Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone

Poletti, Alessandro; Dubey, Siba P.; Colombo, Giovanni; Cugini, Giovanni; Mazzoni, Antonio

doi:10.1016/j.rpor.2015.06.003

Cited by 19 publications

(21 citation statements)

References 23 publications

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“…[ 27 ] Despite the above, other groups have questioned the efficacy of post-operative conventional fractionated radiotherapy due to the significant number of recurrences reported at post surgery (up to 50% after STR and 20% after GTR); the same groups suggested instead the use of radiosurgery in poor surgical candidates or when the morbidity of salvage surgery is deemed significant. [ 19 ] A number of publications seem to support the latter. Cheng et al .…”

Section: Discussionmentioning

confidence: 97%

“…Early GTR is widely regarded as the treatment of choice as it often leads to long disease-free intervals. [ 6 9 11 16 17 26 ] A large number of surgical procedures are available according to the tumor size and extension[ 5 19 ]. Nevertheless, GTR is often unachievable due to local anatomical factors[ 9 10 ] such as cranial nerve involvement and focal complex vascular supply and drainage.…”

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: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Gamma knife radiosurgery in the management of endolymphatic sac tumors

Sinclair¹,

Al-Saffar²,

Brigui³

et al. 2018

Surg Neurol Int

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“…3). Experience with stereotactic radiosurgery (SRS) and with fractionated radiotherapy (RT) is limited [3,8,48,49]. Generally, low-grade adenocarcinomas are unlikely to be radiosensitive and complete resection with negative margins is likely to be the treatment of choice.…”

Section: Treatmentmentioning

confidence: 99%

“…The proximal segment of the endolymphatic sac is contiguous with the endolymphatic duct [3]. The posterior segment of the endolymphatic sac is in the dura of the posterior cranial fossa.…”

Section: Introductionmentioning

confidence: 99%

Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

et al. 2018

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An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues. The likelihood of regional or distant metastases is remote. The optimal treatment is resection with negative margins. Patients with positive margins, gross residual disease, or unresectable tumor are treated with radiotherapy or radiosurgery. Late recurrences are common, so long follow-up is necessary to assess efficacy. The likelihood of cure depends on tumor extent and is probably in the range of 50-75%.

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“…Results on the pituitary adenomas, 10 cavernous sinus meningioma, 11 temporal bone carcinoma, 12 endolymphatic sac tumour of the temporal bone 13 and acoustic neuroma treated by surgery 14 or radiotherapy 15 are reported by the authors who describe and discuss the literature data on the basis of their personal experience.…”

mentioning

confidence: 99%