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Alena Skálová - One of the best experts on this subject based on the ideXlab platform.
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Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone
Advances in Therapy, 2018Co-Authors: William M. Mendenhall, Carlos Suárez, Alena Skálová, Primož Strojan, Asterios Triantafyllou, Kenneth O. Devaney, Michelle D. Williams, Alessandra Rinaldo, Alfio FerlitoAbstract: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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Endolymphatic Sac Tumor (aggressive papillary Tumor of middle ear and temporal bone): report of two cases with analysis of the VHL gene.
Pathology research and practice, 2008Co-Authors: Alena Skálová, Radek Sima, P. Bohuš, R. Čuřík, J. Lukáš, Michal MichalAbstract:Endolymphatic Sac Tumor (Heffner Tumor) (ELST) is a very rare nonmetastasizing, locally aggressive low-grade adenocarcinoma of Endolymphatic Sac origin, which is linked to von-Hippel-Lindau disease (VHLD). VHLD is an autosomal dominant disorder characterized by an inherited genetic abnormality of the VHL gene located on the short arm of chromosome 3 (3p26-p25). VHL gene mutations have been shown both in ELSTs associated with VHLD and in sporadic cases. Because of the rarity of ELST, only a small number of cases have been subjected to molecular genetic analysis. We have encountered two patients with ELST, one of whom presented with a medical and family history of VHLD. The second was a sporadic case, the patient having no symptoms of VHLD. The tissues obtained from Heffner Tumor and cerebellar hemangioblastoma from the patient with inherited VHLD possess a point mutation in exon 1 of VHL gene. This mutation is a C to T exchange at position 194, resulting in amino acid exchange S65L. No mutation was found in any of the three exons analyzed and in the exon-intron junctions of the VHL gene in the sporadic case.
Alfio Ferlito - One of the best experts on this subject based on the ideXlab platform.
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Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone
Advances in Therapy, 2018Co-Authors: William M. Mendenhall, Carlos Suárez, Alena Skálová, Primož Strojan, Asterios Triantafyllou, Kenneth O. Devaney, Michelle D. Williams, Alessandra Rinaldo, Alfio FerlitoAbstract: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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Endolymphatic Sac Tumor (low-grade papillary adenocarcinoma) of the temporal bone.
Acta oto-laryngologica, 2003Co-Authors: Kenneth O. Devaney, Alfio Ferlito, Alessandra RinaldoAbstract:The entity which has come to be known as an Endolymphatic Sac Tumor (ELST) has, in the past, been known as adenocarcinoma of Endolymphatic Sac origin, aggressive papillary Tumor of the temporal bone and Heffner's Tumor. ELSTs arise in the vicinity of the inner ear and may extend to involve both the posterior fossa as well as the middle ear and the external ear canal, which may complicate the differential diagnosis ELSTs are typically seen in adults, with only rare descriptions in pediatric patients. They may be sporadic Tumors or they may arise as part of the symptom complex of von Hippel-Lindau disease. Clinical signs at presentation range from a mass in the external ear canal to sensorineural deafness to cranial nerve palsies. Imaging studies reveal a destructive lesion of the petrous bone which is heterogeneous on MR scanning. Light microscopy reveals two chief patterns: a follicular pattern, reminiscent of thyroid parenchyma; and a papillary/solid pattern. Both patterns are often admixed in the same Tumor, and the individual Tumor cells are cytologically bland. Immunohistochemically, ELSTs are typically keratin-, vimentin- and epithelial membrane antigen-positive; they are often S-100 protein-positive and neuron-specific enolase-positive as well. ELSTs are difficult to extirpate surgically (owing to their locally aggressive nature); nevertheless, surgical excision remains the mainstay of current therapy. These are slow-growing (albeit locally aggressive) Tumors which have only rarely been reported to metastasize; as such, they remain principally a problem of local control.
Robert A. Ratcheson - One of the best experts on this subject based on the ideXlab platform.
