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− Aggressive papillary tumor of endolymphatic sac. Six years after surgical resection, CT scans revealed tumor recurrence CT: computed tomography.

− Aggressive papillary tumor of endolymphatic sac. Six years after surgical resection, CT scans revealed tumor recurrence CT: computed tomography.

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FigurE 1 − Aggressive papillary tumor of endolymphatic sac: CT showing...
FigurE 2 − Aggressive papillary tumor of endolymphatic sac. A) a...
FigurE 3 − Aggressive papillary tumor of endolymphatic sac. Six years...
Aggressive papillary tumor of endolymphatic sac: Case report of a rare neoplasia
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  • Feb 2016
Aggressive papillary endolymphatic sac tumor (ELST) is a rare neoplasm, occasionally related to von Hippel-Lindau disease, characterized by locally aggressive growth with temporal bone destruction. The authors report a case of ELST in a female patient exhibiting fifth through eighth cranial nerve paralysis. Computed tomography (CT) revealed a large...
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... diagnosis of ELST was then established. Six years after initial therapy, CT identified a large mass on the right temporal bone, compatible with lesion recurrence ( Figure 3). ...

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... 3 The prevalence of VHL is 1 case per 39,000 population of whom upto 20% develop ELST, of which 30% cases are bilateral. 4 A wide age range of presentation has been described with mean age of 52.5 years in patients with sporadic ELST and 31.3 years in patients associated with VHL syndrome. 2 VHL syndrome is an autosomal dominant disorder in which affected individuals develop cerebellar and retinal hemangioblastomas, cysts involving pancreas, liver and kidneys, renal cell carcinoma, adrenal phaeochromocytoma and papillary cystadenomas of the epididymis. ...
... Affected individuals may also develop endolymphatic sac of the inner ear, hepatocellular adenoma, paragangliomas and visceral angiomas. 4 Patients characteristically present with progressive, ipsilateral sensorineural hearing loss, associated with tinnitus, vertigo, ataxia, otorrhea and facial nerve paresis. A long-standing history is typical. ...
... These can be eliminated by radiological, histopathological and immunohistochemical correlations. 4 The above patient presented with complaints of sensorineural hearing loss. Due to high association of tumor with VHL syndrome, all patients with ELST should be screened for VHL syndrome and vice versa. ...
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