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Kaposi Sarcoma NCI Grade 3

MedGen UID:
235241
•Concept ID:
C1334321
•
Neoplastic Process

Definition

A Kaposi sarcoma with more than 15% necrosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKaposi Sarcoma NCI Grade 3

Professional guidelines

PubMed

Effect of CD4+ T cell count on treatment-emergent adverse events among patients with and without HIV receiving immunotherapy for advanced cancer.
Odeny TA, Lurain K, Strauss J, Fling SP, Sharon E, Wright A, Martinez-Picado J, Moran T, Gulley JL, Gonzalez-Cao M, Uldrick TS, Yarchoan R, Ramaswami R
J Immunother Cancer 2022 Sep;10(9) doi: 10.1136/jitc-2022-005128. PMID: 37935055Free PMC Article

Recent clinical studies

Etiology

Cancer Immunotherapy Trials Network 12: Pembrolizumab in HIV-Associated Kaposi Sarcoma.
Lurain K, Ramaswami R, Ekwede I, Eulo V, Goyal G, Menon M, Odeny TA, Sharon E, Wagner MJ, Wang CJ, Bhardwaj N, Friedlander PA, Abdul-Hay M, Cornejo Castro EM, Labo N, Marshall VA, Miley W, Moore K, Roshan R, Whitby D, Kask AS, Kaiser J, Han E, Wright A, Yarchoan R, Fling SP, Uldrick TS
J Clin Oncol 2025 Feb;43(4):432-442. Epub 2024 Oct 2 doi: 10.1200/JCO.24.00640. PMID: 39356983
Safety, Activity, and Long-term Outcomes of Pomalidomide in the Treatment of Kaposi Sarcoma among Individuals with or without HIV Infection.
Ramaswami R, Polizzotto MN, Lurain K, Wyvill KM, Widell A, George J, Goncalves P, Steinberg SM, Whitby D, Uldrick TS, Yarchoan R
Clin Cancer Res 2022 Mar 1;28(5):840-850. doi: 10.1158/1078-0432.CCR-21-3364. PMID: 34862247Free PMC Article
Use of pembrolizumab with or without pomalidomide in HIV-associated non-Hodgkin's lymphoma.
Lurain K, Ramaswami R, Mangusan R, Widell A, Ekwede I, George J, Ambinder R, Cheever M, Gulley JL, Goncalves PH, Wang HW, Uldrick TS, Yarchoan R
J Immunother Cancer 2021 Feb;9(2) doi: 10.1136/jitc-2020-002097. PMID: 33608378Free PMC Article
Clinical Features and Outcomes of Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV)-associated Inflammation: Prospective Characterization of KSHV Inflammatory Cytokine Syndrome (KICS).
Polizzotto MN, Uldrick TS, Wyvill KM, Aleman K, Marshall V, Wang V, Whitby D, Pittaluga S, Jaffe ES, Millo C, Tosato G, Little RF, Steinberg SM, Sereti I, Yarchoan R
Clin Infect Dis 2016 Mar 15;62(6):730-738. Epub 2015 Dec 12 doi: 10.1093/cid/civ996. PMID: 26658701Free PMC Article
Excellent clinical outcomes and retention in care for adults with HIV-associated Kaposi sarcoma treated with systemic chemotherapy and integrated antiretroviral therapy in rural Malawi.
Herce ME, Kalanga N, Wroe EB, Keck JW, Chingoli F, Tengatenga L, Gopal S, Phiri A, Mailosi B, Bazile J, Beste JA, Elmore SN, Crocker JT, Rigodon J
J Int AIDS Soc 2015;18(1):19929. Epub 2015 May 29 doi: 10.7448/IAS.18.1.19929. PMID: 26028156Free PMC Article

