Tumor del saco endolinfático y enfermedad de von hippel-lindau. Revisión de la literatura

Minteguiaga, C. De; Ibáñez, L. García; Huy, P. Tran Ba

doi:10.1016/s0001-6519(02)78344-1

Cited by 8 publications

(2 citation statements)

References 24 publications

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“…Пациенты с болезнью фон Гиппеля -Линдау (von Hippel -Lindau) чаще подвержены развитию ОЭМ [44][45][46][47]. Болезнь обусловлена инактивацией гена-супрессора опухоли на коротком плече 3-й хромосомы и наследуется как аутосомно-доминантный признак с переменной экспрессией [48][49][50][51][52][53][54][55][56][57]. У некоторых из этих пациентов с болезнью фон Гиппеля -Линдау ОЭМ могут быть двусторонними, хотя встречаются и спорадические случаи [58][59][60][61].…”

Section: опухоль эндолимфатического мешкаunclassified

Endolymphatic sac tumor: rewiev of literature

et al. 2022

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Endolymphatic sac tumors are rare neoplasms of the temporal bone, histologically benign, but clinically behaves as malignant tumors causing destruction of surrounding tissues. The tumor originates from the posterior surface of petrous part of temporal bone, where endolymphatic sac is situated anatomically. The tumor causes destruction of the posterior surface of the petrous part of the temporal bone, inner ear, mastoid process, etc. Endolymphatic sac tumor can be both sporadically and in patients with von Hippel – Lindau disease. Due to the rarity of this tumor, it is easy to confuse it with other tumors, such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary thyroid carcinoma or papilloma of the vascular plexus. Computed tomography (CT) and magnetic resonance imaging (MRI) are of great diagnostic importance and play an important role in planning treatment tactics. The optimal method of treatment is resection of pathologically altered tissues. In some cases (incomplete resection of tumor, the patients with concomitant diseases or inoperable cases) receive courses of X-ray or radiosurgery. If a tumor is detected in the early stages, the volume of resection can be minimized while preserving hearing and vestibular function of the inner ear. Recurrence usually happens due to difficulty to identify the extension of the tumor. Diagnosis and correct preoperative planning, with embolization if it possible, will facilitate surgery and avoid subtotal tumor resection due to intraoperative bleeding. Long follow-up period is important in order to avoid recurrences. Insufficient coverage of this problem in the literature is associated with a low percentage of its occurrence in otosurgical practice, which complicates the timely diagnosis and treatment of this type of tumors of the temporal bone, worsens the prognosis.

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Section: опухоль эндолимфатического мешкаunclassified

Endolymphatic sac tumor: rewiev of literature

et al. 2022

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“…Thus, particularly in patients with clinical manifestations simulating Ménière's disease, the endolymphatic sac region must be carefully examined in order not to miss an ELST [25]. ELSTs may be sporadic or associated with von Hippel-Lindau (VHL) disease which is attributable to inactivation of a tumor suppressor gene on the short arm of chromosome 3, and inherited as an autosomal dominant trait with variable expression [1,14,15,20,[26][27][28][29][30][31][32][33][34][35][36][37]. VHL predisposes patients to multiple hemangioblastomas of the retina or central nervous system as well as tumors and cysts in various organs, including renal cell carcinoma, pheochromocytoma, paraganglioma, ELST, epididymal cystadenoma, and pancreatic serous cystadenoma or neuroendocrine tumors [1].…”

Section: Clinical and Radiographic Featuresmentioning

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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