Fatal Wilms Tumor Case: Tricuspid Valve Obstruction in Child (2025)

The Heartbreaking Reality: When a Childhood Kidney Cancer Claims a Life Through Delayed Care

Imagine a young child, just eight years old, battling a rare and aggressive form of kidney cancer that silently invades her heart, leading to a swift and preventable death. This isn't just a story—it's a stark warning about the gaps in healthcare systems that allow such tragedies to unfold. But here's where it gets controversial: Could better awareness and quicker interventions in underserved regions actually save lives, or is the root issue deeper, tied to global inequalities in medical access? Let's dive into this compelling case study and explore the critical lessons it holds.

Case Report

Open Access (https://www.springernature.com/gp/open-science/about/the-fundamentals-of-open-access-and-open-research)

Published: 05 November 2025

Journal of Medical Case Reports (https://jmedicalcasereports.biomedcentral.com/) volume 19, Article number: 565 (2025) Cite this article

Abstract

Background

Wilms’ tumor stands out as the primary kidney cancer affecting young children, representing roughly 6% of all pediatric cancers. While it's not uncommon for this tumor to spread into the inferior vena cava—a major vein returning blood to the heart—in about 4–10% of cases, heart involvement is exceedingly rare, occurring in just 1% of instances. In areas with limited resources, late diagnoses and barriers to specialized treatment can result in devastating outcomes, turning what might be manageable elsewhere into a fatal scenario.

Case Presentation

We describe the unfortunate story of an 8-year-old Tanzanian girl who arrived at a national hospital after six months of worsening abdominal swelling, shortness of breath, and leg edema. Scans unveiled a sizable mass on the right side of her kidney that had extended into the inferior vena cava and right atrium, blocking blood flow through the tricuspid valve—a key heart valve. She received a single dose of chemotherapy drugs, intravenous actinomycin D and vincristine, but her health declined rapidly into cardiogenic shock, and she passed away just seven days after admission.

Conclusion

This case emphasizes the urgency of spotting Wilms’ tumor early and ensuring coordinated, multidisciplinary care when it involves heart and blood vessel complications. It calls for bolstering pediatric oncology, surgical, cardiac, and critical care services in low-resource environments to avert such avoidable deaths.

Peer Review Reports (https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05514-6/peer-review)

Introduction

Wilms’ tumor, also known as nephroblastoma, is the leading type of kidney cancer in kids, making up about 6% of all childhood cancers [1,2,3]. It usually pops up between ages 2 and 5 and is often found by accident when a doctor notices an abdominal lump during a check-up [4]. For beginners, think of it as a growth in the kidney that can sometimes mimic a harmless mass but is actually cancerous and needs prompt attention. In its early stages, it might not cause symptoms, but as it advances, children could experience high blood pressure, blood in the urine, stomach pain, or general signs like fever and weight loss [4]. In 4–10% of cases, the tumor forms a clot that travels into the renal vein and inferior vena cava (IVC), the large vein carrying blood back from the lower body to the heart [5]. This can cause partial or full blockage, leading to IVC syndrome—a condition where blood can't flow properly, causing swelling and other issues [6,7,8]. Occasionally, this clot reaches the right atrium of the heart, jamming the tricuspid valve and possibly causing right-sided heart failure, shock, or even a clot traveling to the lungs (pulmonary embolism) [9,10,11,12,13]. Surprisingly, up to half of kids with heart involvement might not show symptoms initially, making early detection tricky [12,13].

Treatment approaches differ globally: The Children’s Oncology Group (COG) often starts with surgery, while the International Society of Pediatric Oncology (SIOP) prefers chemotherapy first to shrink the tumor [8]. Both methods work well in wealthier countries, with over 90% survival rates over five years, but in poorer areas, late diagnoses, poor imaging, and limited specialist care lead to dismal outcomes [14,15,16].

But here's where it gets controversial... Why do some regions see such high rates of advanced cases? Is it purely a matter of access, or do cultural beliefs and misinformation play a role in delaying parents from seeking help? This Ugandan study, for instance, found 19% of Wilms’ cases had heart extension due to prolonged delays [17]. It challenges us to think: Are we doing enough to educate communities, or are systemic failures the bigger culprit?

Here, we share a tragic example of Wilms’ tumor with far-reaching vascular and cardiac spread, shedding light on diagnostic shortcomings and treatment hurdles in under-resourced settings, while offering insights to improve pathways for better outcomes.

Presentation of the Case

An 8-year-old girl hailing from Tanzania’s Lake Zone was brought to a top-tier national facility after a six-month history of increasingly swollen abdomen and a two-week bout of leg swelling plus breathing difficulties. Upon arrival, she was drowsy with a Glasgow Coma Score of 11—a scale measuring consciousness, where 15 is fully alert—along with rapid breathing at 40 breaths per minute and a fast heartbeat of 150 beats per minute. Her legs showed pitting edema, a type of swelling that leaves an indentation when pressed, and this was most noticeable in the lower extremities.

