Vascular sarcoma

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James L. Chen, MD, MS
The Ohio State University
Columbus, OH

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10 regimens on this page
12 variants on this page


Guidelines

ESMO

NCCN

Angiosarcoma, all lines of therapy

Bevacizumab monotherapy

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Regimen

Study Evidence
Agulnik et al. 2013 Phase II

Chemotherapy

21-day cycles

References

  1. Agulnik M, Yarber JL, Okuno SH, von Mehren M, Jovanovic BD, Brockstein BE, Evens AM, Benjamin RS. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013 Jan;24(1):257-63. link to original article contains verified protocol PubMed

Paclitaxel monotherapy

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Regimen

Study Evidence
Penel et al. 2008 (ANGIOTAX) Phase II

Chemotherapy

Supportive medications

28-day cycle for 6 cycles

References

  1. ANGIOTAX: Penel N, Bui BN, Bay JO, Cupissol D, Ray-Coquard I, Piperno-Neumann S, Kerbrat P, Fournier C, Taieb S, Jimenez M, Isambert N, Peyrade F, Chevreau C, Bompas E, Brain EG, Blay JY. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study. J Clin Oncol. 2008 Nov 10;26(32):5269-74. Epub 2008 Sep 22. link to original article contains verified protocol PubMed

Hemangioendothelioma, all lines of therapy

Hemangiopericytoma (solitary fibrous tumor), all lines of therapy

Kaposi sarcoma, all lines of therapy

ABV (Vinblastine)

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ABV: Adriamycin (Doxorubicin), Bleomycin, Vinblastine

Regimen

Study Evidence
Laubenstein et al. 1984 Non-randomized

Chemotherapy

References

  1. Laubenstein LJ, Krigel RL, Odajnyk CM, Hymes KB, Friedman-Kien A, Wernz JC, Muggia FM. Treatment of epidemic Kaposi's sarcoma with etoposide or a combination of doxorubicin, bleomycin, and vinblastine. J Clin Oncol. 1984 Oct;2(10):1115-20. link to original article PubMed

ABV (Vincristine)

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ABV: Adriamycin (Doxorubicin), Bleomycin, Vincristine

Variant #1, 10/15/1

Study Evidence Comparator Comparative Efficacy
Gill et al. 1996 Phase III (C) Liposomal daunorubicin Seems not superior

Gill et al. 1996 described doses but did not clearly say in the paper when these drugs were given, but this schedule is assumed based on the Northfelt et al. 1998 ABV regimen. To our knowledge, this regimen was not tested as an experimental arm in an RCT prior to becoming a standard comparator arm.

Chemotherapy

Supportive medications

  • "No routine premedication was established by the protocol, but it could be provided at the discretion of the investigator"

14-day cycle for at least 2 cycles until complete remission

Variant #2, 20/10/1

Study Evidence Comparator Comparative Efficacy
Northfelt et al. 1998 Phase III (C) Pegylated liposomal doxorubicin Inferior ORR

Note: to our knowledge, this regimen was not tested as an experimental arm in an RCT prior to becoming a standard comparator arm.

Chemotherapy

Supportive medications

  • "Colony-stimulating factors (CSFs) were prescribed at the discretion of the investigators."

14-day cycle for up to 6 cycles

References

  1. Gill PS, Wernz J, Scadden DT, Cohen P, Mukwaya GM, von Roenn JH, Jacobs M, Kempin S, Silverberg I, Gonzales G, Rarick MU, Myers AM, Shepherd F, Sawka C, Pike MC, Ross ME. Randomized phase III trial of liposomal daunorubicin versus doxorubicin, bleomycin, and vincristine in AIDS-related Kaposi's sarcoma. J Clin Oncol. 1996 Aug;14(8):2353-64. link to original article contains verified protocol PubMed
  2. Northfelt DW, Dezube BJ, Thommes JA, Miller BJ, Fischl MA, Friedman-Kien A, Kaplan LD, Du Mond C, Mamelok RD, Henry DH. Pegylated-liposomal doxorubicin versus doxorubicin, bleomycin, and vincristine in the treatment of AIDS-related Kaposi's sarcoma: results of a randomized phase III clinical trial. J Clin Oncol. 1998 Jul;16(7):2445-51. link to original article contains verified protocol PubMed