Diagnosis

Brentuximab vedotin with AVD for stage II-IV HIV-related Hodgkin lymphoma (AMC 085): phase 2 results from an open-label, single arm, multicentre phase 1/2 trial.
Rubinstein PG, Moore PC, Bimali M, Lee JY, Rudek MA, Chadburn A, Ratner L, Henry DH, Cesarman E, DeMarco CE, Costagliola D, Taoufik Y, Ramos JC, Sharon E, Reid EG, Ambinder RF, Mitsuyasu R, Mounier N, Besson C, Noy A; AIDS Malignancy Consortium; Lymphoma Study Association
Lancet Haematol 2023 Aug;10(8):e624-e632. doi: 10.1016/S2352-3026(23)00157-6. PMID: 37532416Free PMC Article
Clinical Features and Outcomes of Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV)-associated Inflammation: Prospective Characterization of KSHV Inflammatory Cytokine Syndrome (KICS).
Polizzotto MN, Uldrick TS, Wyvill KM, Aleman K, Marshall V, Wang V, Whitby D, Pittaluga S, Jaffe ES, Millo C, Tosato G, Little RF, Steinberg SM, Sereti I, Yarchoan R
Clin Infect Dis 2016 Mar 15;62(6):730-738. Epub 2015 Dec 12 doi: 10.1093/cid/civ996. PMID: 26658701Free PMC Article
Phase II study of bevacizumab in patients with HIV-associated Kaposi's sarcoma receiving antiretroviral therapy.
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
J Clin Oncol 2012 May 1;30(13):1476-83. Epub 2012 Mar 19 doi: 10.1200/JCO.2011.39.6853. PMID: 22430271Free PMC Article
Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia.
Ragni MV, Belle SH, Jaffe RA, Duerstein SL, Bass DC, McMillan CW, Lovrien EW, Aledort LM, Kisker CT, Stabler SP
Blood 1993 Apr 1;81(7):1889-97. PMID: 8461474
High-grade non-Hodgkin's lymphoma in patients with AIDS.
Ziegler JL, Bragg K, Abrams D, Beckstead J, Cogan M, Volberding P, Baer D, Wilkinson L, Rosenbaum E, Grant K
Ann N Y Acad Sci 1984;437:412-9. doi: 10.1111/j.1749-6632.1984.tb37161.x. PMID: 6598309

Therapy

Cancer Immunotherapy Trials Network 12: Pembrolizumab in HIV-Associated Kaposi Sarcoma.
Lurain K, Ramaswami R, Ekwede I, Eulo V, Goyal G, Menon M, Odeny TA, Sharon E, Wagner MJ, Wang CJ, Bhardwaj N, Friedlander PA, Abdul-Hay M, Cornejo Castro EM, Labo N, Marshall VA, Miley W, Moore K, Roshan R, Whitby D, Kask AS, Kaiser J, Han E, Wright A, Yarchoan R, Fling SP, Uldrick TS
J Clin Oncol 2025 Feb;43(4):432-442. Epub 2024 Oct 2 doi: 10.1200/JCO.24.00640. PMID: 39356983
Brentuximab vedotin with AVD for stage II-IV HIV-related Hodgkin lymphoma (AMC 085): phase 2 results from an open-label, single arm, multicentre phase 1/2 trial.
Rubinstein PG, Moore PC, Bimali M, Lee JY, Rudek MA, Chadburn A, Ratner L, Henry DH, Cesarman E, DeMarco CE, Costagliola D, Taoufik Y, Ramos JC, Sharon E, Reid EG, Ambinder RF, Mitsuyasu R, Mounier N, Besson C, Noy A; AIDS Malignancy Consortium; Lymphoma Study Association
Lancet Haematol 2023 Aug;10(8):e624-e632. doi: 10.1016/S2352-3026(23)00157-6. PMID: 37532416Free PMC Article
Safety, Activity, and Long-term Outcomes of Pomalidomide in the Treatment of Kaposi Sarcoma among Individuals with or without HIV Infection.
Ramaswami R, Polizzotto MN, Lurain K, Wyvill KM, Widell A, George J, Goncalves P, Steinberg SM, Whitby D, Uldrick TS, Yarchoan R
Clin Cancer Res 2022 Mar 1;28(5):840-850. doi: 10.1158/1078-0432.CCR-21-3364. PMID: 34862247Free PMC Article
Use of pembrolizumab with or without pomalidomide in HIV-associated non-Hodgkin's lymphoma.
Lurain K, Ramaswami R, Mangusan R, Widell A, Ekwede I, George J, Ambinder R, Cheever M, Gulley JL, Goncalves PH, Wang HW, Uldrick TS, Yarchoan R
J Immunother Cancer 2021 Feb;9(2) doi: 10.1136/jitc-2020-002097. PMID: 33608378Free PMC Article
Pomalidomide for Symptomatic Kaposi's Sarcoma in People With and Without HIV Infection: A Phase I/II Study.
Polizzotto MN, Uldrick TS, Wyvill KM, Aleman K, Peer CJ, Bevans M, Sereti I, Maldarelli F, Whitby D, Marshall V, Goncalves PH, Khetani V, Figg WD, Steinberg SM, Zeldis JB, Yarchoan R
J Clin Oncol 2016 Dec;34(34):4125-4131. Epub 2016 Oct 31 doi: 10.1200/JCO.2016.69.3812. PMID: 27863194Free PMC Article

