The Surgical Anatomy of the Endolymphatic Sac

Ik, Arenberg; Rask‐Andersen, Helge; Wilbrand, Hermann; Stahle, Jan

doi:10.1001/archotol.1977.00780180039001

Cited by 85 publications

(13 citation statements)

References 15 publications

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“…The endolymphatic sac is variable in size and irregular in outline. In general, the intraosseous part of the endolymphatic duct/sac system is referred to as the vestibular aqueduct (31).…”

Section: Discussionmentioning

confidence: 99%

Effects of VHL Deficiency on Endolymphatic Duct and Sac

et al. 2005

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The von Hippel-Lindau (VHL) disease is caused by VHL germ line mutation. Inactivation of the wild-type copy of the VHL gene leads to up-regulation of hypoxic response and tumor formation within central nervous system (CNS), kidneys, pancreas, adrenal glands, epididymis, broad ligament, and the endolymphatic sac/petrous bone. Endolymphatic sac tumors (ELST) have been proposed to be derived from endolymphatic sac epithelium, but other possible structures of origin have been implicated. To clarify the anatomic and cellular origin of ELSTs, we did a morphologic and molecular pathologic analysis of 16 tumors. In addition, we investigated effects of VHL deficiency on ''tumor-free'' endolymphatic duct and sac of VHL patients. Several tumors included in this study were <1 cm in size, and their origin could be placed in the intraosseous portion of the endolymphatic duct/sac. Furthermore, by analysis of clinically uninvolved ''tumor-free'' endolymphatic duct and sac tissues of VHL patients, we discovered a variety of VHL-deficient microscopic abnormalities with morphologic similarities to ELSTs. We conclude that most, if not all, ELSTs arise within the intraosseous portion of the endolymphatic duct/sac, the vestibular aqueduct. In analogy to renal parenchyma and selected topographical sites within the CNS, endolymphatic duct/sac epithelia are preferentially and multifocally targeted in VHL disease. The primary effect of VHL deficiency on human endolymphatic duct/sac epithelium seems to be the generation of multifocal sites of VHL-deficient cell proliferations from which tumorigenesis may or may not occur. Therefore, inactivation of the VHL wild-type allele seems necessary but not sufficient for the formation of tumor. (Cancer Res 2005; 65(23): 10847-53)

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“…The endolymphatic sac is variable in size and irregular in outline. In general, the intraosseous part of the endolymphatic duct/sac system is referred to as the vestibular aqueduct (31).…”

Section: Discussionmentioning

confidence: 99%

Effects of VHL Deficiency on Endolymphatic Duct and Sac

et al. 2005

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“…In der Literatur wird vielfach eine gedachte Linie entlang des lateralen Bogengangs als wichtige Landmarke genannt [33,34] , an die angrenzend der Saccus lokalisiert sei. Arenberg et al [35] konnten in einer anatomischen Studie an Pr ä paraten von Gesunden und Patienten mit M. M é ni è re jedoch zeigen, das diese Linie insbesondere bei Patienten mit l ä ngerem Krankheitsverlauf von geringem Wert ist, da sich in dieser Population h ä ufi g Lagevarianten zeigten. In der Patienten-Gruppe lag der Saccus endolymphaticus h ä ufi g inferior der genannten Linie und erreichte diese nicht.…”

Section: Materials Und Methodenunclassified

Die Saccotomie in der Behandlung des M. Ménière: Beurteilung der kurz-, mittel- und langfristigen Ergebnisse anhand eines Patientenfragebogens

Radeloff¹,

Hamad²,

Baier³

et al. 2009

Laryngo-Rhino-Otol

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“…The interior of this system is lined by single-layered cuboidal endolymphatic duct/sac epithelium (62). The intraossesous part of the endolymphatic duct/sac system is also referred to as the vestibular aqueduct (63) and represents the site of origin of ELST (64). The endolymphatic duct/sac system is part of the nonsensory membranous labyrinth of the inner ear with a potential functional role in maintenance of homeostasis and pressure of the inner ear, phagocytosis of debris and immunologic functions (65–68).…”

Section: Endolymphatic Sac Tumorsmentioning

confidence: 99%

Pathology of the Nervous System in Von Hippel-Lindau Disease

Vortmeyer

Alomari

2015

jkcvhl

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Von Hippel-Lindau (VHL) disease is a tumor syndrome that frequently involves the central nervous system (CNS). It is caused by germline mutation of the VHL gene. Subsequent VHL inactivation in selected cells is followed by numerous well-characterized molecular consequences, in particular, activation and stabilization of hypoxia-inducible factors HIF1 and HIF2. The link between VHL gene inactivation and tumorigenesis remains poorly understood. Hemangioblastomas are the most common manifestation in the CNS; however, CNS invasion by VHL disease-associated endolymphatic sac tumors or metastatic renal cancer also occur, and their differentiation from primary hemangioblastoma may be challenging. Finally, in this review, we present recent morphologic insights on the developmental concept of VHL tumorigenesis which is best explained by pathologic persistence of temporary embryonic progenitor cells.

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The Surgical Anatomy of the Endolymphatic Sac

Cited by 85 publications

References 15 publications

Effects of VHL Deficiency on Endolymphatic Duct and Sac

Effects of VHL Deficiency on Endolymphatic Duct and Sac

Die Saccotomie in der Behandlung des M. Ménière: Beurteilung der kurz-, mittel- und langfristigen Ergebnisse anhand eines Patientenfragebogens

Pathology of the Nervous System in Von Hippel-Lindau Disease

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