolymphatic sac tumors

2008 ◽  
Vol 108 (4) ◽  
pp. 751-756 ◽  
Author(s):  
Russell R. Lonser ◽  
Martin Baggenstos ◽  
H. Jeffrey Kim ◽  
John A. Butman ◽  
Alexander O. Vortmeyer
Keyword(s):  
Petrous Bone ◽  
National Institutes Of Health ◽  
Endolymphatic Sac ◽  
Preoperative Imaging ◽  
Pathological Findings ◽  
Von Hippel Lindau ◽  
Vestibular Aqueduct ◽  
Endolymphatic Duct ◽  
Von Hippel Lindau Disease ◽  
Resolution Imaging

Object Although endolymphatic sac tumors (ELSTs) frequently destroy the posterior petrous bone and cause hearing loss, the anatomical origin of these neoplasms is unknown. To determine the precise topographic origin of ELSTs, the authors analyzed the imaging, operative, and pathological findings in patients with von Hippel–Lindau disease (VHL) and ELSTs. Methods Consecutive VHL patients with small (≤ 1.5 cm) ELSTs who underwent resection at the National Institutes of Health were included. Clinical, imaging, operative, and pathological findings were analyzed. Results Ten consecutive VHL patients (6 male and 4 female) with 10 small ELSTs (≤ 1.5 cm; 9 left, 1 right) were included. Serial imaging captured the development of 6 ELSTs and revealed that they originated within the intraosseous (vestibular aqueduct) portion of the endolymphatic duct/sac system. Imaging just before surgery demonstrated that the epicenters of 9 ELSTs (1 ELST was not visible on preoperative imaging) were in the vestibular aqueduct. Inspection during surgery established that all 10 ELSTs were limited to the intraosseous endolymphatic duct/sac and the immediately surrounding region. Histological analysis confirmed tumor within the intraosseous portion (vestibular aqueduct) of the endolymphatic duct/sac in all 10 patients. Conclusions ELSTs originate from endolymphatic epithelium within the vestibular aqueduct. High-resolution imaging through the region of the vestibular aqueduct is essential for diagnosis. Surgical exploration of the endolymphatic duct and sac is required for complete resection.

scholarly journals A Rare Case of Endolymphatic Sac Tumour: Clinicopathologic Study and Surgical Management

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Emanuele Ferri ◽  
Maurizio Amadori ◽  
Enrico Armato ◽  
Ida Pavon
Keyword(s):  
Bone Erosion ◽  
Complete Removal ◽  
Sporadic Case ◽  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Clinicopathologic Study ◽  
Von Hippel Lindau Disease ◽  
Canal Wall Down ◽  
Weighted Sequences

Objective. Endolymphatic sac tumor (ELST) is a rare neoplasm arising from the intrapetrous portion of the endolymphatic sac, either isolated or in association with the von Hippel-Lindau disease. We report a sporadic case of ELST with an overview of the literature and a discussion of clinic-radiological, histopathologic, and surgical findings.Case Report. A young woman presented with a progressive hearing loss in the left ear. Otoscopy showed a reddish, bleeding hypotympanic mass. CT demonstrated an expansile lytic mastoid lesion extending to the middle ear, with bone erosion. MRI confirmed a lesion of increased signal on T1-weighted sequences. The patient underwent a canal wall-down tympanoplasty with complete removal of the tumor. Histopathology was consistent with a papillary ELST. Immunohistochemistry was positive for cytokeratin and chromogranin A.Conclusion. This paper highlights the rarity of ELST, the need for an accurate neuroradiological and immunohistochemical study at the early stages, and the timeliness of surgical treatment.

scholarly journals Pathological and Clinical Features and Management of Central Nervous System Hemangioblastomas in von Hippel-Lindau Disease

2014 ◽  
Vol 1 (4) ◽  
pp. 46-55 ◽  
Author(s):  
Hiroshi Kanno ◽  
Natsuki Kobayashi ◽  
Satoshi Nakanowatari
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Vascular Tumor ◽  
Preoperative Embolization ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Vhl Disease ◽  
Perioperative Hemorrhage

Central nervous system (CNS) hemangioblastoma is the most common manifestation of von Hippel-Lindau (VHL) disease. It is found in 70-80% of VHL patients. Hemangioblastoma is a rare form of benign vascular tumor of the CNS, accounting for 2.0% of CNS tumors. It can occur sporadically or as a familial syndrome. CNS hemangioblastomas are typically located in the posterior fossa and the spinal cord. VHL patients usually develop a CNS hemangioblastoma at an early age. Therefore, they require a special routine for diagnosis, treatment and follow-up. The surgical management of symptomatic tumors depends on many factors such as symptom, location, multiplicity, and progression of the tumor. The management of asymptomatic tumors in VHL patients is controversial since CNS hemangioblastomas grow with intermittent quiescent and rapid-growth phases. Preoperative embolization of large solid hemangioblastomas prevents perioperative hemorrhage but is not necessary in every case. Radiotherapy should be reserved for inoperable tumors. Because of complexities of VHL, a better understanding of the pathological and clinical features of hemangioblastoma in VHL is essential for its proper management.

Endolymphatic sac carcinoma of the right petrous bone in Von Hippel–Lindau disease

2006 ◽  
Vol 13 (4) ◽  
pp. 471-474 ◽  
Author(s):  
D.P. Muzumdar ◽  
A. Goel ◽  
S. Fattepurkar ◽  
N. Goel
Keyword(s):  
Petrous Bone ◽  
Endolymphatic Sac ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Right

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.

The yield and cost of radiological screening in von Hippel-Lindau disease

2019 ◽  
Author(s):  
Timothy McMillan ◽  
Anju Sahdev ◽  
Jane Evanson ◽  
Lorraine McAndrew ◽  
Lee Martin ◽  
...  
Keyword(s):  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease
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