Endolymphatic sac tumor in a patient with von Hippel-Lindau disease: MR imaging findings.
نویسنده
چکیده
A 29-year-old male patient with von Hippel-Lindau disease who had an operation for cerebellar hemangioblastoma six years ago is presented. The patient had a four-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by MR imaging, was almost completely cystic in appearance. Pathological examination after removal of the mass confirmed a papillary endolymphatic sac tumor.
منابع مشابه
Endolymphatic Sac Tumors: Mr Imaging Findings in Two Siblings
Endolymphatic sac tumors are aggressive papillary tumors of the temporal bone that could be associated with von Hippel-Lindau disease (VHL). We present imaging findings of a VHL family with two cases of endolymphatic sac tumors. These tumors spread to the cerebellum and the cerebellopontine cistern and may mimic more frequent tumors of this region. They show predominantly high signals on T1and ...
متن کاملDeafness due to bilateral endolymphatic sac tumours in a case of von Hippel-Lindau syndrome.
A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal bone. Histo...
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CONTEXT Endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and cause irreversible sensorineural hearing loss (SNHL) and vestibulopathy. The underlying mechanisms of audiovestibular morbidity remain unclear and optimal timing of treatment is not known. OBJECTIVE To define the mechanisms underlying audiovestibular pathophysiology associated with ELSTs. DESIGN, SETT...
متن کاملEndolymphatic sac tumours and von Hippel-Lindau disease - case report, molecular analysis and histopathological characterization.
Endolymphatic sac tumours (ELST) are aggressive papillary tumours of the temporal bone. The name was finally determined after the endolymphatic sac was determined as the site of their origin. They should be considered in patients with tumours eroding the petrous part of the temporal bone, extending to the cerebellopontine angle or other adjacent structures. These very rare tumours in the genera...
متن کاملCross-Sectional Imaging Spectrum of von Hippel-Lindau Disease
von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that is associated with germline mutations of VHL tumor suppressor gene, which is located at short arm of chromosome 3. This condition predisposes to the development of various benign and malignant tumors involving multiple organs; the common neoplasms include central nervous system hemangioblastomas (mostly cerebellar and spinal...
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عنوان ژورنال:
- Diagnostic and interventional radiology
دوره 12 1 شماره
صفحات -
تاریخ انتشار 2006