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Vascular sarcoma

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Last updated on 2024-07-23:TBA regimens on this page
TBA variants on this page
Note: this page does not contain any regimens. The following links will take you to disease-specific pages with regimens: 
*Regimens for Angiosarcoma are here.
*Regimens for Kaposi sarcoma are here.
=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.
==ESMO==
*Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. (2014) EuroPMC
==NCCN==
*NCCN Guidelines - Soft Tissue Sarcoma
=Hemangioendothelioma, all lines of therapy=
=Hemangiopericytoma (solitary fibrous tumor), all lines of therapy=
Category:Vascular sarcoma regimens
Category:Disease-specific pages
Category:Soft tissue sarcomas
Veltuzumab (hA20)

==General information==
Class/mechanism: Humanized monoclonal anti-CD20 antibody, which binds to cells expressing CD20 (human B-lymphocyte-restricted differentiation antigen, Bp35). Veltuzumab has been observed to have apoptotic, anti-proliferative, and antibody-dependent cellular cytotoxic (ADCC) effects.Veltuzumab (hA20) manufacturer's websiteGoldenberg DM, Rossi EA, Stein R, Cardillo TM, Czuczman MS, Hernandez-Ilizaliturri FJ, Hansen HJ, Chang CH. Properties and structure-function relationships of veltuzumab (hA20), a humanized anti-CD20 monoclonal antibody. Blood. 2009 Jan 29;113(5):1062-70. Epub 2008 Oct 21. link to original article link to PMC article EuroPMCMorschhauser F, Leonard JP, Fayad L, Coiffier B, Petillon MO, Coleman M, Schuster SJ, Dyer MJ, Horne H, Teoh N, Wegener WA, Goldenberg DM. Humanized anti-CD20 antibody, veltuzumab, in refractory/recurrent non-Hodgkin's lymphoma: phase I/II results. J Clin Oncol. 2009 Jul 10;27(20):3346-53. Epub 2009 May 18. link to original article EuroPMCGoldenberg DM, Morschhauser F, Wegener WA. Veltuzumab (humanized anti-CD20 monoclonal antibody): characterization, current clinical results, and future prospects. Leuk Lymphoma. 2010 May;51(5):747-55. link to original article EuroPMCNegrea GO, Elstrom R, Allen SL, Rai KR, Abbasi RM, Farber CM, Teoh N, Horne H, Wegener WA, Goldenberg DM. Subcutaneous injections of low-dose veltuzumab (humanized anti-CD20 antibody) are safe and active in patients with indolent non-Hodgkin's lymphoma. Haematologica. 2011 Apr;96(4):567-73. Epub 2010 Dec 20. link to original article EuroPMC
Route: IV
Extravasation: no information
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, or the prescribing information.
==Patient drug information==
No information available.
==Also known as==
*Code name: hA20, IMMU-160
==References==
Category:Drugs
Category:Intravenous medications
Category:Anti-CD20 antibodies
Category:Investigational drugs
Vemurafenib (Zelboraf)

==General information==
Class/mechanism: Tyrosine kinase inhibitor of some mutated forms of BRAF serine/threonine
kinase, including BRAF V600E. Also has been observed to inhibit other tyrosine kinases such as RAF1, ARAF, wild-type BRAF, SRMS, ACK1, MAP4K5, and FGR. Certain BRAF mutations, such as V600E, result in constitutive activation of cell proliferation pathways and tumor growth.Vemurafenib (Zelboraf) package insert Vemurafenib (Zelboraf) package insert (locally hosted backup)Zelboraf manufacturer's website
Route: PO
Extravasation: n/a
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, or the prescribing information.
==Diseases for which it is used==
*BRAF-mutated colorectal cancer
*BRAF-mutated melanoma
*BRAF-mutated tumors (tissue-agnostic)
*Craniopharyngioma
*Erdheim-Chester disease
*Hairy cell leukemia
*Langerhans cell histiocytosis
*Multiple myeloma
*Thyroid cancer
==More drug information==
*DailyMed information
*Vemurafenib (Zelboraf) patient drug information (UpToDate)Vemurafenib (Zelboraf) patient drug information (UpToDate)
==History of changes in FDA indication==
*2011-08-17: Initial FDA approval for the treatment of patients with unresectable or metastatic melanoma with BRAF V600E mutation. (Based on BRIM2, BRIM-3, and MO25743)
** Limitation: vemurafenib is not recommended for use in patients with wild-type BRAF melanoma.
*2017-11-06: Granted regular FDA approval for the treatment of patients with Erdheim-Chester Disease (ECD) with BRAF V600 mutation. (Based on VE-BASKET)
==History of changes in EMA indication==
*2012-02-17: Initial authorization as Zelboraf. Zelboraf is indicated in monotherapy for the treatment of adult patients with BRAF-V600-mutation-positive unresectable or metastatic melanoma. (Based on BRIM-3)
==History of changes in Health Canada indication==
*2012-02-15: Initial notice of compliance
==History of changes in PMDA indication==
*2014-12-26: Initial approval for the treatment of unresectable malignant melanoma with BRAF mutation.
==Also known as==
*Code names: PLX-4032, RG-7204, RO-5185426
*Brand name: Zelboraf
==References==
Category:Drugs
Category:Oral medications
Category:Mutation-specific medications
Category:BRAF inhibitors
Category:MAP2K4 inhibitors
Category:MAP3K20 inhibitors
Category:MAP4K5 inhibitors
Category:RAF1 inhibitors
Category:Malignant solid neoplasm, BRAF-mutated medications
Category:Colorectal cancer medications
Category:Craniopharyngioma medications
Category:Erdheim-Chester disease medications
Category:Hairy cell leukemia medications
Category:Langerhans cell histiocytosis medications
Category:Melanoma medications 
Category:Multiple myeloma medications
Category:Thyroid cancer medications
Category:FDA approved in 2011
Category:EMA approved in 2012
Category:Health Canada approved in 2012
Category:PMDA approved in 2014
Verbrinacogene setparvovec (FLT-180a)

