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Modeling Spontaneous Metastatic Renal Cell Carcinoma mRCC in Mice Following Nephrectomy
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Unraveling Smooth Muscle-rich Renal Cell Carcinoma: Clinical, Oncological, Genetic, and Pathological Insights

Ruben Blachman-Braun1, Milan H Patel1, Braden Millan1

  • 1Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.

Urology
|November 19, 2025
PubMed
Abstract

Insights

Smooth Muscle-Rich Renal Cell Carcinoma (smrRCC) often affects Black/African American individuals and presents as bilateral multifocal disease requiring multiple surgeries. Germline ASXL1 variants may increase smrRCC risk, and pathological factors influence outcomes.

Area of Science:

  • Oncology
  • Urology
  • Genetics

Background:

  • Smooth Muscle-Rich Renal Cell Carcinoma (smrRCC) is a rare subtype of renal cell carcinoma.
  • Understanding its clinical presentation, management, and genetic underpinnings is crucial for improving patient outcomes.

Purpose of the Study:

  • To detail institutional experience with smrRCC.
  • Focus on clinical presentation, surgical management, oncologic outcomes, and genetic features.

Main Methods:

  • Retrospective chart review of patients with smrRCC treated between January 2008 and April 2025.
  • Collected and analyzed clinical, surgical, pathological, and genetic data.

Main Results:

  • 26 patients with smrRCC were identified, predominantly male (73.1%) and Black/African American (73.1%).
  • Bilateral multifocal (BMF) renal masses were common (88.5%), necessitating multiple surgeries (48 total) with an average of 4 tumors per surgery.
  • 171 of 216 resected tumors (79.2%) showed smrRCC histology. One patient with a pT3 tumor (ISUP/Furman grade 3) developed metastasis.
  • Germline ASXL1 variants of uncertain significance were found in 30.7% of patients.

Conclusions:

  • smrRCC frequently impacts Black/African American individuals and often presents as BMF disease requiring extensive surgical intervention.
  • While no predominant genetic alterations were found, germline ASXL1 variants suggest a potential increased risk.
  • Pathological grade and stage appear to influence the oncologic behavior and metastatic potential of smrRCC.

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