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Oncocytoma
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- Sometimes referred to as the "seventh sickle cell nephropathy", this is a highly aggressive renal neoplasm that has been linked to sickle cell disease.
- This tumor has also been considered to represent the "aggressive" form of collecting duct carcinoma (which is, in itself, aggressive!).
- Clinical features: young patients, male > female (2:1), sickle cell trait, short duration of symptoms (< 5 months on average).
- Gross features: large tumors (average: 7 cm), with hemorrhage, necrosis, and satellite nodules.
- Histologic features:
- the most common pattern is that of a "reticular" or yolk-sac-like pattern; other patterns: microcystic, adenoid cystic, micropapillary , and solid sheets).
- cells have abundant eosinophilic cytoplasm with large nuclei and prominent nucleoli (somewhat "rhabdoid" in appearance).
- hemorrhage and necrosis are common with a striking neutrophilic infiltrate and formation of "microabscesses"
- Immunohistochemistry: cytokeratin and EMA positive; CEA +/-.
- Prognosis: All of the patients in the original report died within 3-52 weeks of diagnosis.; chemo and radiation do not appear to help.
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