Gene therapy
==Preliminary data==
Note: This is not meant to be an exhaustive list of preliminary results. References included in this section include non-randomized studies published in high-profile journals (e.g., NEJM) and experimental arms of phase 3 RCTs. Inclusion here is not an indication of impending regulatory approval, although many of these drugs do end up receiving approval by a regulatory authority.
===Hemophilia B===
#B-AMAZE: Chowdary P, Shapiro S, Makris M, Evans G, Boyce S, Talks K, Dolan G, Reiss U, Phillips M, Riddell A, Peralta MR, Quaye M, Patch DW, Tuddenham E, Dane A, Watissée M, Long A, Nathwani A. Phase 1-2 Trial of AAVS3 Gene Therapy in Patients with Hemophilia B. N Engl J Med. 2022 Jul 21;387(3):237-247. link to original article PubMed NCT03369444; NCT03641703
==Also known as==
*Code name: FLT-180a
Category:Drugs
Category:Intravenous medications
Category:Inherited coagulopathy medications (investigational)
Category:Investigational drugs
Category:Stub
Vimseltinib (Romvimza)

==Mechanism of action==
From the NCI Drug Dictionary: An orally bioavailable inhibitor of the tyrosine kinase receptor colony stimulating factor 1 receptor (CSF1R; CSF-1R; C-FMS; CD115; M-CSFR), with potential antineoplastic, macrophage checkpoint-inhibitory and immunomodulating activities. Upon administration, vimseltinib targets and binds to CSF1R expressed on monocytes, macrophages, and osteoclasts and inhibits the binding of the CSF1R ligands colony-stimulating factor-1 (CSF-1) and interleukin-34 (IL-34), to CSF1R. This prevents CSF1R activation and CSF1R-mediated signaling in these cells. This blocks the production of inflammatory mediators by macrophages and monocytes and reduces inflammation. By blocking the recruitment to the tumor microenvironment (TME) and activity of CSF1R-dependent tumor-associated macrophages (TAMs), vimseltinib inhibits the immunomodulating activity by macrophages and enhances T-cell infiltration and anti-tumor T-cell immune responses, which inhibits the proliferation of tumor cells.
==Diseases for which it is established==
*Tenosynovial giant cell tumor
==History of changes in FDA indication==
*2025-02-14: Approved for adult patients with symptomatic tenosynovial giant cell tumor (TGCT) for which surgical resection will potentially cause worsening functional limitation or severe morbidity.. (Based on MOTION)
==Also known as==
*Code names: DCC-3014, DP-6865
*Brand name: Romvimza
Category:Drugs
Category:Oral medications
Category:CSF1R inhibitors
Category:Tenosynovial giant cell tumor medications
Category:FDA approved in 2025
Vincristine liposomal (Marqibo)

