Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage

2007 ◽  
Vol 107 (2) ◽  
pp. 421-425 ◽  
Author(s):  
Jay Jagannathan ◽  
John A. Butman ◽  
Russell R. Lonser ◽  
Alexander O. Vortmeyer ◽  
Christopher K. Zalewski ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Mr Imaging ◽  
Disease Status ◽  
Petrous Bone ◽  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Fluid Attenuated Inversion Recovery ◽  
History Of ◽  
Vhl Disease

✓ Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel–Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.

scholarly journals Endolymphatic Sac Tumor: A Rare Case

2020 ◽  
pp. 1-4
Author(s):  
Amal Algarni ◽  
Amal Algarni ◽  
Yaser Orz ◽  
Sofia Muzzafar ◽  
Wafa Alshakweer
Keyword(s):  
Hearing Loss ◽  
Temporal Bone ◽  
Rare Case ◽  
Disease Patient ◽  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Petrous Portion ◽  
Vhl Disease

Endolymphatic sac tumor is rare, locally aggressive and non-metastasizing neoplasm arising from the endolymphatic sac of the petrous portion of the temporal bone. Most occur in adult and present with ipsilateral hearing loss. They can be sporadic or associated with Von-Hippel-Lindau disease. Patient with endolymphatic sac tumor should be screened for VHL disease. We report a case of 52-year-old female with dizziness and headache. Histopathology was consistent with typical features of endolymphatic sac tumor. This was confirmed by cytokeratin and EMA positivity and TTF-1 negativity. This case is presented for its rarity with only few cases reported.

Imaging detection of endolymphatic sac tumor–associated hydrops

2013 ◽  
Vol 119 (2) ◽  
pp. 406-411 ◽  
Author(s):  
John A. Butman ◽  
Edjah Nduom ◽  
H. Jeffrey Kim ◽  
Russell R. Lonser
Keyword(s):  
Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Clinical Findings ◽  
Endolymphatic Sac ◽  
Membranous Labyrinth ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Episodic Vertigo ◽  
Contrast Enhanced ◽  
Vhl Disease

Object To determine if physiologically based MRI sequences can be used to detect endolymphatic sac tumor (ELST)–associated hydrops, the authors performed contrast-enhanced delayed FLAIR imaging in consecutive ELST patients with clinical findings consistent with hydrops. Methods Consecutive patients with von Hippel-Lindau (VHL) disease and clinical findings of endolymphatic hydrops and ELSTs underwent contrast-enhanced delayed FLAIR MRI. Clinical, audiological, operative, and imaging findings were analyzed. Results Three patients (2 male, 1 female) with 4 ELSTs (1 patient had bilateral ELSTs) were identified who had clinical findings consistent with endolymphatic hydrops. Computed tomography and MRI evidence of an ELST was found in all patients. Their mean age at initial evaluation was 39.7 years (range 28–51 years). All patients demonstrated progressive sensorineural hearing loss that was associated with episodic vertigo and tinnitus. Contrast-enhanced delayed FLAIR MRI clearly demonstrated dilation of the membranous labyrinth consistent with hydrops in the affected ears but not the unaffected ears. Two patients underwent resection of the associated ELST that resulted in stabilization of progressive hearing loss, as well as amelioration of tinnitus and vertigo. Conclusions Contrast-enhanced delayed FLAIR MRI can be used to detect ELST-associated hydrops. Noninvasive MRI detection of hydrops can permit earlier detection of ELSTs in patients with VHL disease and provides direct insight into a mechanism that underlies ELST-associated audiovestibular morbidity.

Tumors of the endolymphatic sac in patients with von Hippel–Lindau disease: implications for their natural history, diagnosis, and treatment

2005 ◽  
Vol 19 (2) ◽  
pp. 503-512
Author(s):  
H. Jeffrey Kim ◽  
John A. Butman ◽  
Brewer Carmen ◽  
Christopher Zalewski ◽  
Alexander O. Vortmeyer ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Natural History ◽  
Tumor Resection ◽  
Endolymphatic Sac ◽  
Von Hippel Lindau ◽  
Cranial Nerve Dysfunction ◽  
Imaging Characteristics ◽  
Vestibular Symptoms ◽  
Loss Of Hearing ◽  
Vhl Disease

