{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T20:15:56Z","timestamp":1791490556324,"version":"4.3.4"},"reference-count":21,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2022,5,19]],"date-time":"2022-05-19T00:00:00Z","timestamp":1652918400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health-Ricerca Corrente to Centro Cardiologico Monzino IRCCS"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Wilms tumor (WT) is the most common primary renal malignancy in young children. WT vascular extension to the inferior vena cava (IVC) occurs in 4\u201310% of cases and can reach the right atrium (RA) in 1%. Data on WT clinical presentation and outcome in developing countries are limited. The aim of the present study is to describe the prevalence of intracardiac extension in a consecutive population of WT patients observed in a large non-profit Ugandan hospital. A total of 16 patients with a histological diagnosis of 29 WT were screened in a 6-month period. Patient n\u00b02, a 3 y\/o child, presented with a 3-week history of abdominal distension, difficulty in breathing, and swelling of the lower limbs. A cardiovascular system exam showed rhythmic heart sounds, a heart rate of 110 beats per minute, and a pansystolic murmur on the tricuspid area; the abdomen was grossly distended with a palpable mass in the right flank, hepatomegaly, and splenomegaly. An abdomen ultrasound showed an intra-abdominal tumor, involving the right kidney and the liver and extended to the IVC. An ultrasound guided biopsy showed a picture consistent with WT. Cardiac echo showed a huge, mobile, cardiac mass attached to the right side of the interatrial septum, involving the tricuspid valve annulus, causing a \u201cfunctional\u201d tricuspid stenosis. The patient died of cardiogenic shock 7 days after admission. Patient n\u00b03, a 3 y\/o child, presented with analogue symptoms and the same diagnosis. The cardiac echo showed a round mass in the RA. Thirteen more patients were screened with cardiac echo, showing a normal heart picture. In our limited series, we found WT cardiac extension in three patients over 16 (19%). Cardiac echo performed routinely can lead to a better staging, prognostic, and therapeutic assessment. In our setting, the intra-cardiac extension could be more frequent than previously reported and might have prognostic implications.<\/jats:p>","DOI":"10.3390\/children9050743","type":"journal-article","created":{"date-parts":[[2022,5,19]],"date-time":"2022-05-19T12:25:57Z","timestamp":1652963157000},"page":"743","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Unexpected Huge Prevalence of Intracardiac Extension of Wilms Tumor\u2014A Single Center Experience from a Ugandan Hospital"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9985-7407","authenticated-orcid":false,"given":"Massimo","family":"Mapelli","sequence":"first","affiliation":[{"name":"Centro Cardiologico Monzino, IRCCs, Via Parea 4, 20138 Milan, Italy"},{"name":"Department of Clinical Sciences and Community Health, Cardiovascular Section, University of Milan, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Zagni","sequence":"additional","affiliation":[{"name":"Terapia Intensiva Neonatale, Ospedale Fatebenefratelli P.O. Macedonio Melloni, Via Macedonio Melloni 52, 20129 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Ferrara","sequence":"additional","affiliation":[{"name":"Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Calbi","sequence":"additional","affiliation":[{"name":"San Raffaele Telethon Institute for Gene Therapy (SR-TIGET), IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132 Milan, Italy"},{"name":"Pediatric Immunohematology Unit and BMT Program, IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7781-1691","authenticated-orcid":false,"given":"Irene","family":"Mattavelli","sequence":"additional","affiliation":[{"name":"Centro Cardiologico Monzino, IRCCs, Via Parea 4, 20138 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Muratori","sequence":"additional","affiliation":[{"name":"Centro Cardiologico Monzino, IRCCs, Via Parea 4, 20138 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jackson","family":"Kansiime","sequence":"additional","affiliation":[{"name":"St. Mary\u2019s Hospital Lacor, Gulu P.O. Box 180, Uganda"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cyprian","family":"Opira","sequence":"additional","affiliation":[{"name":"St. Mary\u2019s Hospital Lacor, Gulu P.O. Box 180, Uganda"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piergiuseppe","family":"Agostoni","sequence":"additional","affiliation":[{"name":"Centro Cardiologico Monzino, IRCCs, Via Parea 4, 20138 Milan, Italy"},{"name":"Department of Clinical Sciences and Community Health, Cardiovascular Section, University of Milan, 20122 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1016\/j.ejca.2006.05.010","article-title":"Malignant renal tumours incidence and survival in European children (1978\u20131997): Report from the Automated Childhood Cancer Information System project","volume":"42","author":"Pastore","year":"2006","journal-title":"Eur. J. Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1002\/mpo.2950210305","article-title":"Epidemiology of Wilms tumor","volume":"21","author":"Breslow","year":"1993","journal-title":"Med. Pediatr. Oncol."},{"key":"ref_3","unstructured":"Ries, L.A., Smith, M.A., Gurney, J.G., Linet, M., Tamra, T., Young, J.L., and Bunin, G.R. (1999). Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975\u20131995, National Cancer Institue."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1002\/pbc.24658","article-title":"Outcome of patients with Stage II\/favorable histology Wilms tumor with and without local tumor spill: A report from the National Wilms Tumor Study Group","volume":"61","author":"Green","year":"2014","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_5","unstructured":"Van Tinteren, H. (2015, January 24\u201326). Statistical report. 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