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Malignant rhabdoid tumor of the kidney in a 7-month-old infant: A rare case report with molecular confirmation of SMARCB1 deletion

MoathA AlQahtani, Ahmed A. Alzuwayyid, Salem A. Bahdilh, Abdelazim Abasher and 1 more

Urology Case Reports | Sep 7, 2026

Abstract

Abstract

ABSTRACT Malignant rhabdoid tumor of the kidney (MRTK) is a rare, highly aggressive renal neoplasm predominantly affecting infants, carrying a poor prognosis. We report a 7-month-old female who presented with a 10-day history of painless gross hematuria. Abdominal imaging revealed a large heterogeneous left renal mass without vascular invasion or distant metastases. Left radical nephrectomy with regional lymphadenectomy achieved complete resection (COG Local Stage II, node-negative). Immunohistochemistry demonstrated complete loss of INI1 expression, and next-generation sequencing confirmed a pathogenic SMARCB1 deletion. This case highlights the diagnostic and therapeutic challenges of MRTK in infancy and highlights the indispensable role of molecular confirmation.

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MoathA AlQahtani

first | King Saud Medical City

Ahmed A. Alzuwayyid

middle | King Saud Medical City | ORCID 0009-0003-9757-152X

Salem A. Bahdilh

middle | King Saud Medical City | ORCID 0009-0005-7412-8829

Abdelazim Abasher

middle | King Saud Medical City

Omaya Banihani

last | King Saud Medical City

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Citation

BibTeX

@article{AlQahtani2026Malignant,
  title = {Malignant rhabdoid tumor of the kidney in a 7-month-old infant: A rare case report with molecular confirmation of SMARCB1 deletion},
  author = {MoathA AlQahtani and Ahmed A. Alzuwayyid and Salem A. Bahdilh and Abdelazim Abasher and Omaya Banihani},
  journal = {Urology Case Reports},
  year = {2026},
  doi = {10.1016/j.eucr.2026.103599},
  url = {https://doi.org/10.1016/j.eucr.2026.103599}
}

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