==General information==
Class/mechanism: Vinca alkaloid which causes cell cycle arrest in metaphase and inhibits mitosis. Vincristine stabilizes the spindle apparatus by binding to tubulin, alters microtubule structure and function, and inhibits chromosome separation. Marqibo is a formulation of vincristine sulfate that is encapsulated in sphingomyelin/cholesterol liposomes (Optisomes), which are hypothesized to provide improved tissue drug penetration and reduced toxicity.Vincristine liposomal (Marqibo) package insertVincristine liposomal (Marqibo) package insert (locally hosted backup)Marqibo manufacturer's websiteJeffrey A. Silverman, Walter Aulitzky, John Lister, Lori Maness, Gary Schiller, Karen Seiter, Scott E. Smith, Wendy Stock, Dina Ben Yehuda, Steven R. Deitcher. Marqibo® (vincristine sulfate liposomes injection; VSLI) Optimizes the Dosing, Delivery, and Pharmacokinetic (PK) Profile of Vincristine Sulfate (VCR) in Adults with Relapsed and Refractory Acute Lymphoblastic Leukemia (ALL). ASH 2010 abstract 2142. link to abstract
Route: IV
Extravasation: irritant/vesicant (depending on reference)
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, or the prescribing information.
==Diseases for which it was used==
*B-cell acute lymphoblastic leukemia
*Diffuse large B-cell lymphoma
==Patient drug information==
*Brief patient counseling information is available in the Vincristine liposomal (Marqibo) package insert.
*Vincristine liposomal (Marqibo) patient drug information (Chemocare)Vincristine liposomal (Marqibo) patient drug information (Chemocare)
==History of changes in FDA indication==
*2012-08-09: Accelerated approval for the treatment of adult patients with Philadelphia chromosome-negative (Ph-) acute lymphoblastic leukemia (ALL) in second or greater relapse or whose disease has progressed following two or more anti-leukemia therapies. (Based on RALLY)
**2022-05-02: Accelerated approval for the treatment of adult patients with Philadelphia chromosome-negative (Ph-) acute lymphoblastic leukemia (ALL) in second or greater relapse or whose disease has progressed following two or more anti-leukemia therapies withdrawn at request of manufacturer (Based on Hallmarq)
==Also known as==
*Generic names: vincristine sulfate liposome injection, VSLI
*Brand name: Cytocristin, Marqibo, Vincihal, Vinlieva, Vinlon
==References==
Category:Drugs
Category:Intravenous medications
Category:Irritant
Category:Vesicant
Category:Liposomal chemotherapy
Category:Microtubule inhibitors
Category:Vinca alkaloids
Category:B-cell acute lymphoblastic leukemia medications (historic)
Category:Diffuse large B-cell lymphoma medications (historic)
Category:FDA approved in 2012
Category:Discontinued drugs
Category:FDA withdrawn in 2022
Vindesine (Eldisine)

Not approved by the FDA. Approved for use in some countries e.g., U.K., South Africa.
==General information==
Class/mechanism: Vinca alkaloid, inhibits microtubule formation in the mitotic spindle, causing cell cycle arrest in metaphase.Vindesine (Eldisine) package insert Vindesine (Eldisine) package insert (locally hosted backup)
Route: IV
Extravasation: vesicant
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, or the prescribing information. 
==Diseases for which it is used==
*Adult T-cell leukemia-lymphoma
*Burkitt lymphoma
*CNS lymphoma
*Diffuse large B-cell lymphoma
*HIV-associated lymphoma
*Melanoma
*Neuroblastoma
*Non-small cell lung cancer
*Post-transplant lymphoproliferative disorder
==Diseases for which it was used==
*Acute myeloid leukemia
*Small cell lung cancer
==Patient drug information==
*Vindesine (Eldisine) patient information at Macmillan Cancer SupportVindesine (Eldisine) patient information at Macmillan Cancer Support 
*Vindesine (Eldisine) patient information at Genus PharmaceuticalsVindesine (Eldisine) patient information at Genus Pharmaceuticals
*Vindesine (Eldisine) patient drug information (UpToDate)Vindesine (Eldisine) patient drug information (UpToDate)
==History of changes in EMA indication==
*1979-04-11: EURD
==Also known as==
*Code name: LY-099094
*Generic names: desacetylvinblastine amide, DAVA, DVA, VDS
*Brand names: Eldesine, Eldisin, Eldisine, Enison, Fildesin, Gesidine
==References==
Category:Drugs
Category:Intravenous medications
Category:Vesicant
Category:Microtubule inhibitors
Category:Vinca alkaloids
Category:Adult T-cell leukemia-lymphoma medications
Category:Burkitt lymphoma medications
Category:CNS lymphoma medications
Category:Diffuse large B-cell lymphoma medications
Category:HIV-associated lymphoma medications
Category:Melanoma medications
Category:Neuroblastoma medications
Category:Non-small cell lung cancer medications
Category:Post-transplant lymphoproliferative disorder medications
Category:Acute myeloid leukemia medications (historic)
Category:Small cell lung cancer medications (historic)
Category:EMA approved in 1979
Vinflunine (Javlor)