Object Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel–Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs. Methods Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed. Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence. Conclusions Audiovestibular symptoms, including hearing loss, in patients with VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.

scholarly journals Multicranial neuropathy secondary to endolymphatic sac tumor

2021 ◽  
Author(s):  
Tanvir Hussain ◽  
Thomas J. Crotty ◽  
Eoin F. Cleere ◽  
Mel Corbett ◽  
Aishan Patil ◽  
...  
Keyword(s):  
Skull Base ◽  
Surgical Resection ◽  
Cranial Nerves ◽  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Lateral Skull Base ◽  
Complete Surgical Resection ◽  
Close Proximity ◽  
History Of

<p class="abstract">Endolymphatic sac tumors (ELST) are rare neuroectodermal neoplasms arising from the epithelium of the endolymphatic sac or duct. Despite their benign histopathological features, ELSTs frequently demonstrate a locally destructive growth pattern with involvement of the skull base and cranial nerves. While ELSTs may arise sporadically, the majority of cases occur in association with Von Hippel-Lindau (VHL) syndrome. ELSTs are commonly diagnosed late due to their slow growing nature and non-specific symptomatology. Surgical resection is the treatment of choice. However, due to the location of these tumors in the lateral skull base surgical intervention carries a high risk of injury to critical neurovascular structures in close proximity. We presented the case of a 51 years old gentleman with a three-months history of hearing loss and otalgia. He subsequently developed multiple cranial neuropathies and was diagnosed with a sporadic ELST. He underwent a complete surgical resection and received adjuvant radiotherapy.  </p>

Endolymphatic sac tumors in von Hippel—Lindau disease

2004 ◽  
Vol 100 (3) ◽  
pp. 480-487 ◽  
Author(s):  
Daniel Choo ◽  
Lawrence Shotland ◽  
Maryann Mastroianni ◽  
Gladys Glenn ◽  
Carter van Waes ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Family Members ◽  
Magnetic Resonance Images ◽  
Endolymphatic Sac ◽  
Specific Gene ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

Object. Von Hippel—Lindau (VHL) disease is a hereditary multiple-neoplasia syndrome mapping to chromosome 3p25–26. Endolymphatic sac (ELS) tumors have been identified as a neoplastic manifestation of VHL disease. The purpose of this study was to evaluate comprehensively the natural history of inner ear disease in a large population of patients with confirmed or suspected VHL disease and to correlate the clinical features with the VHL genotype. Methods. The authors collated and analyzed clinical and genotypic data obtained in patients enrolled in an Institutional Review Board—approved protocol in which families and individuals affected by VHL disease were studied. These data included results from multidisciplinary history workups and physical examinations, imaging studies, and a battery of audiological tests. One hundred seventy-five patients were enrolled in the study, 129 with confirmed VHL disease and 46 of their family members in whom test results for VHL disease were negative and who served as controls. Twenty-one patients had ELS tumors that were evident on magnetic resonance images; three of them had bilateral ELS lesions. Hearing loss, often sudden in onset and severe to profound in nature, vestibulopathy, aural fullness, and tinnitus represented the primary symptoms of ELS tumor. Distinct patterns of auditory and vestibular dysfunction occurred at different stages of the disease. Phenotypic data showed that 17 of 21 patients with ELS tumors did not have pheochromocytomas, whereas all had VHL disease affecting the kidney, all but two had VHL disease affecting the central nervous system, and all but one had disease affecting the pancreas. Genotyping revealed 10 rearrangements (partial deletions), eight single bp substitutions, and one 3-bp insertion. Although there was no difference in the incidence of hearing loss between populations, symptoms of imbalance and aural fullness were more common in patients with VHL disease but without imaging evidence of ELS tumor than they were in family members who did not have VHL disease (p < 0.01). Conclusions. Endolymphatic sac tumors are frequently associated with VHL disease. Symptoms of disequilibrium or aural fullness in patients with VHL disease may be an early indication of endolymphatic dysfunction. Patients with VHL disease provide a unique opportunity to examine the effects of specific gene mutations and a discrete neoplastic process on the human inner ear. The study of ELS tumors in this group also provides a pathological model of ELS function and supplies evidence for a role of the ELS in clinical Ménière-like disease(s).

