Latest revision as of 11:23, 13 May 2024

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Section editor
	Elizabeth J. Davis, MD Vanderbilt University Nashville, TN, USA

Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens. For placebo or observational studies in this condition, please visit this page. If you still can't find it, please let us know so we can add it!

5 regimens on this page 7 variants on this page

Guidelines

Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.

French Intergroup

2022: Benech et al. Desmoid tumors located in the abdomen or associated with adenomatous polyposis: French intergroup clinical practice guidelines for diagnosis, treatment, and follow-up (SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO, ACHBT, SFR) PubMed

NCCN

NCCN does not currently have guidelines at this granular level; please see NCCN Guidelines - Soft Tissue Sarcoma.

All lines of therapy

Imatinib monotherapy

Regimen variant #1, fixed dose

Study	Dates of enrollment	Evidence
Kasper et al. 2017 (GISG-01)	2010-2013	Phase 2

Targeted therapy

Imatinib (Gleevec) 800 mg PO once per day

2 years of therapy

Regimen variant #2, BSA-based dosing

Study	Dates of enrollment	Evidence
Chugh et al. 2010	2002-10 to 2005-12	Phase 2

Targeted therapy

Imatinib (Gleevec) by the following BSA-based criteria:
- 1.50 m² or more: 300 mg PO twice per day
- 1.00 to 1.49 m²: 200 mg PO twice per day
- Less than 1.00 m²: 100 mg PO twice per day

Continued indefinitely

References

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):4884-91. Epub 2010 Aug 19. link to original article contains dosing details in manuscript PubMed
GISG-01: 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. Epub 2017 Mar 8. link to original article contains dosing details in manuscript PubMed NCT01137916

Methotrexate & Vinblastine

Regimen

Study	Dates of enrollment	Evidence
Skapek et al. 2007 (POG 9650)	1997-2001	Phase 2
Toulmonde et al. 2019 (DESMOPAZ)	2012-2017	Randomized Phase 2

Note: DESMOPAZ was randomized but non-comparative.

Chemotherapy

Methotrexate (MTX) 30 mg/m² IV once on day 1
Vinblastine (Velban) 5 mg/m² IV once on day 1

7-day cycle for 26 cycles, then 14-day cycle for 13 cycles

References

POG 9650: Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, Hoffer FA, Speights R, Gebhardt MC, Dahl GV, Grier HE; Pediatric Oncology Group. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 2007 Feb 10;25(5):501-6. link to original article contains dosing details in manuscript PubMed
DESMOPAZ: Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. link to original article contains dosing details in manuscript PubMed NCT01876082

Nirogacestat monotherapy

Regimen

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Gounder et al. 2023 (DeFi)	2019-05 to 2020-08	Phase 3 (E-RT-esc)	Placebo	Superior PFS (primary endpoint) PFS24: 76% vs 44% (HR 0.29, 95% CI 0.15-0.55)

Targeted therapy

Nirogacestat (Ogsiveo) 150 mg PO twice per day

28-day cycles

References

DeFi: Gounder M, Ratan R, Alcindor T, Schöffski P, van der Graaf WT, Wilky BA, Riedel RF, Lim A, Smith LM, Moody S, Attia S, Chawla S, D'Amato G, Federman N, Merriam P, Van Tine BA, Vincenzi B, Benson C, Bui NQ, Chugh R, Tinoco G, Charlson J, Dileo P, Hartner L, Lapeire L, Mazzeo F, Palmerini E, Reichardt P, Stacchiotti S, Bailey HH, Burgess MA, Cote GM, Davis LE, Deshpande H, Gelderblom H, Grignani G, Loggers E, Philip T, Pressey JG, Kummar S, Kasper B. Nirogacestat, a γ-Secretase Inhibitor for Desmoid Tumors. N Engl J Med. 2023 Mar 9;388(10):898-912. link to original article contains dosing details in manuscript PubMed NCT03785964

Pazopanib monotherapy

Regimen

Study	Dates of enrollment	Evidence
Toulmonde et al. 2019 (DESMOPAZ)	2012-2017	Randomized Phase 2

Note: this study was randomized but non-comparative.