==Mechanism of action==
From the NCI drug dictionary: A bi-fluorinated derivative of the semi-synthetic vinca alkaloid vinorelbine with antitubulin, antineoplastic, and antiangiogenic activities. Vinflunine inhibits tubulin assembly without any stablization of assembled microtubules at concentrations comparable to those of other vinca alkaloids such as vincristine, vinblastine and vinorelbine; this effect on microtubule dynamics results in cell cycle arrest in mitosis and apoptosis. Compared to other vinca alkaloids, this agent binds weakly to the vinca-binding site, indicating that vinflunine may exhibit reduced neurotoxicity.
==Diseases for which it is used==
*Breast cancer
*Non-small cell lung cancer
*Urothelial carcinoma
==History of changes in EMA indication==
*2009-09-21: Initial marketing authorization at Javlor.
==Also known as==
*Brand name: Javlor
Category:Drugs
Category:Intravenous medications
Category:Vesicant
Category:Microtubule inhibitors
Category:Vinca alkaloids
Category:Breast cancer medications
Category:Non-small cell lung cancer medications
Category:Urothelial carcinoma medications
Category:EMA approved in 2009
Vinorelbine (Navelbine)

==General information==
Class/mechanism: Vinca alkaloid, inhibits microtubule formation in the mitotic spindle, causing cell cycle arrest in metaphase. Vinorelbine may possibly also disrupt: amino acid, cyclic AMP, and glutathione metabolism; calmodulin-dependent calcium transport ATPase activity; and DNA/RNA and lipid synthesis.Vinorelbine (Navelbine) package insertVinorelbine (Navelbine) package insert (locally hosted backup)
Route: IV
Extravasation: vesicant
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, or the prescribing information. 
==Disease for which it is used==
*Breast cancer
*Cervical cancer
*Diffuse large B-cell lymphoma
*Esophageal cancer
**Esophageal squamous cell carcinoma
*Classical Hodgkin lymphoma
*Malignant pleural mesothelioma
*Non-small cell lung cancer
**Non-small cell lung cancer, nonsquamous
*Ovarian cancer
*Soft tissue sarcoma
*Small cell lung cancer
==Patient drug information==
*Vinorelbine (Navelbine) patient drug information (Chemocare)Vinorelbine (Navelbine) patient drug information (Chemocare)
*Vinorelbine (Navelbine) patient drug information (UpToDate)Vinorelbine (Navelbine) patient drug information (UpToDate)
==History of changes in FDA indication==
* 1994-12-23: Initial FDA approval as a single agent or in combination with cisplatin for the first-line treatment of ambulatory patients with unresectable, advanced non-small cell lung cancer (NSCLC). (Based on Crawford et al. 1996, Le Chevalier et al. 1994, SWOG S9308)
==History of changes in EMA indication==
*1989-04-11: EURD
==History of changes in PMDA indication==
*2005-05-31: New indication for the treatment of inoperable or recurrent breast cancer.
==Also known as==
*Code name: KW-2307
*Generic name: NVB, vinorelbine tartrate
*Brand names: Binorel, Biovelbin, Eunades, Flonorbin, Navelbine, Neoben, Relbovin, Vinelbine, Vinorelbel, Vinotec
==References==
Category:Drugs
Category:Intravenous medications
Category:Vesicant
Category:Microtubule inhibitors
Category:Vinca alkaloids
Category:Breast cancer medications
Category:Cervical cancer medications
Category:Diffuse large B-cell lymphoma medications
Category:Esophageal cancer medications
Category:Esophageal squamous cell carcinoma medications
Category:Classical Hodgkin lymphoma medications
Category:Malignant pleural mesothelioma medications
Category:Non-small cell lung cancer medications
Category:Non-small cell lung cancer, nonsquamous medications
Category:Ovarian cancer medications
Category:Soft tissue sarcoma medications 
Category:Small cell lung cancer medications
Category:FDA approved in 1994
Category:EMA approved in 1989
Category:WHO Essential Cancer Medicine
Vismodegib (Erivedge)