During the physical exam, doctors felt a firm, tender mass on the right side of her abdomen that crossed the midline. No fluid buildup in the belly (ascites) was noted, and her bowel sounds were normal. Heart checks revealed a pansystolic murmur—a whooshing sound heard best near the tricuspid valve, indicating possible valve issues.

Labs showed mild anemia with hemoglobin at 10 g/dL (a measure of red blood cells), moderate low platelet count at 65 × 10^9/L (important for clotting), and elevated creatinine at 104.7 µmol/L and blood urea nitrogen at 12.4 mmol/L (signaling kidney stress). Electrolytes were fine, and urine vanillylmandelic acid (VMA)—a marker for certain tumors—was normal.

An echocardiogram, a heart ultrasound, revealed a bright, solid mass (5.7 cm × 3.1 cm) filling the right atrium, severely blocking blood inflow through the tricuspid valve with barely any flow left (Fig. 1). The hepatic part of the IVC was completely blocked (Fig. 2). The right atrial wall was stretched out, showing high pressure inside. No abnormal heart connections (shunts) were seen.

A section of echocardiography showing a huge mass in the right atrium

Full size image (https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05514-6/figures/1)

Treatment and Progress

She was admitted and stabilized, beginning SIOP-guided neoadjuvant chemotherapy. On day 2, she got one dose of intravenous actinomycin D (945 mcg) and vincristine (1 mg). Supportive measures included restricting fluids, treating heart failure symptoms, and watching for metabolic imbalances.

By day 3, her breathing worsened and her mental state declined. She was put on a ventilator and mechanically breathed for. Despite all efforts, her condition kept deteriorating, and on day 7, she died from cardiogenic shock.

Discussion

Wilms’ tumor invading the heart is uncommon but terrifying, demanding sharp clinical awareness and immediate, team-based action. In our Tanzanian context, such cases are rare, but a Ugandan report showed an unusually high 19% with heart extension, likely from long waits before medical help [17]. In many low-income areas, kids only seek care after symptoms have raged on for months, allowing the cancer to invade major vessels and the heart. This highlights how delays in diagnosis worsen progression.

And this is the part most people miss... While heart extension doesn't always doom prognosis [17], the heart complications it brings—like rapid decline into shock—can spiral into death swiftly, as in our patient.

In well-equipped places, echocardiography is the go-to for spotting heart involvement in Wilms’ cases [18]. But in Tanzania, these machines and skilled operators are scarce outside big hospitals. Here, it was done at a national center, but the wait delayed staging and planning. These gaps make it hard to catch vascular spread early, raising complication risks.

Tumor biopsy is key for tailoring treatment in Wilms’ tumor, but in Tanzania, it's often post-surgery. Without pre-op confirmation, doctors rely on exams and scans, complicating things amid late diagnoses.

The girl's death stemmed from cardiogenic shock due to blocked heart inflow, which reduced blood return and heart output, starving organs and causing collapse. In ideal scenarios, 6 weeks of pre-surgery chemo (vincristine weekly, actinomycin D every two weeks, sometimes doxorubicin) shrinks the tumor [7,8]. Surgery follows a week after the last dose, once shrinkage is confirmed [7]. Here, chemo started but advanced disease meant no second dose.

This highlights key failures:

- Late diagnosis and referral: Six months of symptoms before hospital shows poor awareness of kids' cancers and weak referral networks. Early Wilms’ is often silent or just an abdominal bump, so delays let it invade vessels, risking clots or heart issues.

- Weak diagnostic tools: Echocardiography caught the problem, but its inconsistency in local clinics delayed evaluation. Using it routinely for Wilms’ with swelling signs is vital to spot extensions.

- Scarce pediatric cardiac, surgical, and ICU resources: Heart involvement needs cardiac surgeons and bypass machines—rare in poor settings. Here, ventilation helped breathing decline but surgery wasn't possible.

- Fragmented multidisciplinary care: Treating Wilms’ requires oncologists, radiologists, surgeons, cardiologists, anesthesiologists, and ICU teams. Without coordination, care suffers.

Conclusion

Wilms’ tumor with heart involvement poses a tough test, especially in resource-scarce places like Tanzania. Delays in spotting it, poor imaging, erratic chemo supply, and missing specialist surgery and care all worsen results. The shift to cardiogenic shock from tricuspid blockage here could have been avoided with earlier detection and treatment.

What do you think? Do you agree that global health disparities are the main barrier, or could individual choices—like ignoring early symptoms—also contribute? Share your views in the comments—let's discuss this critical issue!

Data Availability

No new data was created for this case report.

