Abstract Details

Title Octreotide Positive Carotid Paragangliomas Causing Progressive Cranial Neuropathies

Topic Neuro-oncology

Presentation(s) P06 - (-)

Poster/Presentation
Number 145

Objective

Background BACKGROUND: A 58-year old man presented with a 5-year history of progressive bulbar difficulties. Prior to neurological review he was diagnosed with obstructive sleep apnoea syndrome and commenced on nocturnal non-invasive ventilation. Examination revealed dysarthria, dysphagia and left hypoglossal and glossopharyngeal nerve palsies. He subsequently developed bilateral Horner's syndrome.

Design/Methods DESIGN/METHODS: Investigations including vasculitic screen, serum ACE and anti-acetycholinesterase antibodies were negative. An MRI brain scan with contrast, CSF and EMG were unremarkable. Whole body CT PET scan demonstrated increased uptake in the parapharyngeal regions of the neck bilaterally initally suggestive of lymphadenopathy so a biopsy was arranged.

Results RESULTS: Histology revealed highly vascular tissue staining positive for neuroendocrine markers synaptyphysin, chromogranin and octreotide consistent with carotid body paraganglioma. MR angiogram showed bilateral large vascular neck masses resulting in splaying of the internal and external carotid artery at the bifurcation referred to as the Lyre sign.The extensive vascularity and anatomical location of the tumours prohibited complete surgical resection and so radiotherapy was preformed. The patient is now undergoing evaluation for treatment with radiolabelled octreotide.

Conclusions CONCLUSIONS: Carotid paragangliomas are rare neuroendocrine tumours. Usually begnin, they typically present as a painless neck mass. If bilateral as in the case presented a genetic cause should be considered. Our patient tested positive for a mutation in the SDHD gene responsible for the syndrome. Some neuroendocrine tumours express high numbers of somatostatin receptors. Labelled octreotide, a somatostatin analogue enables accurate tumour localisation intraoperatively but can also be used as a treatment in those not amenable to surgical resection. To date its use has largely been in gastric neuroendocrine tumours but our case illustrates an emerging role in head and neck tumours.

Authors/Disclosures
Jennifer Williams, MD (Massachusetts General Hospital, Brigham, Harvard) PRESENTER	No disclosure on file
	No disclosure on file
Douglas L. Arnold, MD, FAAN (Montreal Neurological Institute, McGill Univ)	Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for BMS. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Frequency Therapeutics. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck. Dr. Arnold has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Arnold has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Shionogi. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Xfacto communications. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Find therapeutics. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for GSK. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Idorsia. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Kiniksa. Dr. Arnold has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Clario.
Justin Kinsella, MD (St.Vincent's University Hospital)	Dr. Kinsella has nothing to disclose.
	No disclosure on file
	No disclosure on file
Christopher McGuigan, MD (Department of Neurology, St. Vincent's University Hospital)	An immediate family member of Prof. McGuigan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Prof. McGuigan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Prof. McGuigan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. The institution of Prof. McGuigan has received research support from Novartis.
Paul M. Vespa, MD, FAAN (UCLA)	Dr. Vespa has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for ceribell. Dr. Vespa has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for ceribell. Dr. Vespa has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Ceribell. Dr. Vespa has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for multiple. The institution of Dr. Vespa has received research support from NIH. Dr. Vespa has received intellectual property interests from a discovery or technology relating to health care.

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