Malignant Rhabdoid Tumor of the Kidney in a Child With Xeroderma Pigmentosum: Incidence or Coincidence?

Malignant rhabdoid tumor of the kidney (MRTK) is a rare aggressive malignant rhabdoid tumor that mainly affects children. At the onset of the disease, the usual clinical manifestations are gross hematuria, abdominal pain, and abdominal distension. The prognosis remains poor. Patients with rhabdoid tumors (RT) are treated according to institutional preferences that combine surgery, radiation therapy, and chemotherapy. The authors present the rare case of a child with xeroderma pigmentosum (XP) who presented with an abdominal mass accompanied by hematuria and abdominal pain. The radiological and histological results were congruent with the MRTK. The patient received preoperative chemotherapy but unfortunately died of septic shock. This case highlights the importance of being aware of MRTK and its fatal complications, as well as the increased risk of kidney tumors in patients with XP.