Abstract Details

Title Aquaporin-4 IgG Positive Neuromyelitis Optica Spectrum Disorder: Frequency and Type of Cancer Co-Existence, Antigen Expression and Proof of Paraneoplastic Phenotype

Topic Autoimmune Neurology

Presentation(s) P11 - Poster Session 11 (8:00 AM-9:00 AM)

Poster/Presentation
Number 8-018

Objective To report malignancy screening data in a large AQP4+NMOSD cohort and investigate the AQP4 expression in tumor tissues.

Background The link between tumors expressing AQP4 and NMOSD onset is a fascinating area of research. Paraneoplastic AQP4+NMOSD (PAN) was labeled by prior studies to have a male predominance, area postrema syndrome at onset, definite association of adenocarcinomas, without emphasis on demonstrating AQP4 expression on tumors.

Design/Methods Retrospective chart analysis was performed in AQP4+NMOSD cohort; patients with cancers diagnosis independent of the time cutoff from the onset of NMOSD were identified; tumor tissues underwent immunohistochemical (IHC) analysis for AQP4 expression, cases tested positive were classified as PAN. Age-appropriate (AA) malignancy screening data was gathered per American Cancer Society guidelines.

Results

33 NMO patients had coexisting malignancies (male=6, female=27) with median age of onset (MAO) of 57 years (range 15-71), comprising 9% of AQP4-NMOSD cohort.

AQP4 expression was present in 4/13 tumors tested. All 4 were women (MOA 57, range 30-59 years), cancers were identified 29 months (range 1-86) after NMOSD onset. Patient 1, with recurrent ON, had serous ovarian adenocarcinoma; Patient 2, with recurrent LETM, had papillary thyroid carcinoma; Patient 3, with area postrema attack, had ductal carcinoma breast. Patient 4, with multifocal attack had poorly differentiated breast carcinoma.

AQP4 expression was absent- endometrial adenocarcinomas (2), lung squamous cell carcinoma (1), clear cell renal carcinomas (2), Lymphomas (4).

In AQP4+NMOSD cohort (n=394, male=63, female=331), 40% (160, male=36, female=124) were in age of NMOSD onset >50 years subset. Complete AA malignancy screening was pursued in 19% of males, 19% of females; partial AA malignancy screening in 39% of males, 49% of females.

Conclusions

Most of the coexisting malignancies are incidental per IHC analysis. PAN has no specific phenotypic preponderance or a definite association with adenocarcinomas. Further proteomic analysis studies are necessary to guide malignancy screening in AQP4+NMOSD.

Authors/Disclosures
Naga Pradyumna Kothapalli, MD (Stanford University) PRESENTER	Dr. Kothapalli has nothing to disclose.
Yong Guo	Yong Guo has nothing to disclose.
Dean M. Wingerchuk, MD, FAAN (Mayo Clinic)	Dr. Wingerchuk has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Wingerchuk has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Wingerchuk has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Meyer Squibb. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharma. Dr. Wingerchuk has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca. Dr. Wingerchuk has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Wingerchuk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abcuro. Dr. Wingerchuk has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wolters Kluwers.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to AAN interests or activities.
Divyanshu Dubey, MD, FAAN (Mayo Clinic)	The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.
Fernando X. Cuascut Lassus, MD	Dr. Cuascut Lassus has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Cuascut Lassus has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Cuascut Lassus has received personal compensation in the range of $500-$4,999 for serving as a Speaker with MSAA.
Hemali Patel, DO (Baylor College of Medicine)	Dr. Patel has nothing to disclose.
Nanthaya Tisavipat, MD (Mayo Clinic)	Miss Tisavipat has nothing to disclose.
Mark Keegan, MD, FAAN (Mayo Clinic)	Dr. Keegan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Keegan has received publishing royalties from a publication relating to health care. Dr. Keegan has received publishing royalties from a publication relating to health care.
W. O. Tobin, PhD, MBBCh, BAO, FAAN (Mayo Clinic)	Dr. Tobin has received publishing royalties from a publication relating to health care.
Cristina Valencia Sanchez, MD (Mayo Clinic Arizona)	Dr. Valencia Sanchez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG therapeutics. Dr. Valencia Sanchez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Valencia Sanchez has a non-compensated relationship as a member of the medical advisory board with The MOG Project that is relevant to AAN interests or activities.
Claudia F. Lucchinetti, MD, FAAN (University of De Medical School, Health Learning Blg)	The institution of Dr. Lucchinetti has received research support from Biogen Idec. The institution of Dr. Lucchinetti has received research support from NIH/NINDS. The institution of Dr. Lucchinetti has received research support from National Institute of Neurological Disorders and Stroke . The institution of Dr. Lucchinetti has received research support from National Multiple Sclerosis Society. The institution of Dr. Lucchinetti has received research support from National Center for Advancing Translational Sciences. Dr. Lucchinetti has received intellectual property interests from a discovery or technology relating to health care. Dr. Lucchinetti has a non-compensated relationship as a Member with National Institute of Neurological Disorders that is relevant to AAN interests or activities. Dr. Lucchinetti has a non-compensated relationship as a Member with Board of Directors, Association of University Professors of Neurology that is relevant to AAN interests or activities. Dr. Lucchinetti has a non-compensated relationship as a Member with Mayo Clinic Board of Trustees that is relevant to AAN interests or activities. Dr. Lucchinetti has a non-compensated relationship as a Member with Mayo Clinic Board of Governors that is relevant to AAN interests or activities.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology)	Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.

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