NF2/SCHWANNOMATOSIS: STATE OF THE ART 2023 AGENDA

UCLA Luskin Conference Center – Salon C Level 1 (map)

Co-Chairs: Marco Giovannini and P. Leia Nghiemphu (UCLA)

PRINTABLE AGENDA

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Thursday, January 12, 2023

1:00 - 5:00 PM PT

Registration

Patient “Forum”: Meet the experts format

Speakers:
Scott Plotkin (MGH) “Medical Treatments for NF2/SWN”
William Slattery (House Clinic) “Hearing Rehabilitation for NF2-related Vestibular Schwannomas”
Laura Bonelli (UCLA) “Ophthalmological Symptoms in NF2/SWN”
Michel Kalamarides (L’Hôpital de la Pitié Salpêtrière) “Surgical Treatments for NF2/SWN”

Questions & Answers Session:
Panel speakers: Scott Plotkin, Laura Bonelli, Michel Kalamarides, William Slattery, Matthias Karajannis (MSKCC), Vic Riccardi

5:00 - 6:00 PM PT

Welcome Cocktail Reception

Friday, January 13, 2023

8:00 AM - 8:30 AM PT

Patient’s perspective
“Born with a rare disease: building a self-empowering mindset”.
Presentations by NF2-rel & other SWN patients: Michele Holbrook, Clayton Mellina, Onno Faber, Billy Nguyen.

8:30 AM - 10:30 AM PT

HEARING AND FACIAL NERVE PRESERVATION & REHABILITATION
Chairs: Derald Brackmann (House Clinic) and Irene Kim (UCLA).

KEYNOTE:
Gabriel Corfas (U. of Michigan) “Schwann cells in the auditory system”.

Speakers:
Bradley Welling (MEEI) “Genotype influence on surgical intervention in NF2”
Akira Ishiyama (UCLA) “implications for Cochlear implantation based on histopathology”
Scott Plotkin (MGH) “drug therapy for hearing preservation in NF2”

10:30 AM - 10:45 AM PT

Break

10:45 AM - 11:30 AM PT

Speakers:
Irene Kim (UCLA) “Facial nerve rehabilitation”
Kevin Peng (House Clinic) “Middle Fossa approach for hearing preservation”
Tom Roland (NYU) “Cochlear implants in the face of retro cochlear pathology”

11:30 AM - 12:30 PM PT

HEARING AND FACIAL NERVE PRESERVATION & REHABILITATION

Panel Discussion: Patient Management dilemmas and Case Studies
Moderators: Bradley Welling (MEEI); Richard Everson (UCLA)
Panel: Quinton Gopen (UCLA), Akira Ishiyama (UCLA), Scott Plotkin (MGH), Irene Kim (UCLA), Kevin Peng (House Clinic), Tom Roland (NYU)

12:30 PM - 1:30 PM PT

Lunch Buffet
Salon A, Luskin Conf Center

1:30 PM - 3:30 PM PT

GENETICS
Chairs: Miriam Smith (Manchester, UK) and Laura Papi (University of Florence, IT).

KEYNOTE:
Gareth Evans (U. of Manchester, UK) “NF2/Schwannomatosis: an overview and new classification/diagnostic criteria”

Speakers:
Laura Papi “Somatic NF2/SWN mutations in tumors”
Miriam Smith (Manchester, UK) ““Genotype-phenotype correlations of CNVs in NF2-related schwannomatosis””
Alicia Gomes (UAB) “NF Variant Curation Expert Panel (VCEP)“

Oral Abstracts:
Kara Anstett “Unexpected findings in LZTR1: Results of a single center’s approach to referral and assessment of patients with incidental variants”

Gareth Evans “Differential rates of germline heterozygote and mosaic variants in NF2 may show varying propensity for meiotic or mitotic mutation”

3:30 PM -3:45 PM PT

Break

3:45 PM - 5:00 PM PT

PAIN AND OTHER SYMPTOMS
Chairs: Kaleb Yohay (NYU) and Larry Sherman (OHSU)

Speakers:
Larry Sherman (OHSU) “SMARCB1 as factors in Schwann cells that directly mediate pain”
Kimberley Ostrow (JH) “Pain in NF2/SWN”
Stephanie Garcia (UCLA) “Ophthalmological Complications”

Oral Abstracts:
Heather Thompson “Patient reported measures of tinnitus for individuals with neurofibromatosis 2”

Saturday, January 14, 2023

8:00 AM - 9:00 AM PT

QUALITY OF LIFE
Chairs: Ros Ferner (Guy’s and St. Thomas’ Hospital, NHS, London, UK) and Tena Rosser (CHLA).

