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Endolymphatic Sac Tumors
Built from Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
What’s inside
12 sections · 90 slides
Overview
- Scope of this presentation
Definition and historical background
- Definition and site of origin
- Evolution of nomenclature
- Early descriptions of adenomatous temporal bone tumours
- Megerian 1995 temporal bone series
- Significance of the temporal bone findings
Clinical presentation
- Epidemiology of sporadic tumours
- Cochleovestibular symptoms at presentation
- Mechanisms of hearing loss
- Mimicry of Meniere disease
- Cranial neuropathies and diagnostic delay
- Routes of local extension
- Consequences of advanced local extension
- Drop metastasis and cerebrospinal fluid cytology
Association with von Hippel-Lindau disease
- Von Hippel-Lindau disease overview
- von Hippel-Lindau-related tumours
- VHL-associated tumours on contrast MRI
- VHL-associated ELST phenotype
- VHL gene and protein
- pVHL, hypoxia inducible factor and tumorigenesis
Histology and immunohistochemistry
- Gross and microscopic features
- Papillary and follicular patterns compared
- Resected sac tumour and its papillary histology
- Histological differential diagnosis
- Immunohistochemical profile of ELST
- Endolymphatic sac tumor differential diagnosis and immunohistochemical stains
Imaging
- Computed tomography features
- Posterior fossa plate erosion on computed tomography
- Magnetic resonance imaging features
- Intralabyrinthine haemorrhage on MRI
- Heterogeneous mastoid enhancement on contrast MRI
- Somatostatin receptor imaging
- Blood supply and preoperative embolization
- Radiological differential diagnosis
Staging and surveillance
- Bambakidis and Megerian grading system
- Grading and treatment system for endolymphatic sac tumors
- Grade at diagnosis in VHL versus sporadic disease
- Surveillance practice in von Hippel-Lindau disease
- Subclinical disease in von Hippel-Lindau disease
Surgical management
- Principles of surgical management
- Retrolabyrinthine-transdural approach: positioning and exposure
- Retrolabyrinthine-transdural approach: resection
- Retrolabyrinthine-transdural approach: reconstruction
- Retrolabyrinthine-transdural approach for hearing preservation
- Operative sequence of hearing preservation resection
- Limitations of more conservative approaches
- Approach selection by hearing and tumour extent
- Cochlear implantation in endolymphatic sac tumour
Outcomes of microsurgery
- Limitations of the outcome evidence
- House Ear Clinic surgical series
- Functional outcomes in the House Ear Clinic series
- Complications of surgery
- Pooled microsurgical tumour control
- Microsurgical results for endolymphatic sac tumors
- Open question in hearing preservation
Radiation and systemic therapy
- Overall radiotherapy tumour control
- External beam radiation versus stereotactic radiation
- Summary of external beam versus stereotactic radiosurgery outcomes
- Indications for radiation therapy
- Patterns of recurrence and adjuvant strategy
- Chemotherapy and future systemic options
Multidisciplinary care in von Hippel-Lindau disease
- Multidisciplinary management of VHL
- Craniospinal haemangioblastoma in von Hippel-Lindau disease
- Prognostic value of the peritumoral cyst
- Hearing rehabilitation in bilateral disease
Summary
- Key clinical messages
- Key management messages
- References
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- References (continued)
- Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
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