Gamma knife treatment of an endolymphatic sac tumor: Unique features of a case and review of the literature

Xing, Cheng; Qin, Shu Kui; Wang, Xing; Li, Peng; Shrestha, Balkrishna; Wang, Wei

doi:10.4103/0028-3886.84348

Cited by 8 publications

(14 citation statements)

References 17 publications

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“…[ 4 9 ] Patients commonly develop unilateral sensorineural hearing loss; ipsilateral facial nerve paresis, otalgia, otorrhea, tinnitus, vertigo, headache, and ataxia are also common features. [ 5 9 ] As in our case, hydrocephalus may also develop. Depending on tumor extension, dysfunction of other cranial nerves (including Vernet's syndrome and trigeminal symptoms) may also arise.…”

Section: Discussionsupporting

confidence: 62%

“…[ 21 ] Because of ELSTs’ complex microscopic features, the differential diagnosis includes schwannoma, meningioma (including malignant meningioma with adenocarcinoma-like metaplasia), jugular paraganglioma, choroid plexus papillomas, solitary fibrous tumor with salivary gland inclusion as well as metastases from papillary thyroid and renal cell carcinomas. [ 5 6 9 ] In our case, meningioma proved to be the main differential concern.…”

Section: Discussionmentioning

confidence: 57%

“…Depending on tumor extension, dysfunction of other cranial nerves (including Vernet's syndrome and trigeminal symptoms) may also arise. [ 5 7 ] Distant dissemination is very uncommon but a few metastatic cases have been reported. [ 2 3 5 9 22 ]…”

Section: Discussionmentioning

confidence: 99%

“…Hemorrhage, calcifications, siderophages, cholesterol clefts, and inflammatory cells are frequently described. [ 5 ] The immunohistochemical profile is usually positive for cytokeratin (types 5,6,7,8,17,19), Epithelial Membrane Antigen (EMA), Vimentin, and Periodic acid-schiff; ELSTs may also stain positive for Neuron specific enolase (NSE), S100, and Glial FibrillarAcid Protein (GFAP). [ 6 9 ] Transthyretin can be effective to differentiate ELSTs from choroid plexus papillomas; its absence or very limited expression in collected samples points to ELSTs.…”

Section: Discussionmentioning

confidence: 99%

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Gamma knife radiosurgery in the management of endolymphatic sac tumors

Sinclair¹,

Al-Saffar²,

Brigui³

et al. 2018

Surg Neurol Int

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Background:Although widely regarded as rare epithelial tumors with a low grade of malignancy, endolymphatic sac tumors (ELST) often lead to disabling petrous bone destruction and significantly impairing symptoms at the time of primary diagnosis and/or recurrence. ELST is not uncommon in von Hippel Lindau (VHL) patients. Although open surgery is regarded as the best treatment option, recurrence remains a challenge, particularly when gross tumor resection (GTR) is deemed unachievable due to topographic conditions. Tumor recurrence successfully treated with fractionated radiotherapy and radiosurgery have been reported in selected cases. We present the case of a patient with recurrent ELST treated with salvage gamma knife radiosurgery (GKRS) adding a review of current literature.Case Description:A 65-year-old patient underwent GKRS of an unresectable, recurrent ELST. Tumor volumetric analysis showed almost 15% increase in tumor volume in the 4 months between the pre-GKRS magnetic resonance imaging (MRI) and the stereotactic MRI (s-MRI) at treatment. Follow-up MRI at 12 and 20 months showed significant decrease in local tumor volume, decreased contrast enhancement and no perifocal edema. The patient's general and neurological status remains stable to the present day.Conclusion:In the present case, GKRS was effective in the management of a recurrent ELST over the course of 20 months. Because of ELSTs recurrence potential, long-term follow up is required. The present case as well as previous reports might suggest a possible salvage/adjunctive role of radiosurgery in the management of ELST. Further studies are deemed necessary.

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Section: Discussionsupporting

confidence: 62%

Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Gamma knife radiosurgery in the management of endolymphatic sac tumors

Sinclair¹,

Al-Saffar²,

Brigui³

et al. 2018

Surg Neurol Int

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“…In our case, the complete removal of the tumor was possible because of the small size of the mass that did not need preoperative embolization. Other treatment options such as gamma or cyber-knife need to be evaluated on larger series [21]. …”

Section: Discussionmentioning

confidence: 99%

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

Ferri¹,

Amadori

Armato

et al. 2014

Case Reports in Otolaryngology

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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.

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