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1998 Presentations for the Poster and Vignette Sessions
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PRIMARY RHABDOID TUMOR OF THE LUNG. Donald H Strong, MD, Michael Erdmann, MD, Medical College of Wisconsin.

Case: A 44 yo white male presents from an outside facility with increasing lethargy, chest x­ray findings of a cavitary pulmonary nodule and a C.T. scan of the head with findings of multiple posterior fossa nodules. Shortly after arrival, the patient developed respiratory arrest and clinical signs consistent with uncal herniation which was confirmed with a C.T. scan. A percutaneous ventriculostomy tube was placed without improvement in clinical status and ventilatory support was withdrawn. On autopsy, the patient had a single lung nodule, and multiple brain and small bowel lesions consistent with a Rhabdoid tumor by histology, special stains, and electron microscopy.

Discussion: Rhabdoid tumors, first described in 1978, were initially thought to represent a Rhabdomyosarcomatous patten of an especially aggressive Wilm's tumor in the kidneys of children, but has been found to be histologically dissimilar. The majority of Rhabdoid tumors are well defined types of tumors, i.e., Adenocarcinoma and soft tissue sarcomas, with a component of Rhabdoid cells. Extra renal sites include many organ systems. To date, only 01 cases of primary lung Rhabdoid tumors have been reported. Rhabdoid cells represent a common phenotypic endpoint of clonal evolution that serves as a marker for poor prognosis. Little is known about treatment options for this particular tumor type. What is clear, is that rhabdoid cells, regardless of tissue type, confer a more aggressive behavior than similar tumors without a rhabdoid component.

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