==General information==
Class/mechanism: Hedgehog pathway inhibitor. Vismodegib binds to and inhibits the transmembrane protein Smoothened (SMO), which is part of the Hedgehog signal transduction pathway that is clinically relevant in diseases such as basal cell cancer.Vismodegib (Erivedge) package insert Vismodegib (Erivedge) package insert (locally hosted backup)Erivedge manufacturer's website
Route: PO
Extravasation: n/a
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, or the prescribing information.
==Diseases for which it is used==
*Cutaneous basal cell carcinoma
*Hedgehog pathway-mutated tumors (tissue-agnostic)
==Patient drug information==
*Erivedge manufacturer side effect tipsErivedge manufacturer side effect tips
*Vismodegib (Erivedge) patient drug information (UpToDate)Vismodegib (Erivedge) patient drug information (UpToDate)
==History of changes in FDA indication==
*2012-01-30: Initial FDA approval for the treatment of adults with metastatic basal cell carcinoma. (Based on ERIVANCE BCC)
*2012-01-30: Initial FDA approval for the treatment of adults with locally advanced basal cell carcinoma that has recurred following surgery. (Based on ERIVANCE BCC)
*2012-01-30: Initial FDA approval for the treatment of adults with locally advanced basal cell carcinoma who are not candidates for surgery, and who are not candidates for radiation. (Based on ERIVANCE BCC)
==History of changes in EMA indication==
*2013-04-26: Initial authorization
==History of changes in Health Canada indication==
*2013-07-12: Initial notice of compliance
==Also known as==
*Code name: GDC-0449
*Brand name: Erivedge
==References==
Category:Drugs
Category:Oral medications
Category:Hedgehog pathway inhibitors
Category:SMO inhibitors
Category:Cutaneous basal cell carcinoma medications
Category:Malignant solid neoplasm, SMO-mutated or PTCH-1-mutated (Hedgehog) medications
Category:FDA approved in 2012
Category:EMA approved in 2013
Category:Health Canada approved in 2013
Volasertib (BI-6727)

==Mechanism of action==
Polo-like kinase 1 (PLK1) inhibitor
==Preliminary data==
===Acute myeloid leukemia===
# BI 1230.4: Döhner H, Lübbert M, Fiedler W, Fouillard L, Haaland A, Brandwein JM, Lepretre S, Reman O, Turlure P, Ottmann OG, Müller-Tidow C, Krämer A, Raffoux E, Döhner K, Schlenk RF, Voss F, Taube T, Fritsch H, Maertens J. Randomized, phase 2 trial comparing low-dose cytarabine with or without volasertib in AML patients not suitable for intensive induction therapy. Blood. 2014 Aug 28;124(9):1426-33. Epub 2014 Jul 8. link to original article link to PMC article PubMed NCT00804856
==Also known as==
*Code name: BI-6727
Category:Drugs
Category:Intravenous medications
Category:PLK1 inhibitors
Category:Acute myeloid leukemia medications (investigational)
Category:Investigational drugs
Von Willebrand factor and factor VIII complex, human

==General information==
Class/mechanism: Von Willebrand Factor (vWF) and coagulation factor VIII complex; allows for improved platelet function & coagulation in patients with von Willebrand Disease (vWD) and/or hemophilia A. VWF mediates binding between platelets and damaged endothelial tissue in addition to functioning as a carrier protein for factor VIII, which is a participant in the coagulation cascade.Please choose from the products below to find specific drug/manufacturer information: 
*Alphanate (human)Von Willebrand Factor and factor VIII complex, human (Alphanate) package insertVon Willebrand Factor and factor VIII complex, human (Alphanate) (locally hosted backup)
*Humate-P (human)Humate-P manufacturer's websiteVon Willebrand Factor and factor VIII complex, human (Humate-P) package insertVon Willebrand Factor and factor VIII complex, human (Humate-P) (locally hosted backup)
*Wilate (human)Wilate manufacturer's websiteVon Willebrand Factor and factor VIII complex, human (Wilate) package insertVon Willebrand Factor and factor VIII complex, human (Wilate) (locally hosted backup)
Route: IV
Extravasation: no information
For conciseness and simplicity, HemOnc.org currently will focus on treatment regimens and not list information such as: renal/hepatic dose adjustments, metabolism (including CYP450), excretion, monitoring parameters (although this will be considered for checklists), or manufacturer. Instead, for the most current information, please refer to your preferred pharmacopeias such as Micromedex, Lexicomp, UpToDate (courtesy of Lexicomp), or the prescribing information.
==Patient drug information==
*Von Willebrand Factor and factor VIII complex, human patient drug information (UpToDate)Von Willebrand Factor and factor VIII complex, human patient drug information (UpToDate)
==Also known as==
*Generic name: antihemophilic factor/von Willebrand Factor complex
*Brand names: Alphanate, Humate-P, Wilate, Wilfactin
==References==
Category:Fractionated plasma products
Category:Intravenous medications
Category:Inherited coagulopathy medications
Category:Hematology medications
Category:Coagulation factors