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Endolymphatic Sac Tumor metastatic to the spine. Case report.
Journal of neurosurgery. Spine, 2005Co-Authors: Nicholas C. Bambakidis, Cliff A. Megerian, Tina Rodrigue, Robert A. RatchesonAbstract:Endolymphatic Sac Tumors (ELSTs) are aggressive papillary lesions of the temporal bone. Although histologically benign, they may exhibit invasive growth and destruction of the skull base. Patients generally present with symptoms referable to the lesion's location within the middle or posterior cranial fossa. Although well characterized as a distinct entity, ELSTs involved in metastatic dissemination have never been reported. In the present report the authors describe a case of ELST metastatic to the spine treated with resection.
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Differential grading of Endolymphatic Sac Tumor extension by virtue of von Hippel-Lindau disease status.
Otology & neurotology : official publication of the American Otological Society American Neurotology Society [and] European Academy of Otology and Neu, 2004Co-Authors: Nicholas C. Bambakidis, Cliff A. Megerian, Robert A. RatchesonAbstract:Objective:Endolymphatic Sac Tumors are aggressive papillary Tumors of the temporal bone frequently associated with von Hippel-Lindau disease. The goal of this study was to use a newly devised classification system as a means to analyze differences between Endolymphatic Sac Tumor extension in von Hip
Johannes Zenk - One of the best experts on this subject based on the ideXlab platform.
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Sporadic Endolymphatic Sac Tumor--a diagnostic and therapeutic challenge.
International journal of clinical and experimental pathology, 2014Co-Authors: Julian Künzel, Abbas Agaimy, Joachim Hornung, Michael Lell, Oliver Ganslandt, Sabine Semrau, Johannes ZenkAbstract:Endolymphatic Sac Tumor (ELST) is a rare low-grade locally aggressive neoplasm of the inner ear that may occur sporadically or in the setting of von Hippel-Lindau syndrome. We herein present a case of sporadic ELST in a 39-year-old man, treated using an interdisciplinary approach (surgery+radiotherapy), with a 10-year follow-up. The patient presented with hearing loss of sudden onset. The treatment of choice for ELST is radical Tumor resection, which is associated with a good long-term prognosis. Remission may last for years, but there may be local recurrences, probably as a result of incomplete resection. Adjuvant radiotherapy is an option in case of recurrence and could be discussed after incomplete resection. The purpose of this report is to call attention to ELSTs, which are difficult to diagnose due to their rarity and variety of presentations.
E. Kassel - One of the best experts on this subject based on the ideXlab platform.
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CASE REPORT Spinal Metastasis from Endolymphatic Sac Tumor
2015Co-Authors: K.y. Tay, E. KasselAbstract:SUMMARY: We describe a case of Endolymphatic Sac Tumor with drop metastasis to the spine. Our review of the literature showed that this is only the 2nd reported case of such an occurrence. Endolymphatic Sac Tumors (ELSTs) are rare neuroectoder-mal neoplasms of the petrous temporal bone. Patients present with symptoms including insidious onset of neuro-sensory hearing loss, tinnitus, and facial nerve palsy. These Tumors can be confusedwith other Tumors of the petrous tem-poral bone or posterior fossa on the basis of both the radio-logic and histopathologic characteristics. Although ELSTs are known to be locally aggressive, they very rarely metastasize. We describe a case of ELST with metastases to the spine. To our knowledge, this is only the 2nd case of drop metastases reported in the literature.1 Case Report A 27-year-old man with a history of Endolymphatic Sac Tumor was referred forMR imaging of the spine because of saddle numbness. H
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Spinal metastasis from Endolymphatic Sac Tumor.
AJNR. American journal of neuroradiology, 2007Co-Authors: K.y. Tay, E. KasselAbstract:We describe a case of Endolymphatic Sac Tumor with drop metastasis to the spine. Our review of the literature showed that this is only the 2nd reported case of such an occurrence.