Endolymphatic sac tumor with overexpression of V2 receptor mRNA and inner ear hydrops

Kitahara, Tadashi; Maekawa, Chie; Kizawa, Kaoru; Kamakura, Takefumi; Horii, Arata; Inohara, Hidenori

doi:10.3109/00016489.2011.580004

Cited by 11 publications

(4 citation statements)

References 17 publications

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“…When the tumour is bilateral – a situation seen in 30% of syndromic patients 3 and, to the best of our knowledge, never reported in sporadic cases – there is the problem of bilateral deafness as the natural outcome of the evolution of the disease. The mechanisms behind it are direct invasion of the inner ear, endolymphatic hydrops that mimic Meniere's disease 31 , and intralabyrinthine haemorrhage (even in small tumours), which is responsible for sudden sensorineural hearing loss 32 .…”

Section: Discussionmentioning

confidence: 99%

Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies

Zanoletti

Girasoli

Borsetto

et al. 2017

Acta Otorhinolaryngol Ital

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Il carcinoma del sacco endolinfatico è un tumore molto raro come evidente dai dati presenti in letteratura ove anche centri di esperienza riferiscono tuttavia casistiche limitate. E un tumore maligno a lenta crescita, con tendenza allinvasione locale e scarsa tendenza alla disseminazione metastatica. Linsorgenza spesso tardiva dei sintomi e la difficoltà ad eseguire indagini bioptiche a livello della sede di origine, parete posteriore della rocca, ha reso la diagnosi di questo tumore spesso difficile, nonostante si riconoscano attualmente dati radiologici patognomonici della neoplasia. Patterns tipici di presentazione sono evidenziabili alla RM con mezzo di contrasto e alla TC per osso a strato sottile, rendendo nella maggior parte dei casi possibile la diagnosi radiologica. Lincidenza del tumore del sacco endolinfatico è maggiore nei pazienti affetti da sindrome di von Hippel Lindau (VHL), con una frequenza del 25% che fa parte del quadro sindromico. Negli anni dal 2012 al 2015 abbiamo osservato 7 casi, uno di essi con manifestazione della patologia bilaterale, tutti affetti da VHL. Quattro tra questi sono stati sottoposti a chirurgia presso il nostro centro per un totale di 5 procedure chirurgiche. Ogni caso è stato descritto dettagliatamente analizzando sintomi, intervallo tra comparsa dei sintomi, diagnosi e terapia. Non vi è stata morbidità post-operatoria aggiuntiva nei pazienti in cui la diagnosi e la terapia sono state precoci, mentre la gestione di tumori localmente avanzati è stata associata a deficit neurologici postoperatori, in particolare del VII, IX e X nervo cranico. I siti anatomici critici di coinvolgimento della malattia che hanno coinciso con un pianificato rischio di danno neuronale sono risultati essere il canale di Falloppio, il forame giugulare, lapice della rocca petrosa. Lestensione intradurale nella fossa cranica posteriore è stato un altro elemento caratterizzante i tumori in stadio avanzato. I tumori del sacco endolinfatico che lo screening permette di evidenziare precocemente nei pazienti VHL, hanno buona prognosi quando affrontati precocemente, compatibilmente con le esigenze terapeutiche della malattia di base.

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Section: Discussionmentioning

confidence: 99%

Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies

Zanoletti

Girasoli

Borsetto

et al. 2017

Acta Otorhinolaryngol Ital

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“…Accordingly, a – most likely secondary – endolymphatic hydrops has been visualized on inner ear MRI of ELST patients 278 . An upregulation of type 2 vasopressin receptors in the endolymphatic sac is discussed as a possible underlying patholophysiology of secondary endolymphatic hydrops in ELST in addition to a mechanical blockage of the endolymphatic drainage 279 .…”

Section: Episodic Vestibular Syndromesmentioning

confidence: 99%

“…sekundärer – Endolymphhydrops im Innenohr-MRT nachgewiesen 278 . Als Ursache für den Hydrops wird neben einer mechanischen Blockade des Endolymphabflusses auch eine Hochregulation von Typ2-Vasopressin-Rezeptoren im endolymphatischen Sack diskutiert 279 .…”

