Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients.

Mukherji, Suresh K.; Albernaz, Vanessa S.; Lo, William W. M.; Gaffey, M. J.; Megerian, Cliff A.; Feghali, Joseph G.; Brook, Allan; Lewin, Jonathan S.; Lanzieri, Charles F.; Talbot, J. Michael; Meyer, Jacob; Carmody, Raymond F.; Weissman, Jane L.; Smirniotopoulos, James G.; Rao, Vijay M.; Jinkins, J. R.; Castillo, Maurício

doi:10.1148/radiology.202.3.9051037

Cited by 123 publications

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“…Additionally, at MR imaging, endolymphatic sac tumors are heterogeneous, demonstrating areas of T1 and T2 high signal intensities corresponding to cysts filled with blood or high proteinaceous fluid and multiple small signal intensity voids due to calcifications and blood vessels. 9 Other considerations for enhancing petrous bone lesions include meningioma, eosinophilic granuloma, glomus tumor, cartilaginous tumors, chordoma, and metastasis, but these are usually larger when symptomatic. 10 The size of the lesion is an important criterion because it also helps in distinguishing OO from osteoblastoma, another osteoid-producing tumor.…”

Section: Discussionmentioning

confidence: 99%

Osteoid Osteoma Invading the Posterior Labyrinth of the Petrous Bone

Dugert¹,

Lagleyre²,

Brouchet³

et al. 2010

AJNR Am J Neuroradiol

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SUMMARY:We report a painless osteoid osteoma of the inner ear in an elderly woman presenting with hearing loss, tinnitus, and vertigo. CT showed a heterogeneous mass containing calcifications involving the posterior labyrinth, which heterogeneously enhanced on postcontrast MR images. Osteoid osteoma should be included in the differential diagnosis of an osteolytic petrous bone lesion with central calcification and enhancement.ABBREVIATIONS: OO ϭ osteoid osteoma O steoid osteoma is a benign osteoblastic tumor, most frequently diagnosed in young men presenting with characteristic pain at the site of the lesion, increasing at night and relieved by salicylates. On CT, the lesion is characterized by a zone of bony sclerosis surrounding a central radiolucent area. 1 OO usually involves long bones and the vertebral column and extremities and is very uncommon in the skull.2 Temporal bone OO is even rarer, with only 4 cases described in the literature, none of which were located in the inner ear.3-5 We report here the first case of a pathologically proved OO invading the posterior labyrinth and review the relevant literature. Case ReportA 77-year-old healthy woman presented with a several-months' history of right hearing loss, vertigo, and tinnitus. There was no associated pain. Physical examination confirmed hearing loss and an estimated vestibular deficiency of 70% on the right side. CT showed an osteolytic, heterogeneous, rounded, well-demarcated petrous lesion, measuring 11 ϫ 6 mm, with central calcifications (Fig 1A). Although such calcifications have been interpreted as newly produced osteoid trabeculae, remnants of destroyed bone might have been possible as well. The lesion spared the cochlea but invaded the posterior labyrinth and the endolymphatic duct. There was a close relationship with the facial nerve (Fig 1B). No sclerotic bone was seen surrounding the lesion. At MR imaging, the lesion demonstrated intermediate signal intensity on T1 and high signal intensity on T2-weighted images and enhanced intensely and heterogeneously after intravenous gadolinium administration (Fig 2). Technetium Tc99m scintigraphy showed a corresponding increased uptake in the petrous bone. The preoperative diagnosis was hemangioma.A surgical approach through a mastoidectomy revealed a gray lesion. The center of the lesion was soft and hemorrhagic, but its periphery was hard and indistinguishable from the surrounding normal bone. Because of the anatomic contact with the tympanic segment of the facial nerve, the lesion was only partially excised. Definitive pathologic examination revealed a nidus formed by a hypervascular young conjunctival stroma with osteoblasts, multinucleated giant cells, and thin osteoid trabeculae, consistent with osteoid osteoma (Fig 3). The postoperative course was uneventful, with normal facial nerve function. At 6-month follow-up, the vertigo had disappeared but the tinnitus remained unchanged. DiscussionOO is a frequent, benign, primary bone tumor accounting for approximately 10% of all primary bone tumors....

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Section: Discussionmentioning

confidence: 99%

Osteoid Osteoma Invading the Posterior Labyrinth of the Petrous Bone

Dugert¹,

Lagleyre²,

Brouchet³

et al. 2010

AJNR Am J Neuroradiol

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“…In addition to spontaneous otorrhea, these lesions may also cause meningeal tumors [7] . The defects formed by arachnoid granulations are usually lobulated with irregular borders, and the radiologic appearance of the defect is almost always sufficient to diagnose arachnoid granulations [8] . In our study, the radiologic appearances of all defects were compatible with arachnoid granulations.…”

Section: Discussionmentioning

confidence: 99%

Temporal Bone Posterior Wall Defect, a New Clinic Entity?

Ezerarslan

Ataç²,

Keskin³

et al. 2015

Int Adv Otol

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OBJECTIVE:To define clinical and audiological findings in patients with temporal bone posterior wall defects (TBPWD) and to investigate possible relationships between these findings and the characteristics of the defect. MATERIALS and METHODS:The computed tomography (CT) views of 1198 patients with vestibulocochlear symptoms between 2007 and 2012 were retrospectively evaluated, and TBPWD and associated anomalies were investigated. Patients who had TBPWD were called back, and clinical and audiological examinations (tympanometry, pure tone audiometry, acoustic reflexes, and otoacoustic emission) were performed. RESULTS:Twenty-eight (2.34%) patients had TBPWD. Twenty-three of them were eligible for the study. Size of the defect was significantly correlated with the presence of tinnitus and/or vertigo (p<0.005). The cut-off values for the largest size of TBPWD were 1.65 mm [sensitivity: 0.67 and specificity: 0.77 (95% CI: 0.58-0.97); p=0.04] in case of the presence of tinnitus and 1.85 for vertigo (sensitivity: 0.78 and specificity: 0.86 (95% CI: 0.67-0.99); p=0.006). In pure tone audiometry tests, mixed-type hearing loss was present in four (17%) patients, sensorineural hearing loss was present in three (13%) patients, and conductive-type hearing loss was present only in one (4%) patient. Otoacoustic emission tests revealed significant differences in signal/noise ratios at frequencies of 500, 750, 1000, and 6000 Hz. CONCLUSION:For the first time in the literature, we defined clinical and audiological findings in patients with TBPWDs. These defects seem to cause more prevalent symptoms of vertigo and tinnitus and disturb the audiological characteristics of patients.

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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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Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients.

Cited by 123 publications

References 0 publications

Osteoid Osteoma Invading the Posterior Labyrinth of the Petrous Bone

Osteoid Osteoma Invading the Posterior Labyrinth of the Petrous Bone

Temporal Bone Posterior Wall Defect, a New Clinic Entity?

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

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