A New Platform for Discussion of Challenging Desmoid Tumor Cases: DTRF Virtual Tumor Board

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Norah Reeves
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1 A New Platform for Discussion of Challenging Desmoid Tumor Cases: DTRF Virtual Tumor Board Aaron Weiss, DO Associate Professor of Pediatrics Tufts University School of Medicine Pediatric Hematology-Oncology Maine Children s Cancer Program weissa2@mmc.org

2 Tumor Board Definition Multidisciplinary treatment planning approach for reviewing and discussing the medical condition and treatment options of a patient NCI Dictionary of Cancer Terms

3 Background No standardized approach to upfront, progressive or recurrent desmoid tumor management Opinions of experts frequently sought Advice often given with limited information regarding the case

4 Purpose Creation of an established scientific forum for discussion of complex desmoid tumor cases

5 Planning Small working group discussions Surgical oncology Radiology Pathology Medical oncology Pediatric oncology Informatics DTRF

6 Sponsorship Desmoid Tumor Research Foundation Administrative Financial

7 Format Regularly scheduled meetings Quarterly First Wednesday of the month Web-based conference platform GoToMeeting Core of committed specialists

8 Personnel Oncology (medical and pediatric) Radiology Surgical oncology (general and orthopedic) Pathology Radiation oncology

9 Access Open to medical personnel (no patients) Initially North America but more recent international expansion

10 Process Cases solicited ahead of meeting Meeting announcements Powerpoint presentation preferred Presenting site responsible for providing their own radiology/pathology personnel (if applicable)

11 Security HIPAA provisions Removal of Protected Health Information Name, date of birth, age, date of scans Pre-presentation site instructions

12 Sample Cases

13 Sample Case #1 18 yo male with familial adenomatous polyposis (FAP) syndrome Maternal history of FAP Underwent screening colonoscopy in 2014 Found numerous polyps removal of colon Developed lower abdominal/back pain in October 2015 Abdominal mass discovered Needle biopsy performed

14 Sample Case #1

15 Sample Case #1 December 2015: started on sorafenib Mild decrease in symptoms and tumor burden October 2016: changed to tamoxifen/sulindac Worsening pain and new concerns of bowel encased within the tumor Surgery being recommended Would lead to permanent colostomy and patient reluctant

17 Sample Case #1 Questions for the group: Is surgery a reasonable option as a next step? What other, if any, neoadjuvant (presurgical) or adjuvant (postsurgical) therapies should be considered? Is this patient eligible for any open clinical trials?

18 Sample Case #2 17 year old male with two year history of enlarging right shoulder/axillary mass 6 months of pain, decreased mobility, and preferentially using non-dominant hand Active wake boarder, notes he tends to have prolonged scars for years after superficial trauma

19 Sample Case #2 Past Medical History: scoliosis Family History: brother seizures, MGM breast cancer in remission, MGGF colon and prostate cancer, MGGA ovarian cancer, MGGM breast and pancreatic cancer

20 Examination

22 Biopsy

23 Systemic Therapy Literature Review Methotrexate/Vinblastine POG yr PFS 46% CWS Ped Blood & Cancer May children and adolescents 54 initial resection 64 received systemic therapy (40% received MV) SD 39% at 3 months and PR 53% at 6 months 21/35 secondary resection following primary systemic therapy Reports with TKI Agresta L, et al. Ped Blood & Cancer June 2018 Pazopanib 6 patients (PR in 2/7 tumors and SD in 6/7 tumors) Benech N, et al. NEJM June 2017 Two adult female patients with FAP and intra-abdominal tumors Multiple prior therapies Sorafenib 600 mg daily plus celecoxib 400 mg daily 90% and 50% reductions in tumor volume

Tyrosine Kinase Inhibitor Literature Review Trial Design Sorafenib 400 mg 1 Pazopanib 800mg 2 Randomized, placebo crossover Randomized, MTX/VBL crossover n 87 (37 p, 50 S) 72 (24 MV, 48 PZ) n

24 Tyrosine Kinase Inhibitor Literature Review Trial Design Sorafenib 400 mg 1 Pazopanib 800mg 2 Randomized, placebo crossover Randomized, MTX/VBL crossover n 87 (37 p, 50 S) 72 (24 MV, 48 PZ) n assessed Ages yr yr Male: Female 27: 60 26: 46 Results S p PZ MV PR 33% 21% 37% 25% SD 45.7% 30% PD 16% 69% 1 yr PFS 87% 43% 6 mo non-pd 86% 50% 1 Gounder MM, et al. ASCO Toulmonde M, et al. ASCO 2018

25 Sample Case #2 Questions for the group: Systemic therapy options Methotrexate/Vinblastine? Would stable response at any timepoint lead to consideration of tyrosine kinase inhibitor therapy? Systemic therapy versus surgical management or radiation Beyond disease progression, any other indications for local control? Considerations of side effects?

26 Mission Multidisciplinary Resourceful Inclusive Collaborative

27 Challenges Publicity Time zone considerations for international participants

28 Status Meetings December 6, 2017: 2 cases February 7, 2018: 4 cases May 2, 2018: 3 cases August 1, 2018: 4 cases Next: November 7, 2018

29 Acknowledgements Aimee Crago (surgical oncology) Keila Torres (surgical oncology) Rob Lefkowitz (radiology) Matt van de Rijn (pathology) Steve Attia (medical oncology) Robert Benjamin (medical oncology) Palma Dileo (medical oncology) Joe Earl and Tom Ellingson (informatics) Marlene, Jeanne and Lynne (DTRF)

30 Interested in Have Your Case Presented? Aaron Weiss: or Lynne Hernandez:

FEASIBILITY OF PRE- OPERATIVE mtor INHIBITOR SIROLIMUS IN CHILDREN AND YOUNG ADULTS WITH DESMOID TUMOR

FEASIBILITY OF PRE- OPERATIVE mtor INHIBITOR SIROLIMUS IN CHILDREN AND YOUNG ADULTS WITH DESMOID TUMOR Aaron Weiss, DO Associate Professor of Pediatrics Tufts University School of Medicine Pediatric Hematology-Oncology