Endolymphaticsac tumor – case report

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.

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

Journal of Neurosurgery ◽

10.3171/jns.1997.87.3.0445 ◽

1997 ◽

Vol 87 (3) ◽

pp. 445-449 ◽

Cited By ~ 11

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.

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ENDOLYMPHATIC SAC TUMOR AS INITIAL MANIFESTATION OF VON HIPPEL–LINDAU SYNDROME

Retinal Cases & Brief Reports ◽

10.1097/icb.0b013e318124a4c8 ◽

2008 ◽

Vol 2 (4) ◽

pp. 332-334 ◽

Cited By ~ 2

Author(s):

Pedro F. Salazar ◽

Carol L. Shields ◽

Miguel A. Materin ◽

Jerry A. Shields

Keyword(s):

Endolymphatic Sac ◽

Initial Manifestation ◽

Endolymphatic Sac Tumor ◽

Von Hippel Lindau ◽

Von Hippel Lindau Syndrome

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Clinical response to pazopanib in a patient with endolymphatic sac tumor not associated with von Hippel-Lindau syndrome

CNS Oncology ◽

10.2217/cns-2019-0019 ◽

2020 ◽

Vol 9 (1) ◽

pp. CNS50

Author(s):

Thomas Nelson ◽

Jethro Hu ◽

Serguei Bannykh ◽

Xuemo Fan ◽

Jeremy Rudnick ◽

...

Keyword(s):

Surgical Intervention ◽

Kinase Inhibitor ◽

Endolymphatic Sac ◽

Vascular Tumors ◽

Subtotal Resection ◽

Variable Response ◽

Endolymphatic Sac Tumor ◽

Von Hippel Lindau ◽

Von Hippel Lindau Syndrome ◽

Early Surgical Intervention

Endolymphatic sac tumors (ELSTs) are rare, locally invasive, vascular tumors of the temporal bone. These lesions are associated with von Hippel-Lindau syndrome but may arise sporadically. Early surgical intervention is recommended to prevent permanent neurologic deficits; however, many ELSTs are unresectable or are subtotally resected due to neurovascular compromise. Chemotherapeutic salvage therapy in trials of neoplasms of associated syndromes has targeted angiogenesis with variable response. We present the case of a sporadic ELST, previously minimally responsive to bevacizumab, treated with pazopanib, a multi-kinase inhibitor and antiangiogenic, with good response. Cases such as our patient may demonstrate the utility of novel antiangiogenics in the treatment of these rare neoplasms, particularly when the tumor is unresectable or necessitates subtotal resection.

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Multicranial neuropathy secondary to endolymphatic sac tumor

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20213657 ◽

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>

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Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage

Journal of Neurosurgery ◽

10.3171/jns-07/08/0421 ◽

2007 ◽

Vol 107 (2) ◽

pp. 421-425 ◽

Cited By ~ 14

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.

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Endolymphatic Sac Tumor: A Rare Case

Journal of Surgical Oncology ◽

10.31487/j.jso.2020.04.04 ◽

2020 ◽

pp. 1-4

Author(s):

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.

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Endolymphatic Sac Tumor

Philippine Journal of Otolaryngology Head and Neck Surgery ◽

10.32412/pjohns.v24i1.717 ◽

2009 ◽

Vol 24 (1) ◽

pp. 37-38

Author(s):

Jose M. Carnate ◽

Amado O. Tandoc

Keyword(s):

Facial Nerve ◽

Middle Ear ◽

Nerve Palsy ◽

Facial Nerve Palsy ◽

Thyroid Tissue ◽

Bone Destruction ◽

Endolymphatic Sac ◽

Endolymphatic Sac Tumor ◽

Left Posterior ◽

History Of

We present the case of a 48 year old lady with a history of episodic hearing loss and tinnitus of several years duration. One month prior to consult, there was note of left occipital pain. No history of dizziness, vertigo or facial nerve palsy was elicited. She was neither a smoker nor an alcoholic beverage drinker. No other co-morbidities were elicited. Physical examination revealed a 4 cm diameter left posterior auricular mass which was tender. There was note of a bluish bulge on the left posterior wall of the external auditory canal. The tympanic membrane was intact. The MRI revealed a 5 cm diameter, irregular, avidly enhancing mass at the left mastoid bone with permeative bone destruction and indentation of the left cerebellar hemisphere and left superior temporal lobe but without evidence of brain invasion. A biopsy was performed followed by a pre-operative tumor embolization then a sub-total petrosectomy with mastoid obliteration. Histologic sections showed an unencapsulated mass with bony invasion composed of cystically dilated glandular structures containing colloid-like material (Fig. 1) while other areas showed simple and coarse papillae (Fig. 2). The cells were cuboidal to columnar and had a bland cytomorphology with little nuclear pleomorphism (Fig. 3). Mitoses and necrosis were absent. The general histology had a striking resemblance to either normal thyroid tissue or papillary thyroid carcinoma. A TTF-1 immunohistochemical stain however showed negative nuclear staining (Fig. 4). We signed out the case as an Endolymphatic Sac Tumor. This tumor has been known in the past by such synonyms as “Aggressive Papillary Middle Ear Tumor”, “Heffner Tumor” and “Low-grade Adenocarcinoma of the Middle Ear”. It is rare, affects both sexes in roughly equal frequencies and often presents with hearing and vestibular dysfunctions, facial nerve palsy and a mass. It presents radiologically as a multilocular lytic lesion in the petrous area of the temporal bone with bone destruction. Because of the histologic resemblance to thyroid tissue, a metastatic thyroid neoplasm is a differential diagnosis. Metastases to this area are rare, cases invariably have a known primary focus and otologic symptoms are uncommon. Immunohistochemical studies and clinical correlation are helpful in ruling out a metastasis. Treatment is primarily surgical. Prognosis is generally good but is dependent on the extent of the lesion at presentation. It is locally destructive, has the capacity to damage adjacent nerves and is recurrent if incompletely excised. Death may result from a large, destructive lesion in a vital area. To date, there are no reports of metastasis which may make the term “adenocarcinoma” not entirely appropriate. We have limited follow-up information on our present case at this time.

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