Aaron Weiss, DO

Associate Professor of Pediatrics

Tufts University School of Medicine

Pediatric Hematology-Oncology

Maine Children’s Cancer Program

weissa2@mmc.org

A New Platform for

Discussion of Challenging

Desmoid Tumor Cases: DTRF

Virtual Tumor Board

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

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

Purpose

Creation of an established scientific forum for

discussion of complex desmoid tumor cases

Planning

Small working group discussions

Surgical oncology

Radiology

Pathology

Medical oncology

Pediatric oncology

Informatics

DTRF

Sponsorship

Desmoid Tumor Research Foundation

Administrative

Financial

Format

Regularly scheduled meetings

Quarterly

First Wednesday of the month

Web-based conference platform

GoToMeeting

Core of committed specialists

Personnel

Oncology (medical and pediatric)

Radiology

Surgical oncology (general and orthopedic)

Pathology

Radiation oncology

Access

Open to medical personnel (no patients)

Initially North America but more recent

international expansion

Process

Cases solicited ahead of meeting

Meeting announcements

Powerpoint presentation preferred

Presenting site responsible for providing their

own radiology/pathology personnel (if

applicable)

Security

HIPAA provisions

Removal of Protected Health Information

Name, date of birth, age, date of scans

Pre-presentation site instructions

Sample Cases

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

Sample Case #1

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

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?

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

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

Examination

Biopsy

Systemic Therapy Literature Review

Methotrexate/Vinblastine

POG 9650

2 yr PFS 46%

CWS Ped Blood & Cancer

May 2018

90 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

Sorafenib 400 mg1 Pazopanib 800mg2

Trial Design Randomized, placebo crossover

Randomized, MTX/VBL crossover

n 87 (37 p, 50 S) 72 (24 MV, 48 PZ)

n assessed 75 66

Ages 18-72 yr 18 -79 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%

1Gounder MM, et al. ASCO 20182Toulmonde M, et al. ASCO 2018

Tyrosine Kinase Inhibitor Literature Review

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?

Mission

Multidisciplinary

Resourceful

Inclusive

Collaborative

Challenges

Publicity

Time zone considerations for international

participants

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

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)

Interested in Have Your Case

Presented?

Aaron Weiss: weissa2@mmc.org

or

Lynne Hernandez: lynne@dtrf.org