Speakers:
Vanessa Merker (MGH) “Impact of Novel Therapy on NF2 Schwannomatosis Quality of Life”

Panel Discussion: Clinical Cases
Tena Rosser “Pediatric NF2-SWN”
Ros Ferner “Adult NF2-SWN”
Victoria Williams “Other SWN”

9:00 AM - 10:15 AM PT

SURGERY AND RADIOSURGERY
Chairs: Michel Kalamarides (Hôpital La Pitié Salpêtrière, Paris, FR) and Leia Nghiemphu (UCLA)

KEYNOTE:
David Raleigh (UCSF) “Meningioma DNA methylation groups”

Speakers:
Michel Kalamarides (Paris University, France) “Spinal Ependymomas Treatments”
John Golfinos (NYU) “Surgeries in NF2”
William Slattery (House Clinic) “Surgeries of NF2-related Schwannomas”

10:15 AM - 10:30 AM PT

Break

10:30 AM - 11:15 AM PT

SURGERY AND RADIOSURGERY

Speakers:
Allan Belzberg (JH) “Peripheral Nerve Surgery – Schwannomatosis”
John Hegde (UCLA) “Radiation treatments of NF2-rel VS”

Oral Abstracts:
Gareth Evans “Radiation treatment of benign tumours in NF2-schwannomatosis: a national study of 266 irradiated patients showing a significant increase in malignancy”

Ina Ly “Long-term follow-up of SWN growth behavior in adult NF2 and SWN patients using whole-body MRI” (presented by Justin Jordan)

11:15 AM - 12:30 PM PT

SURGERY AND RADIOSURGERY

Panel discussion: Patient Management dilemmas and Case Studies (of non-VS tumor types).
Moderators: John Golfinos (NYU), Leia Nghiemphu (UCLA)
Panel: Michel Kalamarides (Paris University, France), William Slattery (House Clinic), Allan Belzberg (JH), John Hegde (UCLA), Fausto Rodriguez (UCLA)

12:30 PM - 1:30 PM PT

Lunch Buffet
Salon A, Luskin Conf Center

1:30 PM - 3:00 PM PT

BASIC AND TRANSLATIONAL RESEARCH
Chairs: Andi McClatchey (MGH) and Jeremie Vitte (UCLA)

Speakers:
Andi McClatchey (MGH) “Mapping schwannoma heterogeneity”
David Parkinson (Plymouth, UK) “Targetting Hippo pathway in Schwann cells”
Lei Xu (MGH) “Mouse model of spine/peripheral schwannoma/pain”
Masahiro Toda (Tokyo, Japan) “VEGF receptor vaccine”
Jeremie Vitte (UCLA) “Swine model of NF2-rel SWN”

3:00 PM - 3:15 PM PT

Break

3:15 PM - 4:00 PM PT

BASIC AND TRANSLATIONAL RESEARCH

Oral Abstracts:
Grace Gregory “Sporadic and NF2-rela SWN VS Share Similar Tumour Microenvironments”
Elisabeth Castellanos “Alteration of pluripotency and differentiation capacities in Merlin-deficient iPSCs”
Pipasha Biswas “Development of a novel in vitro model for NF2…” (presented by K. Meyer)
Judith Kempfie “A mouse model of neurofibromatosis type 2 with Lin28 associated glial cell proliferation”
Adam Paul Jones “High dimensional imaging highlights the T-cell compartment as a potential therapeutic for both NF2-rel SWN and sporadic VS”

4:00 PM - 5:00 PM PT

BASIC AND TRANSLATIONAL RESEARCH

Panel Discussion: Future directions for NF2/SWN research pipeline: From discovery to therapeutic development
Moderator: Andi McClatchey (MGH) and Annette Bakker (CTF)

Speaker: Annette Bakker (CTF) “Schwannomatoses Target Product Profiles”

Panel: David Parkinson (UK), Long-Sheng Chang (NationwideCH).

6:00 PM - 9:00 PM PT

RECEPTION
Salon A, Luskin Conference Center

Sunday, January 15, 2023

8:00 AM - 9:45 AM PT

CURRENT MEDICAL TREATMENTS
Chairs: Richard Everson (UCLA) & Jaishri Blakeley (John Hopkins)

Speakers:
Prashant Chittiboina (NIH) “Natural History Study of NF2-SWN”
Leia Nghiemphu (UCLA) “Previous Published Clinical Trials in NF2/SWN”
Scott Plotkin (MGH) “Ongoing Clinical Trials (INTUITT-NF2)”
Matthias Karajannis (MSKCC) “Upcoming Clinical Trial Options in NF2/SWN”

Oral Abstracts:
Matthias Karajannis “Phase II Study of Axitinib in Patients with Neurofibromatosis Type 2 and Progressive Vestibular Schwannomas”
Justin Jordan “Guideline Concordant Care in NF2-Related Schwannomatosis”

Panel discussion: Patient Management dilemmas and Case Studies
Moderator: Jaishri Blakeley (JH). Richard Everson (UCLA)
Panel: Leia Nghiemphu, Scott Plotkin, Matthias Karajannis, Fausto Rodriguez

9:45 AM - 10:00 AM PT

Break

10:00 AM - 12:00 PM PT

FUTURE TREATMENTS
Chairs: Scott Plotkin (MGH) and Marco Giovannini (UCLA)

KEYNOTE:
David Nathanson (UCLA) [Drug Development of brain-penetrant EGFr inhibitors]

Speakers
Gilles Atlan (NF2 Biosolutions)
Gary Brenner (MGH) “Bacterial-based Immune Therapy”

Oral Abstracts:
Kathrin Meyer “A Highly Promising Adeno-Associated Viral (AAV) Gene Replacement Therapy Vector Effectively Reduces Tumor Growth in In Vitro and In Vivo Schwannoma Models”
Long-Sheng Chang “Combining brigatinib with mTOR inhibition to effectively treat NF2-deficient meningiomas”

Audience Discussion