Prognosis

Safety, Activity, and Long-term Outcomes of Pomalidomide in the Treatment of Kaposi Sarcoma among Individuals with or without HIV Infection.
Ramaswami R, Polizzotto MN, Lurain K, Wyvill KM, Widell A, George J, Goncalves P, Steinberg SM, Whitby D, Uldrick TS, Yarchoan R
Clin Cancer Res 2022 Mar 1;28(5):840-850. doi: 10.1158/1078-0432.CCR-21-3364. PMID: 34862247Free PMC Article
A Phase Ib Study of Sorafenib (BAY 43-9006) in Patients with Kaposi Sarcoma.
Uldrick TS, Gonçalves PH, Wyvill KM, Peer CJ, Bernstein W, Aleman K, Polizzotto MN, Venzon D, Steinberg SM, Marshall V, Whitby D, Little RF, Wright JJ, Rudek MA, Figg WD, Yarchoan R
Oncologist 2017 May;22(5):505-e49. Epub 2017 Mar 24 doi: 10.1634/theoncologist.2016-0486. PMID: 28341759Free PMC Article
Phase 1/2 trial of BMS-275291 in patients with human immunodeficiency virus-related Kaposi sarcoma: a multicenter trial of the AIDS Malignancy Consortium.
Brinker BT, Krown SE, Lee JY, Cesarman E, Chadburn A, Kaplan LD, Henry DH, Von Roenn JH
Cancer 2008 Mar 1;112(5):1083-8. doi: 10.1002/cncr.23108. PMID: 18224669
Fractionated doses of oral etoposide in the treatment of patients with aids-related kaposi sarcoma: a clinical and pharmacologic study to improve therapeutic index.
Sprinz E, Caldas AP, Mans DR, Cancela A, DiLeone L, Dalla Costa T, Schwartsmann G
Am J Clin Oncol 2001 Apr;24(2):177-84. doi: 10.1097/00000421-200104000-00016. PMID: 11319295
High-grade non-Hodgkin's lymphoma in patients with AIDS.
Ziegler JL, Bragg K, Abrams D, Beckstead J, Cogan M, Volberding P, Baer D, Wilkinson L, Rosenbaum E, Grant K
Ann N Y Acad Sci 1984;437:412-9. doi: 10.1111/j.1749-6632.1984.tb37161.x. PMID: 6598309

Clinical prediction guides

HIV-associated cancers and lymphoproliferative disorders caused by Kaposi sarcoma herpesvirus and Epstein-Barr virus.
Lurain KA, Ramaswami R, Krug LT, Whitby D, Ziegelbauer JM, Wang H-W, Yarchoan R
Clin Microbiol Rev 2024 Sep 12;37(3):e0002223. Epub 2024 Jun 20 doi: 10.1128/cmr.00022-23. PMID: 38899877Free PMC Article
Assessment of the safety of nivolumab in people living with HIV with advanced cancer on antiretroviral therapy: the AIDS Malignancy Consortium 095 Study.
Rajdev L, Jackie Wang CC, Joshi H, Lensing S, Lee J, Ramos JC, Baiocchi R, Ratner L, Rubinstein PG, Ambinder R, Henry D, Streicher H, Little RF, Chiao E, Dittmer DP, Einstein MH, Cesarman E, Mitsuyasu R, Sparano JA; AIDS Malignancy Consortium
Cancer 2024 Mar 15;130(6):985-994. Epub 2023 Nov 14 doi: 10.1002/cncr.35110. PMID: 37962072Free PMC Article
A Phase Ib Study of Sorafenib (BAY 43-9006) in Patients with Kaposi Sarcoma.
Uldrick TS, Gonçalves PH, Wyvill KM, Peer CJ, Bernstein W, Aleman K, Polizzotto MN, Venzon D, Steinberg SM, Marshall V, Whitby D, Little RF, Wright JJ, Rudek MA, Figg WD, Yarchoan R
Oncologist 2017 May;22(5):505-e49. Epub 2017 Mar 24 doi: 10.1634/theoncologist.2016-0486. PMID: 28341759Free PMC Article
Phase II study of bevacizumab in patients with HIV-associated Kaposi's sarcoma receiving antiretroviral therapy.
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
J Clin Oncol 2012 May 1;30(13):1476-83. Epub 2012 Mar 19 doi: 10.1200/JCO.2011.39.6853. PMID: 22430271Free PMC Article
Acquired immunodeficiency syndrome-associated non-Hodgkin's lymphomas and other malignancies in patients with hemophilia.
Ragni MV, Belle SH, Jaffe RA, Duerstein SL, Bass DC, McMillan CW, Lovrien EW, Aledort LM, Kisker CT, Stabler SP
Blood 1993 Apr 1;81(7):1889-97. PMID: 8461474

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