HCI Sarcoma Services Desmoid Tumors: The Nonmalignant Malignancy R. Lor Randall MD, FACS The L.B. and Olive S. Young Presidential Chair for Cancer Research Director, Sarcoma Services & SARC Fellowship, Lab Medical Director, HCH Surgical Services Co-Leader Sarcoma Disease Oriented Research Team Co-Director, Huntsman-Intermountain Adolescent & Young Adult Cancer Care Program Huntsman Cancer Institute & Primary Children’s Hospital University of Utah Sara Shaw, BS John Groundland, MD DTRF Western Region Meeting April 28, 2017 SLC, UT
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HCI Sarcoma Services
Desmoid Tumors:The Nonmalignant Malignancy
R. Lor Randall MD, FACSThe L.B. and Olive S. Young Presidential Chair for Cancer Research
associated with familial adenomatous polyposis. Sarcoma. 2012;2012:726537
Studies Investigating Desmoid Response to Medicine•Chugh R, Wathen JK, Patel SR, Maki RG, Meyers PA, Schuetze SM, Priebat DA, Thomas DG, Jacobson JA, Samuels
BL, Benjamin RS, Baker LH; Sarcoma Alliance for Research through Collaboration (SARC). Efficacy of imatinib in
aggressive fibromatosis: Results of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC)
trial. Clin Cancer Res. 2010 Oct 1;16(19):4884-9
•Dufresne A, Bertucci F, Penel N, Le Cesne A, Bui B, Tubiana-Hulin M, Ray-Coquard I, Cupissol D, Chevreau C, Perol
D, Goncalves A, Jimenez M, Bringuier PP, Blay JY. Identification of biological factors predictive of response to imatinib
mesylate in aggressive fibromatosis. Br J Cancer. 2010 Aug 10;103(4):482-5.
•Kasper B, Gruenwald V, Reichardt P, Bauer S, Rauch G, Limprecht R, Sommer M, Dimitrakopoulou-Strauss A, Pilz L,
Haller F, Hohenberger P. Imatinib induces sustained progression arrest in RECIST progressive desmoid tumours: Final
results of a phase II study of the German Interdisciplinary Sarcoma Group (GISG). Eur J Cancer. 2017 May;76:60-67.
•Kummar S, O'Sullivan Coyne G, Do KT, Turkbey B, Meltzer PS, Polley E, Choyke PL, Meehan R, Vilimas R, Horneffer
Y, Juwara L, Lih A, Choudhary A, Mitchell SA, Helman LJ, Doroshow JH, Chen AP. Clinical Activity of the γ-Secretase
Inhibitor PF-03084014 in Adults With DesmoidTumors (Aggressive Fibromatosis). J Clin Oncol. 2017 May
10;35(14):1561-1569.
•Penel N, Le Cesne A, Bui BN, Perol D, Brain EG, Ray-Coquard I, Guillemet C, Chevreau C, Cupissol D, Chabaud S,
Jimenez M, Duffaud F, Piperno-Neumann S, Mignot L, Blay JY. Imatinib for progressive and recurrent aggressive
fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up. Ann Oncol.
2011 Feb;22(2):452-7
•Skapek SX, Anderson JR, Hill DA, Henry D, Spunt SL, Meyer W, Kao S, Hoffer FA, Grier HE, Hawkins DS, Raney RB.
Safety and efficacy of high-dose tamoxifen and sulindac for desmoid tumor in children: results of a Children's Oncology
Group (COG) phase II study. Pediatr Blood Cancer. 2013 Jul;60(7):1108-12
Additional References
Radiation
•Keus RB, Nout RA, Blay JY, de Jong JM, Hennig I, Saran F, Hartmann JT, Sunyach MP, Gwyther SJ,
Ouali M, Kirkpatrick A, Poortmans PM, Hogendoorn PC, van der Graaf WT. Results of a phase II pilot
study of moderate dose radiotherapy for inoperable desmoid-type fibromatosis--an EORTC STBSG and
ROG study. Ann Oncol. 2013 Oct;24(10):2672-6.
Ultrasound Ablation of Desmoid Tumors
•Bucknor MD, Rieke V.. MRgFUS for desmoid tumors within the thigh: early clinical experiences. J Ther
Ultrasound. 2017 Feb 3;5:4
•Ghanouni P, Dobrotwir A, Bazzocchi A, Bucknor M, Bitton R, Rosenberg J, Telischak K, Busacca M,
Ferrari S, Albisinni U, Walters S, Gold G, Ganjoo K, Napoli A, Pauly KB, Avedian R. Magnetic resonance-
guided focused ultrasound treatment of extra-abdominal desmoidtumors: a retrospective multicenter study.
Eur Radiol. 2017 Feb;27(2):732-740
Biology of Desmoid Tumors•Trang SH, Joyner DE, Damron TA, Aboulafia AJ, Randall RL. Potential for functional redundancy in EGF and TGFalpha signaling in desmoid cells: a cDNA microarray analysis. Growth Factors. 2010 Feb;28(1):10-23.
•Joyner DE, Trang SH, Aboulafia AJ, Damron TA, Randall RL. FAP-associated desmoid invasiveness correlates with in vitro resistance to doxorubicin. Fam Cancer. 2009;8(4):569-80.
•Joyner DE, Trang SH, Damron TA, Aboulafia AJ, Cummings JE, Randall RL. Desmoid cell motility is induced in vitro by rhEGF. J Orthop Res. 2009 Sep;27(9):1258-62