Bevacizumab monotherapy

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Regimen

Study Evidence
Uldrick et al. 2012 Phase II

Chemotherapy

  • Bevacizumab (Avastin) as follows:
    • Loading: 15 mg/kg IV once as a loading dose; start regular therapy 7 days later after this loading dose
    • Cycle 1 onwards: 15 mg/kg IV once on day 1

Supportive medications

  • "Antihypertensive therapy was initiated for systolic blood pressure greater than 160 mmHg or diastolic blood pressure greater than 95 mmHg persisting for more than 1 week or for systolic blood pressure greater than 210 mmHg or diastolic blood pressure greater than 120 mmHg at any time."
  • "HIV-positive patients with CD4 count of less than 200 cells/µL received Pneumocystis jiroveci prophylaxis."
  • "Mycobacterium avium prophylaxis was considered if CD4 count was less than 75 cells/µL."
  • Patients with HIV/AIDS continued HAART
  • Filgrastim (Neupogen) "used as clinically indicated"

21-day cycles

References

  1. Uldrick TS, Wyvill KM, Kumar P, O'Mahony D, Bernstein W, Aleman K, Polizzotto MN, Steinberg SM, Pittaluga S, Marshall V, Whitby D, Little RF, Yarchoan R. Phase II study of bevacizumab in patients with HIV-associated Kaposi's sarcoma receiving antiretroviral therapy. J Clin Oncol. 2012 May 1;30(13):1476-83. Epub 2012 Mar 19. link to original article contains verified protocol link to PMC article PubMed

Daunorubicin liposomal monotherapy

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Regimen

Study Evidence Comparator Comparative Efficacy
Presant et al. 1993 Phase II
Gill et al. 1996 Phase III (E) ABV Seems not superior

Note: Gill et al. 1996 stopped treatment if CR was achieved.

Chemotherapy

Supportive medications

  • "No routine premedication was established by the protocol, but it could be provided at the discretion of the investigator"

14-day cycles

References

  1. Presant CA, Scolaro M, Kennedy P, Blayney DW, Flanagan B, Lisak J, Presant J. Liposomal daunorubicin treatment of HIV-associated Kaposi's sarcoma. Lancet. 1993 May 15;341(8855):1242-3. link to original article PubMed
  2. Gill PS, Wernz J, Scadden DT, Cohen P, Mukwaya GM, von Roenn JH, Jacobs M, Kempin S, Silverberg I, Gonzales G, Rarick MU, Myers AM, Shepherd F, Sawka C, Pike MC, Ross ME. Randomized phase III trial of liposomal daunorubicin versus doxorubicin, bleomycin, and vincristine in AIDS-related Kaposi's sarcoma. J Clin Oncol. 1996 Aug;14(8):2353-64. link to original article contains verified protocol PubMed

Doxorubicin pegylated liposomal monotherapy

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Regimen

Study Evidence Comparator Comparative Efficacy
Stewart et al. 1998 Phase III (E) BV Superior ORR
Northfelt et al. 1998 Phase III (E) ABV Superior ORR

Chemotherapy

Supportive medications

  • "Colony-stimulating factors (CSFs) were prescribed at the discretion of the investigators."

14-day cycle for up to 6 cycles

References

  1. Stewart S, Jablonowski H, Goebel FD, Arasteh K, Spittle M, Rios A, Aboulafia D, Galleshaw J, Dezube BJ; International Pegylated Liposomal Doxorubicin Study Group. Randomized comparative trial of pegylated liposomal doxorubicin versus bleomycin and vincristine in the treatment of AIDS-related Kaposi's sarcoma. J Clin Oncol. 1998 Feb;16(2):683-91. link to original article contains protocol PubMed
  2. Northfelt DW, Dezube BJ, Thommes JA, Miller BJ, Fischl MA, Friedman-Kien A, Kaplan LD, Du Mond C, Mamelok RD, Henry DH. Pegylated-liposomal doxorubicin versus doxorubicin, bleomycin, and vincristine in the treatment of AIDS-related Kaposi's sarcoma: results of a randomized phase III clinical trial. J Clin Oncol. 1998 Jul;16(7):2445-51. link to original article contains verified protocol PubMed

