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| Malignant Rhaboid Tumor | ||
| Etiology •Unknown. | ||
| Pathogenesis •Unknown. •Histogenesis of this tumor is also unknown., | ||
| Epidemiology •Mean age at the time of diagnosis is 11 months. •Male to female ratio: 1.5 to 1.0 | ||
| General Gross Description •Tumors are unilateral, usually less than 500 grams. •Tumors are soft and on cut section have a uniform appearance, bulge from the cut surface, and are well demarcated from the adjacent renal parenchyma. The tumor is light tan colored. •Examples: | ||
| General Microscopic Description •Unlike Wilms tumor, malignant rhabdoid tumors are monomorphous. •The tumor is composed of sheets of monotonous round to ovoid cells. •The characteristic tumor cells show eccentric nuclei due to ill-defined pale cytoplasmic inclusion bodes. •On electron microscopy, the intracytoplasmic inclusions are due to whorled aggregates of intermediate filaments. •Examples: | ||
| Clinical Correlation •Clinical presentation is similar to Wilms tumor with discovery of a renal mass in an infant. •Diagnosis is made histologically. •Malignant rhabdoid tumor is one of the most lethal of childhood malignancies. •Most patients die of their tumor within one year. | ||
| References •Murphy WM, Beckwith JB, and Farrow GM. Tumors of the Kidney, Bladder, and Related Urinary Structures. Washington, AFIP, 1994. Please be patient during transfer. Medline will open in a new window. To return, close the Medline Window Malignant Rhaboid Tumor
| Synopsis by: Harold Yamase M.D. (T71000M88003)[236]
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