Abstract
Malignant rhabdoid tumor of the kidney (MRTK) is a rare neoplasm of infancy. We report a case of a nine-month-old male infant who presented to the pediatrics outpatient department with the history of fever, lethargy, and abnormal head movements. On gross examination, the patient had a firm, non-tender, intra-abdominal mass at the right lumbar region with irregular margins. Computed tomography scan of the abdomen revealed a lobulated soft tissue arising from the kidney with areas of necrosis. Brain magnetic resonance imaging was also performed, which showed a large heterogeneous lesion in the posterior fossa. Histopathologic study revealed loss of INI1 protein. Since MRTK and atypical teratoid rhabdoid tumor (ATRT) of the brain share a common mutation in the gene (hSNF5/INI1), hence a diagnosis of MRTK with co-existent ATRT of the brain was established. Actinomycin-D and vincristine failed to show any improvement and the condition of the patient deteriorated progressively, resulting in his death within 15 days of hospital admission.
Highlights
Rhabdoid tumor of the kidney (RTK), a rare and highly malignant neoplasm of infancy [1], constitutes only 1.7% of all renal tumors [2]
We report a case of a nine-month-old male infant who presented to the pediatrics outpatient department with the history of fever, lethargy, and abnormal head movements
Malignant rhabdoid tumor of the kidney (MRTK) is significantly associated with primary brain tumors or brain metastasis
Summary
Rhabdoid tumor of the kidney (RTK), a rare and highly malignant neoplasm of infancy [1], constitutes only 1.7% of all renal tumors [2]. In 1978, Beckwith and Palmer described malignant rhabdoid tumor of the kidney (MRTK) as a "rhabdomyosarcomatoid variant of Wilm’s tumor" because of the resemblance of cells to rhabdomyoblasts [3]. It was recognized as a unique malignant renal tumor characterized genetically by deletion/mutation of SMARCBI/INI gene on the long arm of chromosome 22 [4]. Brain magnetic resonance imaging (MRI) displayed a large heterogeneous lesion, measuring approximately 4.7 x 4.2 x 3.7 cm in the posterior fossa (Figure 2) along midline causing compression of the fourth ventricle (Figure 3) and dilation of the third ventricle Another smaller, solid mass was found in the region of foramen of munro with dimensions of 2.9 x 2.1 cm (Figure 4). No response to the therapy was observed and the patient’s clinical condition deteriorated rapidly resulting in his death within 15 days of presentation
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