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Kaposi sarcoma

MedGen UID:
11321
Concept ID:
C0036220
Neoplastic Process
Synonyms: Kaposi's sarcoma; Kaposi's sarcoma (disease); Mediterranean Kaposi sarcoma
SNOMED CT: KS - Kaposi's sarcoma (109385007); Kaposi's sarcoma (clinical) (109385007); Kaposi's sarcoma (109385007); Kaposi sarcoma (109385007); Multiple hemorrhagic sarcoma (1179374007); Kaposi's sarcoma (1179374007); Kaposi sarcoma (1179374007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related gene: IL6
 
HPO: HP:0100726
Monarch Initiative: MONDO:0005055
Orphanet: ORPHA33276

Definition

Kaposi sarcoma (KS) is an invasive angioproliferative inflammatory condition that occurs commonly in men infected with human immunodeficiency virus (HIV; see 609423). In the early stages of KS, lesions appear reactive and are stimulated to grow by the actions of inflammatory cytokines and growth factors. In the late stages of KS, a malignant phenotype that appears to be monoclonal can develop. Infection with human herpesvirus-8 (HHV-8), also known as KS-associated herpesvirus (KSHV), is necessary but not sufficient for KS development. Coinfection with HIV markedly increases the likelihood of KS development, and additional environmental, hormonal, and genetic cofactors likely contribute to its pathogenesis (summary by Foster et al., 2000). Suthaus et al. (2012) noted that HHV-8 is the etiologic agent not only of KS, but also of primary effusion lymphoma and plasma cell-type multicentric Castleman disease (MCD). [from OMIM]

Conditions with this feature

Combined immunodeficiency due to STIM1 deficiency
MedGen UID:
440575
Concept ID:
C2748557
Disease or Syndrome
Immunodeficiency-10 (IMD10) is an autosomal recessive primary immunodeficiency characterized by onset of recurrent infections in childhood due to defective T- and NK-cell function, although the severity is variable. Affected individuals may also have hypotonia, hypohidrosis, or dental enamel hypoplasia consistent with amelogenesis imperfecta (summary by Parry et al., 2016).
Kaposi sarcoma, susceptibility to
MedGen UID:
761233
Concept ID:
C3538945
Finding
Combined immunodeficiency due to OX40 deficiency
MedGen UID:
816383
Concept ID:
C3810053
Disease or Syndrome
Immunodeficiency-16 is an autosomal recessive primary immunodeficiency associated with classic Kaposi sarcoma of childhood and poor T-cell recall immune responses due to complete functional OX40 deficiency (Byun et al., 2013).

Professional guidelines

PubMed

Stoneham S, Hunter A, Raahimi M, Livesey A, Mitchell CD, Keohane S
Clin Exp Dermatol 2023 Jul 21;48(8):866-872. doi: 10.1093/ced/llad166. PMID: 37127912
Subramaniam A, Giani C, Napolitano A, Ravi V, Frezza AM, Jones RL
Surg Oncol Clin N Am 2022 Jul;31(3):485-510. doi: 10.1016/j.soc.2022.03.014. PMID: 35715146
Schneider JW, Dittmer DP
Am J Clin Dermatol 2017 Aug;18(4):529-539. doi: 10.1007/s40257-017-0270-4. PMID: 28324233Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Kaposi Sarcoma, 2022

Recent clinical studies

Etiology

Wang LL, Lin SK, Stull CM, Shin TM, Higgins HW, Giordano CN, McMurray SL, Etzkorn JR, Miller CJ, Walker JL
Dermatol Clin 2023 Jan;41(1):141-162. doi: 10.1016/j.det.2022.07.012. PMID: 36410975
Johnston WT, Erdmann F, Newton R, Steliarova-Foucher E, Schüz J, Roman E
Cancer Epidemiol 2021 Apr;71(Pt B):101662. Epub 2020 Jan 8 doi: 10.1016/j.canep.2019.101662. PMID: 31924557
Vangipuram R, Tyring SK
Cancer Treat Res 2019;177:1-21. doi: 10.1007/978-3-030-03502-0_1. PMID: 30523619
Yarchoan R, Uldrick TS
N Engl J Med 2018 Mar 15;378(11):1029-1041. doi: 10.1056/NEJMra1615896. PMID: 29539283Free PMC Article
Schneider JW, Dittmer DP
Am J Clin Dermatol 2017 Aug;18(4):529-539. doi: 10.1007/s40257-017-0270-4. PMID: 28324233Free PMC Article

Diagnosis

Legesse TB, Twaddell WS, Ng VY, Davis DL, Ciner AT, Kallen ME
Histopathology 2023 Jul;83(1):149-150. Epub 2023 Apr 11 doi: 10.1111/his.14914. PMID: 37040901
Addula D, Das CJ, Kundra V
Abdom Radiol (NY) 2021 Nov;46(11):5297-5306. Epub 2021 Jul 13 doi: 10.1007/s00261-021-03205-6. PMID: 34255129Free PMC Article
Iftode N, Rădulescu MA, Aramă ȘS, Aramă V
Rom J Intern Med 2020 Dec 1;58(4):199-208. Epub 2020 Dec 17 doi: 10.2478/rjim-2020-0017. PMID: 32681788
Schneider JW, Dittmer DP
Am J Clin Dermatol 2017 Aug;18(4):529-539. doi: 10.1007/s40257-017-0270-4. PMID: 28324233Free PMC Article
Radu O, Pantanowitz L
Arch Pathol Lab Med 2013 Feb;137(2):289-94. doi: 10.5858/arpa.2012-0101-RS. PMID: 23368874

