the role of medical therapy in the management of desmoid tumor · sunitinib: kit, pdgfr , vegfr and...
TRANSCRIPT
![Page 1: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/1.jpg)
The role of medical therapy in the management of
desmoid tumor
Andrea Marrari, MDVisiting Research Fellow
Center for Sarcoma and Bone Oncology
Dana-Farber Cancer Institute
![Page 2: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/2.jpg)
Sporadic tumors are rare (600-1200 cases per year in the US)
Patients with familial adenomatous polyposis have an increased risk of developing desmoid tumor
Unpredictable clinical behaviour
Desmoid tumor
![Page 3: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/3.jpg)
Desmoids can be found in a wide variety of locations
10% head and neck
22% scapular girdle
10% upper extremity
25% abdominal wall
5% intra-abdominal
5% pelvic girdle
23% lower extremity
![Page 4: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/4.jpg)
Surgery is the primary treatment for DT
![Page 5: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/5.jpg)
Survival is virtually unaffected by
extra-abdominal DT
![Page 6: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/6.jpg)
Some anatomic sites may become
life-threatening:mesentery and head
and neck
![Page 7: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/7.jpg)
Medical therapy for DT
Who to treat
What to use
How long
Toxicity
![Page 8: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/8.jpg)
Treatment strategy
Resectable Unresectable
Stable
Progressing Surgery
Surgery Observation
Medical therapy+/- surgery
Observation
Medical therapy+/- surgery
![Page 9: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/9.jpg)
Active drugs
Anti-estrogens
Nonsteroidal anti-inflammatory drugs
Chemotherapy
Molecular targeted therapy
Interferons
![Page 10: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/10.jpg)
Tamoxifen
Tamoxifen 20 mg/day for 2 years
Courtesy of Gronchi A., INT
![Page 11: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/11.jpg)
Anti-inflammatory drugs
Nishida Y. et al, JCO 2010
![Page 12: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/12.jpg)
Combination strategy
![Page 13: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/13.jpg)
Low dose chemotherapy
Methotrexate and vinblastine
Methotrexate and vinorelbine
Vinorelbine
![Page 14: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/14.jpg)
Methotrexate and vinorelbine
Methotrexate and vinorelbine for 26 cycles
Courtesy of Gronchi A., INT
![Page 15: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/15.jpg)
Conventional chemotherapy
Adriamycin
Adriamycin and dacarbazine
Liposomal doxorubicin
![Page 16: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/16.jpg)
Liposomal doxorubicin
Liposomal doxorubicin after 9 months
Bertagnolli M.M. et al, Eur J Cancer 2006
![Page 17: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/17.jpg)
Molecular targeted therapy
![Page 18: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/18.jpg)
Molecular targeted therapy
Cell proliferationAngiogenesisMetabolism
Motility…
Ligand
Receptor
STOP
Intracellular space
Extracellular space
Adapted from Krause D.S. et al, NEJM 2005
![Page 19: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/19.jpg)
Molecular targeted therapy
Imatinib: ABL, KIT and PDGFR
Sunitinib: KIT, PDGFR , VEGFR and RET
Sorafenib: KIT, PDGFR, VEGFR, RET and RAF
![Page 20: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/20.jpg)
Imatinib
Post 14 months8.7 cm
Baseline13.9 cm
Heinrich M.C. et al, JCO 2006
Imatinib400 mg twice daily
![Page 21: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/21.jpg)
Molecular targeted therapy
Imatinib: ABL, KIT and PDGFR
Sunitinib: KIT, PDGFR , VEGFR and RET
Sorafenib: KIT, PDGFR, VEGFR, RET and RAF
![Page 22: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/22.jpg)
Molecular targeted therapy
Imatinib: ABL, KIT and PDGFR
Sunitinib: KIT, PDGFR , VEGFR and RET
Sorafenib: KIT, PDGFR, VEGFR, RET and RAF
![Page 23: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/23.jpg)
Sorafenib
Gounder M.M. et al, ASCO 2010
Sorafenib400 mg twice daily
Post 11 monthsBaseline
![Page 24: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/24.jpg)
Multidisciplinary assessment and treatment planning is critical
Treatment should be tailored according to patients characteristics and tumor behaviour
Chemotherapy is effective in disease control
Conclusion
![Page 25: The role of medical therapy in the management of desmoid tumor · Sunitinib: KIT, PDGFR , VEGFR and RET Sorafenib: KIT, PDGFR, VEGFR, RET and RAF. Sorafenib Gounder M.M. et al, ASCO](https://reader035.vdocument.in/reader035/viewer/2022063009/5fbfa09fa1f17d7c563c513f/html5/thumbnails/25.jpg)
GRAZIE!
Milan Cathedral, 1368-1965