Targeted therapy

Pazopanib (Votrient) 800 mg PO once per day

28-day cycle for up to 13 cycles (1 year)

References

DESMOPAZ: Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. link to original article contains dosing details in manuscript PubMed NCT01876082

Pegylated liposomal doxorubicin monotherapy

Regimen

Study	Dates of enrollment	Evidence
Constantinidou et al. 2009	2006-2009	Case series

Chemotherapy

Pegylated liposomal doxorubicin (Doxil) 50 mg/m² IV over 60 minutes once on day 1

28-day cycles

References

Case series: Constantinidou A, Jones RL, Scurr M, Al-Muderis O, Judson I. Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer. 2009 Nov;45(17):2930-4. Epub 2009 Sep 18. link to original article PubMed

Sorafenib monotherapy

Regimen

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Gounder et al. 2018 (Alliance A091105)	2014-2016	Phase 3 (E-esc)	Placebo	Superior PFS PFS24: 81% vs 36% (HR 0.13, 95% CI 0.05-0.31)

Targeted therapy

Sorafenib (Nexavar) 400 mg PO once per day on days 1 to 28

28-day cycles

References

Alliance A091105: Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ, Riedel RF, Sabagh T, Tap WD, Horvat N, Basch E, Schwartz LH, Maki RG, Agaram NP, Lefkowitz RA, Mazaheri Y, Yamashita R, Wright JJ, Dueck AC, Schwartz GK. Sorafenib for Advanced and Refractory Desmoid Tumors. N Engl J Med. 2018 Dec 20;379(25):2417-2428. link to original article contains dosing details in abstract link to PMC article PubMed NCT02066181

