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>

Phosphaturic Mesenchymal Tumors of the Sinonasal Area and Skull Base: Experience at a Single Institution

2021 ◽  
pp. 000348942110374
Author(s):  
Davis P. Argersinger ◽  
Catherine T. Haring ◽  
John E. Hanks ◽  
Kevin J. Kovatch ◽  
S. Ahmed Ali ◽  
...  
Keyword(s):  
Skull Base ◽  
Surgical Resection ◽  
Medical Center ◽  
Fibroblast Growth Factor 23 ◽  
Academic Medical Center ◽  
Relevant Literature ◽  
Case Series ◽  
Radiological Evidence ◽  
Lateral Skull Base ◽  
Complete Surgical Resection

Objectives: Phosphaturic mesenchymal tumor (PMT) is a rare, polymorphous neoplasm with a highly variable presentation and natural history and unpredictable clinical course. The primary objective was to describe our clinical experience with and management of 4 markedly different cases of sinonasal and skull base PMT. Methods: A retrospective case series with chart review, and relevant literature review, was performed at a tertiary academic medical center between 1998 and 2020. Adult patients treated for PMTs of the sinonasal area and skull base were included. Our main outcome measures included postoperative laboratory findings and radiological evidence of disease remission. Results: Four patients (2 Males, 2 Females; Mean Age: 63.5 years) with PMTs of the skull base have been managed at our institution since 1998. Patient presentations varied, ranging from severe phosphorus wasting and osteoporosis to symptoms secondary to mass effect, including nasal obstruction and rhinorrhea. All 4 patients were eventually found to have elevated levels of fibroblast growth factor 23. Tumors were located in the sinonasal area (right frontal sinus, right ethmoid sinus, and right nasal cavity, respectively) in 3 patients and in the lateral skull base (right jugular foramen) in 1 patient. All 4 patients underwent complete surgical resection of their tumors. PMT tissue pathology was confirmed in all cases. Gross total resection was achieved in all patients. There was no chemical or radiological evidence of disease recurrence in any patients at follow-up. Conclusions: The presentation of skull base PMT is variable, and it may mimic other mass pathologies of the head and neck. Complete surgical resection with negative margins is potentially curative.

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.

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.

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

VHL gene inactivation in an endolymphatic sac tumor associated with von Hippel- Lindau disease

Neurology ◽  
1999 ◽  
Vol 53 (1) ◽  
pp. 208-208 ◽  
Author(s):  
N. Kawahara ◽  
H. Kume ◽  
K. Ueki ◽  
K. Mishima ◽  
T. Sasaki ◽  
...  
Keyword(s):  
Gene Inactivation ◽  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Vhl Gene ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

Primary facial nerve paraganglioma: report and review of the literature

2020 ◽  
Vol 13 (12) ◽  
pp. e237537
Author(s):  
Jonathan Austin Berry ◽  
Cherie Ann O Nathan ◽  
Ashley B Flowers ◽  
Gauri Mankekar
Keyword(s):  
Facial Nerve ◽  
Surgical Resection ◽  
Facial Paralysis ◽  
Clinical Importance ◽  
Mri Imaging ◽  
Review Of The Literature ◽  
Surveillance Imaging ◽  
Complete Surgical Resection ◽  
History Of ◽  
Ct And Mri

This report describes the diagnosis and treatment of a patient with a rare primary facial nerve paraganglioma as well as a review of the current literature. A 60-year-old male patient presented to our clinic with a 4-month history of left-sided progressive facial paralysis House-Brackmann V. Biopsy taken during facial nerve (FN) decompression confirmed the diagnosis of paraganglioma. The left FN was sacrificed during resection of the mass and a 12-7 jump graft, using the left greater auricular nerve, was performed with acceptable outcomes. The rarity of these tumours does not discount their clinical importance or the necessity to include them in the differential when presented with unilateral FN paralysis. Investigation should begin with CT and MRI imaging to identify and localise the potential mass. Histologic confirmation requires tissue. While surveillance imaging is occasionally an option, often complete surgical resection of the mass and sacrifice of the nerve is necessary.

Endolymphatic sac tumor and angiomatous lesions of the nasal and pharyngeal mucosa as a first manifestation of von Hippel-Lindau disease

2012 ◽  
Vol 122 (10) ◽  
pp. 2300-2303 ◽  
Author(s):  
Clemens Honeder ◽  
Wolfgang Gstoettner ◽  
Christian Matula ◽  
Christian Czerny ◽  
Andreas Gruber ◽  
...  
Keyword(s):  
Endolymphatic Sac ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Pharyngeal Mucosa ◽  
Von Hippel Lindau Disease

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel—Lindau disease

1997 ◽  
Vol 87 (3) ◽  
pp. 445-449 ◽  
Author(s):  
Jean-Christophe Ouallet ◽  
Kathlyn Marsot-Dupuch ◽  
Remy Van Effenterre ◽  
Michele Kujas ◽  
Jean-Michel Tubiana
Keyword(s):  
Temporal Bone ◽  
Imaging Techniques ◽  
Endolymphatic Sac ◽  
Cystic Tumor ◽  
Content Type ◽  
Von Hippel Lindau ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
Magnetic Resonance Imaging Techniques ◽  
Temporal Bone Tumor

✓ This report describes a patient with von Hippel—Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.

scholarly journals Endolymphatic sac tumor and von Hippel-Lindau disease in a single family

2009 ◽  
Vol 67 (4) ◽  
pp. 1097-1099 ◽  
Author(s):  
Patrícia Pires Netto Safatle ◽  
Luciano Farage ◽  
André Sampaio ◽  
Frederico Abreu Soares Ferreira ◽  
Heloisa Pires Netto Safatle ◽  
...  
Keyword(s):  
Endolymphatic Sac ◽  
Single Family ◽  
Endolymphatic Sac Tumor ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease
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