{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:35:09Z","timestamp":1760146509896,"version":"build-2065373602"},"reference-count":39,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2024,11,8]],"date-time":"2024-11-08T00:00:00Z","timestamp":1731024000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Institutes of Health (NIH)","award":["U54CA254569"],"award-info":[{"award-number":["U54CA254569"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background\/Objectives: Kaposi sarcoma (KS) is a common lymphatic endothelial cancer among children with and without HIV in central and eastern Africa. Despite its clinical heterogeneity, its various clinical phenotypes are often grouped together in staging and treatment algorithms. Patients with KS tumor-associated edema, referring to hard, non-pitting lesions which often lead to chronic disability, represent a unique, understudied subgroup of children with KS. To continue our work defining the distinct phenotypes of pediatric KS, this study aimed to assess the clinical progression and outcomes of KS edema in children. Methods: A retrospective cohort study was conducted at Kamuzu Central Hospital in Lilongwe, Malawi, focusing on children diagnosed with KS edema between 2010 and 2023. Results: We identified 52 children with KS edema, representing 27% of all patients with KS. Initial chemotherapy resulted in a clinical response in 92% of patients, but 46% experienced relapse or disease progression with a median time to first relapse of 12 months. Multiple progressions were common, with 31% of patients experiencing two or more events. Event-free survival at two years was 32%, dropping to 24% at five years, while overall survival was 73% at two years and 57% at five years. Relapse was more common among patients with KS edema versus those without it (relative risk = 2.1; 95%CI, 1.4\u20133.2; p &lt; 0.001). Eight patients (15%) relapsed with visceral disease, five of whom originally presented with KS edema alone. Conclusions: Patients with KS edema have a unique, relapsing-remitting pattern of disease with a high risk of relapse relative to other forms of KS with subsequent long-term mortality, even after initial positive treatment responses. Late relapse and mortality with visceral disease are possible even among children presenting initially with KS edema alone. Children with KS edema require long-term follow-up, and novel treatment approaches tailored towards preventing frequent relapse are needed.<\/jats:p>","DOI":"10.3390\/cancers16223769","type":"journal-article","created":{"date-parts":[[2024,11,8]],"date-time":"2024-11-08T06:05:41Z","timestamp":1731045941000},"page":"3769","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Tumor-Associated Edema in Children with Kaposi Sarcoma: 14 Years\u2019 Experience at Kamuzu Central Hospital, Lilongwe, Malawi"],"prefix":"10.3390","volume":"16","author":[{"given":"Fatsani Rose","family":"Manase","sequence":"first","affiliation":[{"name":"Baylor College of Medicine Children\u2019s Foundation, Lilongwe P.O. Box B-397, Malawi"},{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Allison","family":"Silverstein","sequence":"additional","affiliation":[{"name":"Department of Pediatrics-Palliative Care Medicine, University of Colorado School of Medicine, Aurora, CO 80045, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William","family":"Kamiyango","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine Children\u2019s Foundation, Lilongwe P.O. Box B-397, Malawi"},{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3845-8186","authenticated-orcid":false,"given":"Jimmy","family":"Villiera","sequence":"additional","affiliation":[{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"},{"name":"University of North Carolina Chapel Hill Project Malawi, Lilongwe P.O. Box A-104, Malawi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clement","family":"Dziwe","sequence":"additional","affiliation":[{"name":"Lighthouse Clinic, Lilongwe P.O. Box 106, Malawi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Wallrauch","sequence":"additional","affiliation":[{"name":"Lighthouse Clinic, Lilongwe P.O. Box 106, Malawi"},{"name":"Institute of Infectious Disease and Tropical Medicine, LMU University Hospital Munich, 80802 M\u00fcnchen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-4626-0592","authenticated-orcid":false,"given":"Tom","family":"Heller","sequence":"additional","affiliation":[{"name":"Lighthouse Clinic, Lilongwe P.O. Box 106, Malawi"},{"name":"International Training and Education Center for Health, University of Washington, Seattle, WA 98195, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark","family":"Zobeck","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tamiwe","family":"Tomoka","sequence":"additional","affiliation":[{"name":"University of North Carolina Chapel Hill Project Malawi, Lilongwe P.O. Box A-104, Malawi"},{"name":"Kamuzu Central Hospital, Lilongwe P.O. Box 30377, Malawi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8379-6088","authenticated-orcid":false,"given":"Michael E.","family":"Scheurer","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carl E.","family":"Allen","sequence":"additional","affiliation":[{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"},{"name":"Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5344-7875","authenticated-orcid":false,"given":"Nmazuo","family":"Ozuah","sequence":"additional","affiliation":[{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"},{"name":"Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rizine","family":"Mzikamanda","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine Children\u2019s Foundation, Lilongwe P.O. Box B-397, Malawi"},{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nader Kim","family":"El-Mallawany","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3968-4668","authenticated-orcid":false,"given":"Casey L.","family":"McAtee","sequence":"additional","affiliation":[{"name":"Texas Children\u2019s Hospital Global HOPE, Lilongwe P.O. Box B-397, Malawi"},{"name":"Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,11,8]]},"reference":[{"key":"ref_1","first-page":"35","article-title":"Pediatric Kaposi Sarcoma in Context of the HIV Epidemic in Sub-Saharan Africa: Current Perspectives","volume":"9","author":"McAtee","year":"2018","journal-title":"Pediatric Health Medicine Ther."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3165","DOI":"10.1172\/JCI84418","article-title":"Kaposi Sarcoma\u2013Associated Herpesvirus: Immunobiology, Oncogenesis, and Therapy","volume":"126","author":"Dittmer","year":"2016","journal-title":"J. Clin. 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