Therapy

Wang LL, Lin SK, Stull CM, Shin TM, Higgins HW, Giordano CN, McMurray SL, Etzkorn JR, Miller CJ, Walker JL
Dermatol Clin 2023 Jan;41(1):141-162. doi: 10.1016/j.det.2022.07.012. PMID: 36410975
Dupin N
Curr Opin Oncol 2020 Mar;32(2):122-128. doi: 10.1097/CCO.0000000000000601. PMID: 31815777
Etemad SA, Dewan AK
Dermatol Clin 2019 Oct;37(4):505-517. Epub 2019 Jul 10 doi: 10.1016/j.det.2019.05.008. PMID: 31466590
Lebbe C, Garbe C, Stratigos AJ, Harwood C, Peris K, Marmol VD, Malvehy J, Zalaudek I, Hoeller C, Dummer R, Forsea AM, Kandolf-Sekulovic L, Olah J, Arenberger P, Bylaite-Bucinskiene M, Vieira R, Middleton M, Levy A, Eggermont AM, Battistella M, Spano JP, Grob JJ, Pages C; European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organisation for Research and Treatment of Cancer (EORTC)
Eur J Cancer 2019 Jun;114:117-127. Epub 2019 May 13 doi: 10.1016/j.ejca.2018.12.036. PMID: 31096150
Francès C, Lebbé C
Cancer Treat Res 2009;146:299-309. doi: 10.1007/978-0-387-78574-5_24. PMID: 19415211

Prognosis

Johnston WT, Erdmann F, Newton R, Steliarova-Foucher E, Schüz J, Roman E
Cancer Epidemiol 2021 Apr;71(Pt B):101662. Epub 2020 Jan 8 doi: 10.1016/j.canep.2019.101662. PMID: 31924557
Stephanie S, Mckinney B, von Rosenvinge EC
Clin Gastroenterol Hepatol 2020 Jan;18(1):A36. Epub 2018 Sep 8 doi: 10.1016/j.cgh.2018.09.001. PMID: 30201595
Dalla Pria A, Pinato DJ, Bracchi M, Bower M
F1000Res 2019;8 Epub 2019 Jun 26 doi: 10.12688/f1000research.17401.1. PMID: 31297181Free PMC Article
Schneider JW, Dittmer DP
Am J Clin Dermatol 2017 Aug;18(4):529-539. doi: 10.1007/s40257-017-0270-4. PMID: 28324233Free PMC Article
Restrepo CS, Ocazionez D
Semin Ultrasound CT MR 2011 Oct;32(5):456-69. doi: 10.1053/j.sult.2011.03.007. PMID: 21963166

Clinical prediction guides

Dauguet M, Lebbé C, Vignes S
J Med Vasc 2023 Nov-Dec;48(5-6):181-187. Epub 2023 Nov 8 doi: 10.1016/j.jdmv.2023.10.007. PMID: 38035924
Davis DA, Shrestha P, Yarchoan R
J Med Virol 2023 Sep;95(9):e29071. doi: 10.1002/jmv.29071. PMID: 37665216Free PMC Article
Moorad R, Kasonkanji E, Gumulira J, Gondwe Y, Dewey M, Pan Y, Peng A, Pluta LJ, Kudowa E, Nyasosela R, Tomoka T, Tweya H, Heller T, Gugsa S, Phiri S, Moore DT, Damania B, Painschab M, Hosseinipour MC, Dittmer DP
Int J Cancer 2023 Dec 15;153(12):2082-2092. Epub 2023 Aug 21 doi: 10.1002/ijc.34689. PMID: 37602960
Böni M, Rieble L, Münz C
Viruses 2022 Dec 2;14(12) doi: 10.3390/v14122709. PMID: 36560713Free PMC Article
Dreyfus DH
J Allergy Clin Immunol 2013 Dec;132(6):1278-86. Epub 2013 Apr 20 doi: 10.1016/j.jaci.2013.02.039. PMID: 23611298

Recent systematic reviews

Zhang H, Cai S, Xia Y, Lin Y, Zhou G, Yu Y, Feng M
Virol J 2023 Dec 4;20(1):288. doi: 10.1186/s12985-023-02234-5. PMID: 38049836Free PMC Article
Barron K, Omiunu A, Celidonio J, Cruz-Mullane A, Din-Lovinescu C, Chemas-Velez MM, Baredes S, Eloy JA, Fang CH
Otolaryngol Head Neck Surg 2023 Mar;168(3):269-281. Epub 2023 Jan 24 doi: 10.1177/01945998221105059. PMID: 35671149
Liew YCC, Tam YCS, Oh CC
Int J Dermatol 2022 Nov;61(11):1311-1324. Epub 2022 Jul 1 doi: 10.1111/ijd.16318. PMID: 35775738
Ibrahim Khalil A, Franceschi S, de Martel C, Bray F, Clifford GM
Int J Cancer 2022 Jun 15;150(12):1948-1957. Epub 2022 Feb 16 doi: 10.1002/ijc.33951. PMID: 35085400
Régnier-Rosencher E, Guillot B, Dupin N
J Am Acad Dermatol 2013 Feb;68(2):313-31. Epub 2012 Jun 12 doi: 10.1016/j.jaad.2012.04.018. PMID: 22695100

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2022
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Kaposi Sarcoma, 2022

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