Endolymphatic sac tumors: radiologic appearance.

Lo, William W. M.; Applegate, L J; Carberry, Joseph N.; Solti-Bohman, L.G.; House, John W.; Brackmann, Derald E.; Waluch, V.; Li, J. C.

doi:10.1148/radiology.189.1.8372194

Cited by 94 publications

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“…In one study, retrolabyrinthine bone destruction was centered at the external aperture of the vestibular aqueduct in all four patients with ELSTs who underwent computed tomography (CT) and magnetic resonance (MR). These radiological changes may help distinguish ELSTs from other tumors of the temporal bone and posterior fossa [9]. In our observation, the tumor in the saccule looked like a papillary tumor.…”

Section: Discussionsupporting

confidence: 53%

Small papillary tumor in the saccule

2007

International Journal of Pediatric Otorhinolaryngology

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SummaryPapillary tumors of the ear are aggressive neoplasms. Previously, a tumor growing in the saccule had not been reported. We report a tumor found incidentally in the saccule of a patient. Serial sections of both temporal bones of the patient were studied in order to analyze the tumor's origin and influence on audio vestibular function. In the right inner ear, there was a small papillary lesion in the saccule, which looked like a papillary tumor without aggression invasion. The tumor was located in the membranous labyrinth of the saccule, not in the endolymphatic duct and sac. It was not related to Von Hippel-Lindau (VHL) disease, nor was it an endolymphatic sac tumor. The tumor did not influence the hearing and vestibular function in the right ear, although this patient presented a severe sensorineural hearing loss and vestibular function loss because of the vestibular schwannoma in the left ear.

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Section: Discussionsupporting

confidence: 53%

Small papillary tumor in the saccule

2007

International Journal of Pediatric Otorhinolaryngology

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“…3). 5 The endolymphatic duct is connected to the membranous labyrinth of the inner ear by the saccular and utricular ducts. These ducts are directly connected to the saccule and utricule, respectively, by bidirectional valves.…”

Section: Discussion Endolymphatic Sac and Duct Anatomymentioning

confidence: 99%

The vestibular aqueduct: site of origin of endolymphatic sac tumors

Lonser

Baggenstos

Kim

et al. 2008

JNS

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Object-Although endolymphatic sac tumors (ELSTs) frequently destroy the posterior petrous bone and cause hearing loss, the anatomical origin of these neoplasms is unknown. To determine the precise topographic origin of ELSTs, the authors analyzed the imaging, operative, and pathological findings in patients with von Hippel-Lindau disease (VHL) and ELSTs.Methods-Consecutive VHL patients with small (≤ 1.5 cm) ELSTs who underwent resection at the National Institutes of Health were included. Clinical, imaging, operative, and pathological findings were analyzed.Results-Ten consecutive VHL patients (6 male and 4 female) with 10 small ELSTs (≤ 1.5 cm; 9 left, 1 right) were included. Serial imaging captured the development of 6 ELSTs and revealed that they originated within the intraosseous (vestibular aqueduct) portion of the endolymphatic duct/sac system. Imaging just before surgery demonstrated that the epicenters of 9 ELSTs (1 ELST was not visible on preoperative imaging) were in the vestibular aqueduct. Inspection during surgery established that all 10 ELSTs were limited to the intraosseous endolymphatic duct/sac and the immediately surrounding region. Histological analysis confirmed tumor within the intraosseous portion (vestibular aqueduct) of the endolymphatic duct/sac in all 10 patients.Conclusions-ELSTs originate from endolymphatic epithelium within the vestibular aqueduct. High-resolution imaging through the region of the vestibular aqueduct is essential for diagnosis. Surgical exploration of the endolymphatic duct and sac is required for complete resection. Keywordsanatomy; endolymphatic sac tumor; origin; pathology; vestibular aqueduct Endolymphatic sac tumors were first established as a pathological entity by Heffner in 1989 3 after he described the unique anatomical and histological features in 20 benign papillary-cystic neoplasms in the region of the posterior temporal bone. Based on anatomical estimations derived from the approximate epicenter of large tumors, Heffner concluded that the "probable"

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“…Our patient (Case 2) had minimum enhancement and high T1 WI problably related to blood products. CT evaluation is always necessary because of its suberb capability in bone evaluation and play an adjunct role on inner and middle ear evaluation 2,4,9,[16][17][18][19][20] .…”

Section: Discussionmentioning

confidence: 99%

Endolymphatic sac tumor and von Hippel-Lindau disease in a single family

Safatle

Farage

Sampaio

et al. 2009

Arq. Neuro-Psiquiatr.

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Endolymphatic sac tumors are low-grade adenocarcinoma of temporal bone, locally invasive 1-3 . Their origin was controversial but showed to originate from endolymphatic sac and duct epithelium 2,4,5 . They are uncommon tumors and occur sporadically, or more frequently, associated with von Hippel-Lindau (VHL) 3,6-8 disease. The most common clinical presentations are: progressive deafness, tinnitus and vertigo. The hearing loss is typically irreversible, acute, progressive or gradual 6,9 . We illustrate a familial appearence of endolymphatic sac tumor. During the evaluation of other family members the diagnosis of von Hippel-Lindau disease came up. VHL is a genetic affection, autosomic dominant, with almost complete penetration (95%). The syndrome includes renal cysts and renal carcinoma, pancreatic cysts, neuroendocrine tumors, pheochromocytoma, cystadenomas of the reproductive adnexal organs, and hemangioblastomas of the nervous central system and retina 8,10 .We describe two cases of endolymphatic sac tumors in two siblings from different fathers and with a VHL affected mother. casEs Case 1A male patient presented hearing reduction in the left ear since he was 17 years old. At that time, he was evaluated by computed tomography (CT) of the ear that showed temporal bone erosion. He was submitted to surgical resection with histological confirmation of adenocarcinoma of endolymphatic sac. He was treated at an outside institution and we don't have any further information. Case 2A female patient 15 years old, five years after the diagnosis of her brother (Case 1) presented a progressive left side hearing loss associated with vertigo during the last 6 months. Audiometric evaluation showed a severe sensorineural hearing loss (high frequency). Temporal bone destruction envolving the endolymphatic sac and posterior semicircular canal was seen at CT at left side (solid arrow), compared to the normal contralateral structures (open arrow) (Fig 1A and B). At magnetic resonance imaging (MRI) a minimally enhanced mass between the sigmoid sinus and the internal auditory canal, measuring 2.5 cm (Fig 2A and B) and destroying the posterior semicircular canal was found (Fig 2C).She underwent surgical resection by a retrolabyrinthinetransdural approach. Using an extended postauricular incision,

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Endolymphatic sac tumors: radiologic appearance.

Abstract: These radiologic changes may help distinguish ELSTs from other tumors of the temporal bone and posterior fossa.

Cited by 94 publications

References 0 publications

Small papillary tumor in the saccule

Small papillary tumor in the saccule

The vestibular aqueduct: site of origin of endolymphatic sac tumors

Endolymphatic sac tumor and von Hippel-Lindau disease in a single family

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