Endolymphaticsac tumor – case report

2017 ◽  
Vol 6 (1) ◽  
pp. 39-42
Author(s):  
Jerzy Kuczkowski ◽  
Wojciech Brzoznowski ◽  
Tomasz Nowicki ◽  
Jolanta Szade
Keyword(s):  
Temporal Bone ◽  
Bone Destruction ◽  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
History Of ◽  
Temporal Bone Tumor ◽  
Von Hippel Lindau Syndrome ◽  
Tumor Remission

The aim of this paper is to present the case of a 70-year-old women with endolymphatic sac tumor and temporal bone destruction treated at Otolaryngology Department of MUG. The patient was admitted to our Department due to a 3-year history of hearing loss, dizziness and ear pain. The first diagnosis was temporal bone tumor connected with von Hippel-Lindau syndrome (VHL). The patient was surgically treated. During intraoperative examination, a neoplasm was determined. The tumor was excised via transmastoid approach with sigmoid sinus skeletonization. After treatment, her pains disappeared. Histopathological and immunohistochemical examination revealed endolymphatic sac tumor. Follow-up CT showed no tumor remission.

Tumors of the endolymphatic sac in patients with von Hippel—Lindau disease: implications for their natural history, diagnosis, and treatment

2005 ◽  
Vol 102 (3) ◽  
pp. 503-512 ◽  
Author(s):  
H. Jeffrey Kim ◽  
John A. Butman ◽  
Carmen Brewer ◽  
Christopher Zalewski ◽  
Alexander O. Vortmeyer ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Natural History ◽  
Tumor Resection ◽  
Endolymphatic Sac ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Cranial Nerve Dysfunction ◽  
Vestibular Symptoms ◽  
Vhl Disease ◽  
Fine Print

Object. Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel—Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs. Methods. Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed. Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence. Conclusions. Audiovestibular symptoms, including hearing loss, in patients with VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.

Endolymphatic Sac Tumor Screening and Diagnosis in von Hippel-Lindau Disease: A Consensus Statement

2021 ◽  
Author(s):  
Gautam U. Mehta ◽  
H. Jeffery Kim ◽  
Paul W. Gidley ◽  
Anthony B. Daniels ◽  
Mia E. Miller ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Clinical Evaluation ◽  
Evaluation Framework ◽  
Screening Tools ◽  
Endolymphatic Sac ◽  
Expert Committee ◽  
Specific Test ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

Abstract Objective Endolymphatic sac tumors (ELSTs) are a frequent cause of hearing loss and other audiovestibular dysfunction in patients with von Hippel-Lindau disease (VHL). Unified screening recommendations for VHL patients have not been established. To develop consensus guidelines, the VHL Alliance formed an expert committee to define evidence-based clinical screening recommendations. Patients and Methods Recommendations were formulated by using the Grading of Recommendations, Assessment, Development, and Evaluation framework after a comprehensive literature review. Results Diagnosis of ELSTs in VHL requires a combination of clinical evaluation and imaging and audiometric findings. Audiovestibular signs/symptoms are often an early feature of small ELSTs, including those that are not visible on imaging. Diagnostic audiograms have the greatest sensitivity for the detection of ELST-associated sensorineural hearing loss and can help confirm clinically relevant lesions, including those that may not be radiographically evident. Magnetic resonance imaging (MRI) can be a more specific test for ELSTs in VHL particularly when supplemented with computed tomography imaging for the identification of small tumors. VHL patients between the ages 10 and 60 years carry high preponderance for ELST presentation. Conclusion We recommend that clinical evaluation (yearly) and diagnostic audiograms (every other year) be the primary screening tools for ELSTs in VHL. We suggest that screening be performed between the ages 11 and 65 years or with the onset of audiovestibular signs/symptoms for synchronicity with other testing regimens in VHL. We recommend that baseline imaging (MRI of the internal auditory canals) can be performed between the ages of 15 and 20 years or after positive screening.

Endolymphatic Sac Tumor and von Hippel-Lindau Disease

2000 ◽  
Vol 175 (3) ◽  
pp. 925-926 ◽  
Author(s):  
K. Ayadi ◽  
Kh. Ben Mahfoudh ◽  
M. Khannous ◽  
J. Mnif
Keyword(s):  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease
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