---
title: "Sporadic Renal Angiomyolipoma with Tumor Thrombus Extending to IVC and Right Heart: Multidisciplin"
id: "pubmed-42664153"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42664153"
content_type: "clinical_feed_article"
specialty: "Oncology"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42664153/"
doi: "10.12659/AJCR.952664"
published_at: "2026-08-28T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Sporadic Renal Angiomyolipoma with Tumor Thrombus Extending to IVC and Right Heart: Multidisciplin
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42664153
- **Specialty:** [Oncology](https://medichelpline.com/clinical-feed/oncology.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42664153/)
- **DOI:** [10.12659/AJCR.952664](https://doi.org/10.12659%2FAJCR.952664)
- **Published At:** 2026-08-28T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- A 36-year-old woman with rheumatoid arthritis presented with progressive left upper-quadrant abdominal pain, nausea, and back discomfort. - Cross-sectional imaging identified a large left **renal angiomyolipoma** measuring 19.5 × 18.0 × 10.5 cm. - There was a contiguous **tumor thrombus** tracking from the left renal vein into the **inferior vena cava (IVC)** and extending into the right atrium, intermittently prolapsing across the tricuspid valve into the right ventricle. - Echocardiography confirmed a mobile intracardiac mass without valvular obstruction. - Genetic testing for **TSC1** and **TSC2** mutations was negative, supporting a sporadic angiomyolipoma rather than tuberous sclerosis complex–associated disease. - The patient underwent a coordinated, multidisciplinary operation: en bloc left **radical nephrectomy** with complete venous thrombectomy and IVC reconstruction. - Final pathology documented complete excision with negative margins and absence of epithelioid histology. - The case demonstrates that significant vascular and cardiac invasion can occur in sporadic AML, challenging assumptions that aggressive vascular extension is confined to TSC-associated or epithelioid lesions. - The report emphasizes the need for early recognition, comprehensive imaging, individualized operative planning, and long-term radiographic surveillance for complex AML presentations.
## Clinical Analysis & Structured Key Points
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Affiliations Expand ### Affiliations * 1 Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA. * 2 Department of Hematology and Oncology, Covenant Medical Center, Lubbock, TX, USA. * 3 Department of Internal Medicine, Covenant Medical Center, Lubbock, TX, USA. * PMID: **42664153** * DOI: [ 10.12659/AJCR.952664 ](https://doi.org/10.12659/ajcr.952664) Item in Clipboard Case Reports # Aggressive Sporadic Renal Angiomyolipoma With Tumor Thrombus Extending Into the Inferior Vena Cava, Right Atrium, and Right Ventricle: A Multidisciplinary Case Report Caroline J Cushman et al. Am J Case Rep. 2026. Show details Display options Display options Format Abstract PubMed PMID Am J Case Rep Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Am+J+Case+Rep%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Am+J+Case+Rep%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42664153/) . 2026 Aug 28:27:e952664. doi: 10.12659/AJCR.952664. ### Authors [Caroline J Cushman](https://pubmed.ncbi.nlm.nih.gov/?term=Cushman+CJ&cauthor_id=42664153)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42664153/#short-view-affiliation-1 "Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA."), [Kolos K Nagy](https://pubmed.ncbi.nlm.nih.gov/?term=Nagy+KK&cauthor_id=42664153)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42664153/#short-view-affiliation-1 "Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA."), [Rodolfo Martinez](https://pubmed.ncbi.nlm.nih.gov/?term=Martinez+R&cauthor_id=42664153)[ 2 ](https://pubmed.ncbi.nlm.nih.gov/42664153/#short-view-affiliation-2 "Department of Hematology and Oncology, Covenant Medical Center, Lubbock, TX, USA."), [Kaiser R Tarafdar](https://pubmed.ncbi.nlm.nih.gov/?term=Tarafdar+KR&cauthor_id=42664153)[ 3 ](https://pubmed.ncbi.nlm.nih.gov/42664153/#short-view-affiliation-3 "Department of Internal Medicine, Covenant Medical Center, Lubbock, TX, USA.") ### Affiliations * 1 Texas Tech University Health Sciences Center, School of Medicine, Lubbock, TX, USA. * 2 Department of Hematology and Oncology, Covenant Medical Center, Lubbock, TX, USA. * 3 Department of Internal Medicine, Covenant Medical Center, Lubbock, TX, USA. * PMID: **42664153** * DOI: [ 10.12659/AJCR.952664 ](https://doi.org/10.12659/ajcr.952664) Item in Clipboard Cite Display options Display options Format Abstract PubMed PMID ## Abstract BACKGROUND Renal angiomyolipoma (AML) is a typically benign mesenchymal tumor with an indolent clinical course. Rarely, AML demonstrate aggressive vascular invasion, most commonly in association with tuberous sclerosis complex (TSC). Intracardiac extension, particularly into the right ventricle, is exceptionally uncommon in sporadic cases and presents significant diagnostic and surgical challenges. CASE REPORT A 36-year-old woman with a history of rheumatoid arthritis presented with progressive left upper-quadrant abdominal pain, nausea, and back discomfort. Cross-sectional imaging revealed a massive left renal angiomyolipoma measuring 19.5×18.0×10.5 cm, with a contiguous tumor thrombus extending from the left renal vein through the inferior vena cava into the right atrium, intermittently prolapsing across the tricuspid valve into the right ventricle. Echocardiography confirmed a mobile intracardiac mass without evidence of valvular obstruction. Genetic testing was negative for TSC1 and TSC2 mutations, consistent with a sporadic angiomyolipoma. The patient underwent successful en bloc left radical nephrectomy with complete venous thrombectomy and inferior vena cava reconstruction through a coordinated multidisciplinary approach. Final pathology confirmed complete excision with negative margins and absence of epithelioid features. CONCLUSIONS This case was an exceptionally rare presentation of sporadic renal angiomyolipoma with extensive intracardiac extension into the right ventricle. It demonstrates that significant vascular and cardiac involvement can occur even in the absence of TSC-associated genetic mutations or epithelioid histology, thereby challenging traditional assumptions regarding AML behavior. These findings underscore the importance of early recognition, comprehensive imaging, and individualized operative planning in the management of complex AML presentations. Given the potential for aggressive progression in otherwise benign tumors, close long-term radiographic surveillance remains essential. 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