References

Pastore G, Znaor A, Spreafico F, Graf N, Pritchard-Jones K, Steliarova-Foucher E. Malignant renal tumours incidence and survival in European children (1978–1997): report from the automated childhood cancer information system project. Eur J Cancer. 2006. https://doi.org/10.1016/j.ejca.2006.05.010.
Chiou SS. Malignant renal tumors in childhood. Pediatr Neonatol. 2014. https://doi.org/10.1016/j.pedneo.2014.01.003.
Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993. https://doi.org/10.1002/mpo.2950210305.
Dumba M, Jawad N, McHugh K. Neuroblastoma and nephroblastoma: a radiological review. Cancer Imaging. 2015. https://doi.org/10.1186/s40644-015-0040-6.
Emir S. Wilms tumor with intravascular tumor thrombus. Transl Pediatr. 2014. https://doi.org/10.3978/j.issn.2224-4336.2014.01.03.
Sahu I, Ruhela M, Ola R. Left atrial metastasis of a Wilms’ tumor: a rare occurrence. J Family Med Prim Care. 2020. https://doi.org/10.4103/jfmpc.jfmpc90820.
Murthi GV, Kocyildirim E, Sellathury S, Cuckow PM, Wilcox DT, Michalski A, et al. Wilms’ tumour with persistent intravascular extension: a review of the surgical aspects of management. J Pediatr Urol. 2006. https://doi.org/10.1016/j.jpurol.2005.10.004.
Ekenze SO, Agugua-Obianyo NEN, Odetunde OA. The challenge of nephroblastoma in a developing country. Ann Oncol. 2006. https://doi.org/10.1093/annonc/mdl167.
Yadav SC, Sathe PA, Ghodke RK, Fernandes GC. Giant untreated Wilms tumor with intracardiac extension: a rare case. Indian J Pathol Microbiol. 2013. https://doi.org/10.4103/0377-4929.116159.
Namboodiri N, Krishnamoorthy KM, Tharakan JA. Intra-atrial extension of Wilms’ tumor. J Am Soc Echocardiogr. 2008. https://doi.org/10.1016/j.echo.2007.05.041.
Nakayama DK, Norkool P, DeLorimier AA, O’Neill JA, D’Angio GJ. Intracardiac extension of Wilms’ tumor. A report of the national Wilms’ tumor study. Ann Surg. 1986. https://doi.org/10.1097/00000658.
Abdullah Y, Karpelowsky J, Davidson A, Thomas J, Brooks A, Hewitson J, et al. Management of nine cases of Wilms’ tumour with intracardiac extension - A single centre experience. J Pediatr Surg. 2013. https://doi.org/10.1016/j.jpedsurg.2012.11.024.
Gortani G, Anzini M, Berton E, Rabusin M, Murru F, Benettoni A. Abrupt intracardiac growth of a Wilms tumor. J Pediatr. 2013. https://doi.org/10.1016/j.jpeds.2013.03.062.
Green DM, Breslow NE, D’Angio GJ, Malogolowkin MH, Ritchey ML, Evans AE, et al. 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. Pediatr Blood Cancer. 2014. https://doi.org/10.1002/pbc.24658.
Apple A, Lovvorn HN. Wilms tumor in sub-Saharan Africa: molecular and social determinants of a global pediatric health disparity. Front Oncol. 2020. https://doi.org/10.3389/fonc.2020.606380.
Cunningham ME, Klug TD, Nuchtern JG, Chintagumpala MM, Venkatramani R, Lubega J, et al. Global disparities in Wilms tumor. J Surg Res. 2020. https://doi.org/10.1016/j.jss.2019.10.044.
Mapelli M, Zagni P, Ferrara R, Calbi V, Mattavelli I, Muratori M, et al. Unexpected huge prevalence of intracardiac extension of Wilms tumor—A single center experience from a Ugandan hospital. Children (Basel). 2022. https://doi.org/10.3390/children9050743.
Szymanska A, Augustyn C, Stankowski T, Walek E, Kowalski JP, Koltowski P, et al. Wilms’ tumor With intra-atrial extension: treatment and management. World J Pediatr Congenit Hear Surg. 2016. https://doi.org/10.1177/2150135115583728.

Acknowledgements

We thank the patient's guardian for allowing us to share this case report.

Funding

No financial support was received for this work.

Author Information

Authors and Affiliations

1. Department of Pediatrics and Child Health, Muhimbili University of Health and Allied Sciences, P.O Box 65001, Dar Es Salaam, Tanzania

Joyce Gimonge & Deogratias Nkya

School of Medicine, Muhimbili University of Health and Allied Sciences, P.O Box 65001, Dar Es Salaam, Tanzania

David Muhunzi
Department of Pediatric Cardiology, Jakaya Kikwete Cardiac Institute, P.O Box 65003, Dar Es Salaam, Tanzania

Deogratias Nkya

Authors

1. Joyce Gimonge

2. David Muhunzi

3. Deogratias Nkya

Contributions

Conceptualization: JG and DM. Writing the first draft: JG. Writing the second draft: DM. Proofreading and editing: DN. Approving the final draft: JG, DM, and DN.

Corresponding Author

Correspondence to Joyce Gimonge.

Ethics Declarations

Ethics Approval and Consent to Participate

Not applicable.

Consent for Publication

Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing Interests

The authors declare no competing interests concerning this case report.

Additional Information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and Permissions

Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.