Section: 421 Klinische Präsentation Und Bildgebungunclassified

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

Dlugaiczyk

2021

Laryngorhinootologie

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ZusammenfassungDie Differenzialdiagnose von Erkrankungen des vestibulären Labyrinths stellt eine grosse Herausforderung dar, da sich hinter dem sehr häufigen Leitsymptom «Schwindel» viele verschiedene und v. a. seltene Erkrankungen verbergen können. Der vorliegende Beitrag gibt einen Überblick über die für den HNO-Arzt wichtigen seltenen Erkrankungen des vestibulären Labyrinths ausgehend von ihrer klinischen Präsentation als akutes (AVS), episodisches (EVS) oder chronisches vestibuläres Syndrom (CVS). Der Schwerpunkt liegt dabei auf den EVS, sortiert nach ihrer Dauer und dem Vorhandensein von Triggern (Sekunden, ohne Trigger: Vestibularisparoxysmie, Tumarkin-Krise; Sekunden, lärm- und druckinduziert: Syndrome des «dritten Fensters»; Sekunden bis Minuten, positionsabhängig: seltene Varianten und Differenzialdiagnosen des benignen paroxysmalen Lagerungsschwindels; Stunden bis Tage, spontan: intralabyrinthäre Schwannome, Tumoren des endolymphatischen Sacks, Autoimmunerkrankungen des Innenohres). Des Weiteren werden seltene Differenzialdiagnosen eines AVS (Neuritis vestibularis inf., Otolithenfunktionsstörungen, vaskuläre Ursachen, akute bilaterale Vestibulopathie) und eines CVS (bilaterale Vestibulopathie) erläutert. Dabei werden insbesondere die entscheidenden diagnostischen Massnahmen für die Identifikation der einzelnen Krankheitsbilder und die Warnzeichen für potentiell gefährliche Ursachen (z. B. Labyrinthinfarkt/-blutung) dargelegt. Somit dient dieser Beitrag dem HNO-Arzt in Klinik und Praxis als eine Art «Vademecum» für die zügige Identifikation und zeitnahe Therapie seltener Erkrankungen des Gleichgewichtsorgans.

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“…4 -7 Although the IT administration of GBCM is an off-label use and requires puncture of the tympanic membrane, various institutions have employed this method. [8][9][10][11][12][13][14] On the other hand, recent studies of the relationship between the degree of endolymphatic hydrops and patient symptoms have used the IV administration of single-dose GBCM (IV-SD-GBCM). [15][16][17] A previous study in volunteers set the time delay after IV-SD-GBCM at around 4 hours, 18 and a significant rise in perilymph signal beginning 1.5 hours after IV-SD-GBCM in volunteers has been reported.…”

Section: Introductionmentioning

confidence: 99%

Time Course for Measuring Endolymphatic Size in Healthy Volunteers Following Intravenous Administration of Gadoteridol

et al. 2014

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Purpose: We developed semi-quantitative methods to measure endolymphatic size on images obtained 4 hours after intravenous administration of single-dose gadolinium-based contrast medium (IV-SD-GBCM) and found little variation in results between observers. We used the methods to measure the size of the endolymph in healthy volunteers at various times after IV-SD-GBCM and attempted to determine the optimal timing for the evaluation.Methods: In 8 healthy male volunteers, we obtained heavily T 2 -weighted 3-dimensional fluid-attenuated inversion recovery (hT 2 W-3D-FLAIR) images 1.5, 3, 4.5, and 6 hours after IV-SD-GBCM as positive perilymph images (PPI) as well as acquiring positive endolymph images (PEI) and magnetic resonance cisternography (MRC). To evaluate the endolymph, we generated 2 kinds of processed images (HYDROPS-Mi2 and HY-DROPS2-Mi2) by subtracting PEI or MRC from PPI as previously proposed. We semiquantitatively measured the ratio of the area of the endolymph (%EL) to that of total lymph on the 2 kinds of generated images for the cochlea and vestibule according to the previously proposed method. We analyzed statistics to evaluate the change in %EL over time and used analysis of variance (ANOVA) for a 2 © 4 repeated-measures design to assess difference in image type. We adopted 5% as a significance level.Results: The %EL was significantly larger at 1.5 hours after IV-SD-GBCM than at 3, 4.5, and 6 hours in both the cochlea and vestibule for both kinds of generated images. Between 4.5 and 6 hours, the %EL plateaued for both the cochlea and vestibule, and the 2 kinds of generated images did not differ significantly.Conclusion: A delay of 1.5 hours after IV-SD-GBCM is not sufficient to evaluate endolymphatic size. The %EL plateaus between 4.5 and 6 hours. These data might be valuable for further clinical studies.

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Endolymphatic sac tumor with overexpression of V2 receptor mRNA and inner ear hydrops

Cited by 11 publications

References 17 publications

Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies

Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

Time Course for Measuring Endolymphatic Size in Healthy Volunteers Following Intravenous Administration of Gadoteridol

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