@@ Line 3: / Line 3: @@
 [[#top|Back to Top]]
 </div>
-{{#lst:Section editor transclusions|sarcoma}}
+{{#lst:Editorial board transclusions|sarcoma}}
-Note: these are regimens tested in subtype-specific populations, please see the '''[[Soft_tissue_sarcoma|main soft tissue sarcoma page]]''' for other regimens.
+''Note: these are regimens tested in subtype-specific populations, please see the '''[[Soft_tissue_sarcoma|main soft tissue sarcoma page]]''' for other regimens. For placebo or observational studies in this condition, please visit [[Desmoid tumor - null regimens|this page]]. If you still can't find it, please let us know so we can add it!''
 {| class="wikitable" style="float:right; margin-right: 5px;"
 |-
@@ Line 11: / Line 11: @@
 |}
 {{TOC limit|limit=3}}
+=Guidelines=
+'''Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.'''
+==French Intergroup==
+*'''2022:''' Benech et al. [https://doi.org/10.1016/j.dld.2022.03.004 Desmoid tumors located in the abdomen or associated with adenomatous polyposis: French intergroup clinical practice guidelines for diagnosis, treatment, and follow-up (SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO, ACHBT, SFR)] [https://pubmed.ncbi.nlm.nih.gov/35508462/ PubMed]
+==NCCN==
+*''NCCN does not currently have guidelines at this granular level; please see [https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1464 NCCN Guidelines - Soft Tissue Sarcoma].''
 =All lines of therapy=
 ==Imatinib monotherapy {{#subobject:hg35dc|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #1, fixed dose {{#subobject:1jv5c2|Variant=1}}===
 {| class="wikitable sortable" style="width: 60%; text-align:center;"
 !style="width: 33%"|Study
-!style="width: 33%"|Years of enrollment
+!style="width: 33%"|Dates of enrollment
 !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 |-
@@ Line 25: / Line 33: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Imatinib (Gleevec)]] 800 mg PO once per day
 '''2 years of therapy'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen variant #2, BSA-based dosing {{#subobject:ugu9ac|Variant=1}}===
 {| class="wikitable sortable" style="width: 60%; text-align:center;"
 !style="width: 33%"|Study
-!style="width: 33%"|Years of enrollment
+!style="width: 33%"|Dates of enrollment
 !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 |-
 |[https://doi.org/10.1158/1078-0432.ccr-10-1177 Chugh et al. 2010]
-|2002-2005
+|2002-10 to 2005-12
 | style="background-color:#91cf61" |Phase 2
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
-*[[Imatinib (Gleevec)]] by the following criteria:
+*[[Imatinib (Gleevec)]] by the following BSA-based criteria:
-**BSA of 1.5 m<sup>2</sup> or more: 300 mg PO twice per day
+**1.50 m<sup>2</sup> or more: 300 mg PO twice per day
-**BSA between 1 and 1.49 m<sup>2</sup>: 200 mg PO twice per day
+**1.00 to 1.49 m<sup>2</sup>: 200 mg PO twice per day
-**BSA less than 1 m<sup>2</sup>: 100 mg PO twice per day
+**Less than 1.00 m<sup>2</sup>: 100 mg PO twice per day
 '''Continued indefinitely'''
+</div></div>
 ===References===
 #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):4884-91. Epub 2010 Aug 19. [https://doi.org/10.1158/1078-0432.ccr-10-1177 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/20724445/ PubMed]
-#'''GISG-01:''' 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. Epub 2017 Mar 8. [https://doi.org/10.1016/j.ejca.2017.02.001 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/28282612/ PubMed] NCT01137916
+#'''GISG-01:''' 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. Epub 2017 Mar 8. [https://doi.org/10.1016/j.ejca.2017.02.001 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/28282612/ PubMed] [https://clinicaltrials.gov/study/NCT01137916 NCT01137916]
 ==Methotrexate & Vinblastine {{#subobject:f3a6dz|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:jgc1q1|Variant=1}}===
 {| class="wikitable sortable" style="width: 60%; text-align:center;"
 !style="width: 33%"|Study
-!style="width: 33%"|Years of enrollment
+!style="width: 33%"|Dates of enrollment
 !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 |-
@@ Line 71: / Line 80: @@
 |}
 ''Note: DESMOPAZ was randomized but non-comparative.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Methotrexate (MTX)]] 30 mg/m<sup>2</sup> IV once on day 1
 *[[Vinblastine (Velban)]] 5 mg/m<sup>2</sup> IV once on day 1
 '''7-day cycle for 26 cycles, then 14-day cycle for 13 cycles'''
+</div></div>
 ===References===
 #'''POG 9650:''' Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, Hoffer FA, Speights R, Gebhardt MC, Dahl GV, Grier HE; Pediatric Oncology Group. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 2007 Feb 10;25(5):501-6. [https://doi.org/10.1200/jco.2006.08.2966 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/17290057/ PubMed]
-#'''DESMOPAZ:''' Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. [https://doi.org/10.1016/s1470-2045(19)30276-1 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/31331699/ PubMed] NCT01876082
+#'''DESMOPAZ:''' Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. [https://doi.org/10.1016/s1470-2045(19)30276-1 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/31331699/ PubMed] [https://clinicaltrials.gov/study/NCT01876082 NCT01876082]
+==Nirogacestat monotherapy {{#subobject:f3agdc|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen {{#subobject:1fc9c2|Variant=1}}===
+{| class="wikitable sortable" style="width: 100%; text-align:center;"
+!style="width: 20%"|Study
+!style="width: 20%"|Dates of enrollment
+!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]]
+!style="width: 20%"|Comparator
+!style="width: 20%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]]
+|-
+|[https://doi.org/10.1056/NEJMoa2210140 Gounder et al. 2023 (DeFi)]
+|2019-05 to 2020-08
+| style="background-color:#1a9851" |Phase 3 (E-RT-esc)
+|[[Desmoid_tumor_-_null_regimens#Placebo|Placebo]]
+| style="background-color:#1a9850" |Superior PFS (primary endpoint)<br>PFS24: 76% vs 44%<br>(HR 0.29, 95% CI 0.15-0.55)
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Targeted therapy====
+*[[Nirogacestat (Ogsiveo)]] 150 mg PO twice per day
+'''28-day cycles'''
