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24-year-old female with endolymphatic sac tumor. T2W axial MR image reveals a heterogeneous mass lesion involving the left endolymphatic sac, petrosal bone, and mastoid air cells.
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Endolymphatic sac tumor is an uncommon, locally aggressive tumor. The tumor is located in the medial and posterior petrosal bone region and may involve the dura. A hypervascular tumor involving the endolymphatic sac with destructive changes, it involves the bone and may show reactive new bone formation. Diagnosis is based on clinical, radiological,...
Citations
... Опухоль эндолимфатического мешка (ОЭМ) -редкая, медленно растущая, но агрессивная опухоль. Первое описание ОЭМ появилось в литературе в 1989 г. Это было эпителиальное новообразование, которое, по предположению автора, возникло в эндолимфатическом мешке (ЭМ) и было обозначено как аденокарцинома [1]. Новообразование в начальной стадии расположено на задней поверхности каменистой части височной кости и медиальнее, может вовлекать в патологический процесс твердую мозговую оболочку [2]. ...
... У пациентов с ОЭМ часто наблюдаются сенсоневральная потеря слуха и шум в ухе, реже оталгия, головокружение, атаксия, нейропатия лицевого нерва, оторея, которые прогрессируют постепенно [48,76]. По мере прогрессирования опухоли пациенты могут испытывать головокружение, атаксию, поражение тройничного, лицевого и преддверно-улиткового нервов, боль, шум в ухе [1]. Парез или паралич лицевого нерва наблюдается в случаях распространения опухоли до канала лицевого нерва и поражения самого нерва. ...
... Оптимальным методом лечения является резекция патологически измененных тканей до здоровых [1]. В некоторых случаях невозможно достичь полного удаления опухоли [96]. ...
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.
... Our assumption was based on the location of the tumour, extending more superiorly than tympanojugular paragangliomas typically do. However, endolymphatic sac tumours are usually centred at the level of the posterior semicircular canal and generally spare jugular foramen [17]. In our first case, the tumour centred at the jugular foramen and from there extended superiorly to erode the posterior semicircular canal. ...
Low-grade fibromyxoid sarcoma (LGFMS) is a rare slow-growing malignant tumour with a deceptively benign histologic appearance. Herein, we report two cases of LGFMS with variable clinical presentations. The first case was a 17-year-old female who referred to our department due to deaf ear on the right together with ipsilateral gag reflex impairment and globus sensation in the pharynx. The second case was a 35-year-old female with recurrent LGFMS, suffering from headaches, vertigo, and episodes of loss of consciousness. LGFMS of the temporal bone is a rare pathology, and to the best of our knowledge, no such cases have been reported before.
A good knowledge of the normal anatomy of the temporal bone is key to the accurate interpretation of temporal bone imaging. Correlation with clinical history and physical examination is essential to provide a correct diagnosis or appropriate differential. Computed tomography is the method of choice for imaging of middle ear disease, conductive hearing loss, and temporal bone fractures, although MRI can add important information for cholesteatomatous disease. Patients with suspected lesions of the cerebellopontine angle or internal auditory canal are best examined with MRI. In cases of inner ear congenital malformations and petrous apex lesions, MRI and computed tomography are complementary. The value of both modalities is discussed in this chapter.
Endolymphatic sac tumors (ELSTs) are rare, papillary adenomatous tumors that arise from the endothelium of the endolymphatic sac. We demonstrate a difficult case of endolymphatic sac tumor and how it is managed via transcanal endoscopic assisted technique, with discussion of feasibility of transcanal approach to lateral skull base tumor.