Etoposide monotherapy

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Variant #1, 8 cycles

Study Evidence Comparator Comparative Efficacy
Hosseinipour et al. 2018 (REACT-KS) Phase III (C) As-needed Etoposide Seems not superior

Chemotherapy

14-day cycle for up to 8 cycles

Variant #2, indefinite

Study Evidence
Evans et al. 2002 Phase II

Chemotherapy

14-day cycles

References

  1. Evans SR, Krown SE, Testa MA, Cooley TP, Von Roenn JH. Phase II evaluation of low-dose oral etoposide for the treatment of relapsed or progressive AIDS-related Kaposi's sarcoma: an AIDS Clinical Trials Group clinical study. J Clin Oncol. 2002 Aug 1;20(15):3236-41. link to original article contains verified protocol PubMed
  2. REACT-KS: Hosseinipour MC, Kang M, Krown SE, Bukuru A, Umbleja T, Martin JN, Orem J, Godfrey C, Hoagland B, Mwelase N, Langat D, Nyirenda M, MacRae J, Borok M, Samaneka W, Moses A, Mngqbisa R, Busakhala N, Martínez-Maza O, Ambinder R, Dittmer DP, Nokta M, Campbell TB; A5264/AMC-067 REACT-KS Team. As-needed vs immediate etoposide chemotherapy in combination with antiretroviral therapy for mild-to-moderate AIDS-associated Kaposi sarcoma in resource-limited settings: A5264/AMC-067 randomized clinical trial. Clin Infect Dis. 2018 Jul 2;67(2):251-260. link to original article PubMed

Interferon alfa monotherapy

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Regimen

Study Evidence
de Wit et al. 1988 Phase II
Lane et al. 1988 Non-randomized

Immunotherapy

References

  1. de Wit R, Schattenkerk JK, Boucher CA, Bakker PJ, Veenhof KH, Danner SA. Clinical and virological effects of high-dose recombinant interferon-alpha in disseminated AIDS-related Kaposi's sarcoma. Lancet. 1988 Nov 26;2(8622):1214-7. link to original article PubMed
  2. Lane HC, Kovacs JA, Feinberg J, Herpin B, Davey V, Walker R, Deyton L, Metcalf JA, Baseler M, Salzman N, Quinnan G, Fauci AS. Anti-retroviral effects of interferon-alpha in AIDS-associated Kaposi's sarcoma. Lancet. 1988 Nov 26;2(8622):1218-22. link to original article PubMed

Paclitaxel monotherapy

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Regimen

Study Evidence
Saville et al. 1995 Phase II

Chemotherapy

21-day cycles

References

  1. Saville MW, Lietzau J, Pluda JM, Feuerstein I, Odom J, Wilson WH, Humphrey RW, Feigal E, Steinberg SM, Broder S, Yarchoan R. Treatment of HIV-associated Kaposi's sarcoma with paclitaxel. Lancet. 1995 Jul 1;346(8966):26-8. link to original article contains protocol PubMed

Placebo

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Regimen

Study Evidence Comparator Comparative Efficacy
Noy et al. 2005 Phase III (C) IM862 Seems to have superior TTP

No active antineoplastic treatment.

References

  1. Noy A, Scadden DT, Lee J, Dezube BJ, Aboulafia D, Tulpule A, Walmsley S, Gill P. Angiogenesis inhibitor IM862 is ineffective against AIDS-Kaposi's sarcoma in a phase III trial, but demonstrates sustained, potent effect of highly active antiretroviral therapy: from the AIDS Malignancy Consortium and IM862 Study Team. J Clin Oncol. 2005 Feb 10;23(5):990-8. Epub 2004 Dec 14. link to original article PubMed