+</div></div>
+===References===
+#'''DeFi:''' Gounder M, Ratan R, Alcindor T, Schöffski P, van der Graaf WT, Wilky BA, Riedel RF, Lim A, Smith LM, Moody S, Attia S, Chawla S, D'Amato G, Federman N, Merriam P, Van Tine BA, Vincenzi B, Benson C, Bui NQ, Chugh R, Tinoco G, Charlson J, Dileo P, Hartner L, Lapeire L, Mazzeo F, Palmerini E, Reichardt P, Stacchiotti S, Bailey HH, Burgess MA, Cote GM, Davis LE, Deshpande H, Gelderblom H, Grignani G, Loggers E, Philip T, Pressey JG, Kummar S, Kasper B. Nirogacestat, a γ-Secretase Inhibitor for Desmoid Tumors. N Engl J Med. 2023 Mar 9;388(10):898-912. [https://doi.org/10.1056/NEJMoa2210140 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/36884323/ PubMed] [https://clinicaltrials.gov/study/NCT03785964 NCT03785964]
 ==Pazopanib monotherapy {{#subobject:f3agdc|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:0gc1c2|Variant=1}}===
 {| class="wikitable sortable" style="width: 60%; text-align:center;"
 !style="width: 33%"|Study
-!style="width: 33%"|Years of enrollment
+!style="width: 33%"|Dates of enrollment
 !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 |-
@@ Line 94: / Line 128: @@
 |}
 ''Note: this study was randomized but non-comparative.''
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
 *[[Pazopanib (Votrient)]] 800 mg PO once per day
+'''28-day cycle for up to 13 cycles (1 year)'''
-'''Up to 12 months of therapy'''
+</div></div>
 ===References===
-#'''DESMOPAZ:''' Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. [https://doi.org/10.1016/s1470-2045(19)30276-1 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/31331699/ PubMed] NCT01876082
+#'''DESMOPAZ:''' Toulmonde M, Pulido M, Ray-Coquard I, Andre T, Isambert N, Chevreau C, Penel N, Bompas E, Saada E, Bertucci F, Lebbe C, Le Cesne A, Soulie P, Piperno-Neumann S, Sweet S, Cecchi F, Hembrough T, Bellera C, Kind M, Crombe A, Lucchesi C, Le Loarer F, Blay JY, Italiano A. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol. 2019 Sep;20(9):1263-1272. Epub 2019 Jul 19. [https://doi.org/10.1016/s1470-2045(19)30276-1 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/31331699/ PubMed] [https://clinicaltrials.gov/study/NCT01876082 NCT01876082]
 ==Pegylated liposomal doxorubicin monotherapy {{#subobject:cd6479|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:3cc773|Variant=1}}===
 {| class="wikitable sortable" style="width: 60%; text-align:center;"
 !style="width: 33%"|Study
-!style="width: 33%"|Years of enrollment
+!style="width: 33%"|Dates of enrollment
 !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 |-
@@ Line 114: / Line 149: @@
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Chemotherapy====
 *[[Pegylated liposomal doxorubicin (Doxil)]] 50 mg/m<sup>2</sup> IV over 60 minutes once on day 1
 '''28-day cycles'''
+</div></div>
 ===References===
 #'''Case series:''' Constantinidou A, Jones RL, Scurr M, Al-Muderis O, Judson I. Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer. 2009 Nov;45(17):2930-4. Epub 2009 Sep 18. [https://doi.org/10.1016/j.ejca.2009.08.016 link to original article] [https://pubmed.ncbi.nlm.nih.gov/19767198/ PubMed]
 ==Sorafenib monotherapy {{#subobject:f3a4c7|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
 ===Regimen {{#subobject:0dc9c2|Variant=1}}===
 {| class="wikitable sortable" style="width: 100%; text-align:center;"
 !style="width: 20%"|Study
-!style="width: 20%"|Years of enrollment
+!style="width: 20%"|Dates of enrollment
 !style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]]
 !style="width: 20%"|Comparator
@@ Line 134: / Line 169: @@
 |2014-2016
 | style="background-color:#1a9851" |Phase 3 (E-esc)
-|Placebo
+|[[Desmoid_tumor_-_null_regimens#Placebo|Placebo]]
-| style="background-color:#1a9850" |Superior PFS
+| style="background-color:#1a9850" |Superior PFS<br>PFS24: 81% vs 36%<br>(HR 0.13, 95% CI 0.05-0.31)
 |-
 |}
+<div class="toccolours" style="background-color:#b3e2cd">
 ====Targeted therapy====
-*[[Sorafenib (Nexavar)]] 400 mg PO once per day
+*[[Sorafenib (Nexavar)]] 400 mg PO once per day on days 1 to 28
+'''28-day cycles'''
-'''Continued indefinitely'''
+</div></div>
 ===References===
-# '''Alliance A091105:''' Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ, Riedel RF, Sabagh T, Tap WD, Horvat N, Basch E, Schwartz LH, Maki RG, Agaram NP, Lefkowitz RA, Mazaheri Y, Yamashita R, Wright JJ, Dueck AC, Schwartz GK. Sorafenib for Advanced and Refractory Desmoid Tumors. N Engl J Med. 2018 Dec 20;379(25):2417-2428. [https://doi.org/10.1056/NEJMoa1805052 link to original article] '''contains dosing details in abstract''' [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6447029/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/30575484 PubMed] NCT02066181
+# '''Alliance A091105:''' Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ, Riedel RF, Sabagh T, Tap WD, Horvat N, Basch E, Schwartz LH, Maki RG, Agaram NP, Lefkowitz RA, Mazaheri Y, Yamashita R, Wright JJ, Dueck AC, Schwartz GK. Sorafenib for Advanced and Refractory Desmoid Tumors. N Engl J Med. 2018 Dec 20;379(25):2417-2428. [https://doi.org/10.1056/NEJMoa1805052 link to original article] '''contains dosing details in abstract''' [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6447029/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/30575484/ PubMed] [https://clinicaltrials.gov/study/NCT02066181 NCT02066181]
 [[Category:Desmoid tumor regimens]]
 [[Category:Disease-specific pages]]
 [[Category:Soft tissue sarcomas]]

Difference between revisions of "Desmoid tumor"

Latest revision as of 11:23, 13 May 2024

Contents

Guidelines

French Intergroup

NCCN

All lines of therapy

Imatinib monotherapy

Regimen variant #1, fixed dose

Targeted therapy

Regimen variant #2, BSA-based dosing

Targeted therapy

References

Methotrexate & Vinblastine

Regimen

Chemotherapy

References

Nirogacestat monotherapy

Regimen

Targeted therapy

References

Pazopanib monotherapy

Regimen

Targeted therapy

References

Pegylated liposomal doxorubicin monotherapy

Regimen

Chemotherapy

References

Sorafenib monotherapy

Regimen

Targeted therapy

References

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