Endolymphatic sac carcinoma of the right petrous bone in Von Hippel–Lindau disease

Muzumdar, Dattatraya; Goel, Atul; Fattepurkar, S.; Goel, Naina

doi:10.1016/j.jocn.2005.04.030

Cited by 8 publications

(3 citation statements)

References 18 publications

(16 reference statements)

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“…These tumours may grow large enough to extend into the CPA and eventually compress the brain stem [45]. Endolymphatic sac tumours occur sporadically or in the context of von Hipple-Lindau disease [46,47]. Endolymphatic sac tumour is an extradural tumour that erodes and destroys the retrolabyrinthine petrous bone with geographic or moth-eaten margins at CT, and may exhibit possible calcifications [48].…”

Section: Endolymphatic Sac Tumoursmentioning

confidence: 99%

Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

Savatovsky²,

2007

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Computed tomography (CT) and magnetic resonance (MR) imaging reliably demonstrate typical features of vestibular schwannomas or meningiomas in the vast majority of mass lesions responsible for cerebellopontine angle (CPA) syndrome. However, a large variety of unusual lesions can also be encountered in the CPA. Covering the entire spectrum of lesions potentially found in the CPA, these articles explain the pertinent neuroimaging features that radiologists need to know to make clinically relevant diagnoses in these cases, including data from diffusion- and perfusion-weighted imaging or MR spectroscopy, when available. A diagnostic algorithm based on the lesion's site of origin, shape and margins, density, signal intensity and contrast material uptake is also proposed. Non-enhancing extra-axial CPA masses are cystic (epidermoid cyst, arachnoid cyst, neurenteric cyst) or contain fat (dermoid cyst, lipoma). Tumours can also extend into the CPA by extension from the skull base (paraganglioma, chondromatous tumours, chordoma, cholesterol granuloma, endolymphatic sac tumour). Finally, brain stem or ventricular tumours can present with a significant exophytic component in the CPA that may be difficult to differentiate from an extra-axial lesion (lymphoma, hemangioblastoma, choroid plexus papilloma, ependymoma, glioma, medulloblastoma, dysembryoplastic neuroepithelial tumour).

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Section: Endolymphatic Sac Tumoursmentioning

confidence: 99%

Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

Savatovsky²,

2007

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“…It is more frequently bilateral and advanced as opposed to the non-VHL disease patient. [ 5 ] Patients characteristically present with unilateral sensorineural hearing loss, tinnitus, otalgia, otorrhea, vertigo, ataxia, and facial nerve paresis. Large tumors growing along the posteromedial vector may cause symptoms secondary to cerebellopontine angle invasion.…”

Section: Discussionmentioning

confidence: 99%

Endolymphatic sac tumor in von Hippel–Lindau disease: A rare case report

et al. 2017

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Endolymphatic sac tumor (ELST) is a rare papillary neoplasm with locally destructive behavior which can occur sporadically or in association with Von Hippel–Lindau (VHL) disease. We herein present a case of ELST associated with VHL disease in a 14-year-old girl and discuss clinico-radiological, immunohistopathologic findings, and management by staged surgery and postoperative radiotherapy to the residual lesion.

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“…Endolymphatic sac tumors associated with a diagnosis of VHL disease appear to affect a younger population of patients than non-VHL disease cases and occur in women twice as often as in men when associated with VHL disease. In addition, tumors are more frequently bilateral and advanced in the VHL disease patient as opposed to the non-VHL disease patient [3,9]. Historically, nomenclature of invasive adenoid tumours in the petrous bone has been divergent, the term papillary adenocarcinoma used most frequently.…”

Section: Discussionmentioning

confidence: 99%

Endolymphatic Sac Tumour: A Neoplastic Cause for Meniere’s Syndrome

Raghunandhan

Krishnan

Murali

et al. 2011

Indian J Otolaryngol Head Neck Surg

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Patients with intractable vertigo often present a diagnostic dilemma to the treating physician. A wide spectrum of diseases, ranging from those of the labyrinth onto the central nervous system, may present predominantly with vertigo. In some cases, it requires the clinical acumen of an experienced neuro-otologist, to decipher these vertiginous symptoms and arrive at a definitive diagnosis. Meniere's syndrome is one such phenomenon, where the endolymphatic hydrops may be attributable to varied aetiology. We report a case of sporadic (non-syndromic) Endolymphatic Sac Tumor which presented to us, mimicking a classical Meniere's syndrome. We discuss its clinical presentation, diagnostic modalities, operative technique and histo-pathological features. The case is reported along with a review of the world literature on this tumor, highlighting the diagnostic and management protocols advocated for this rare entity. Endolymphatic sac tumor is a rare entity which may masquerade as a classical case of Meniere's syndrome, wherein the triad of symptoms (as in endolymphatic hydrops) may not be alleviated by the usual treatment protocols. Tracing the aetiology of these symptoms, remains the most crucial factor in treating such patients.

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Endolymphatic sac carcinoma of the right petrous bone in Von Hippel–Lindau disease

Cited by 8 publications

References 18 publications

Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

Endolymphatic sac tumor in von Hippel–Lindau disease: A rare case report

Endolymphatic Sac Tumour: A Neoplastic Cause for Meniere’s Syndrome

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