Objectives: To report a new case of neuroendocrine renal cell carcinoma.

Methods: We report the case of a 76-year-old woman with neuroendocrine renal cell carcinoma who underwent radical nephrectomy without any further adjuvant treatment. We performed a bibliographic review about this rare renal neoplasia of which there are less than 20 published case reports.

Results: Patient is asymptomatic four years after surgery, although she has local recurrence.

Conclusions: Small cell renal cell carcinoma is a very rare neoplasia, affecting people over the age of 60 years, large in size, and metastatic at diagnosis. It has bad prognosis, with short survival times. The most adequate treatment has not been determined due to the scarcity of published cases; the combination of surgery and chemotherapy is the most frequently used.

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