Malignant Rhabdoid Tumor Clinical Presentation

Updated: Sep 27, 2021
  • Author: James I Geller, MD, FAAP; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Presentation

History

Children with malignant rhabdoid tumor (MRT) of the kidney present with signs and symptoms related to an intrarenal mass.

Pain is difficult to assess because the median age at presentation is about 11 months. However, fussiness is reported in most patients.

Gross hematuria is a presenting feature in approximately 60% of patients. By contrast, only 20% of patients with Wilms tumor have gross hematuria.

Fever is a presenting symptom in 50% of patients with a MRT of the kidney, compared with 25% of patients with a Wilms tumor.

As many as 20% of patients with a MRT of the kidney have synchronous or metachronous CNS lesions, including both metastases and second primary AT/RTs.

A detailed family cancer history should be obtained.

Next:

Physical Examination

The physical findings of patients with MRT depend on the site of origin of the tumor. [29, 30]

For MRT of the kidney, the physical examination is most remarkable for a large intra-abdominal mass.

Hypertension, defined as blood pressure greater than the 95th percentile, is observed in up to 70% of patients.

In contrast to a Wilms tumor, a MRT is not associated with WAGR syndrome, which consists of a Wilms tumor, aniridia, genitourinary anomalies, and range of developmental difficulties, or with Beckwith-Wiedemann syndrome, which consists of organomegaly, large birth weight, macroglossia, and hemihypertrophy.

Evidence of focal neurologic signs or increased intracranial pressure should be evaluated in light of the prevalence of synchronous AT/RTs.

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