RESUMO
INTRODUCTION: Neuroendocrine renal carcinoma represents less than 1% of all primary neoplasia of the kidney. Most frequently poorly differentiated carcinoma is diagnosed in advanced stages and they have an aggressive evolution and limited survival rate. Neuroendocrine carcinomas that arise from the renal pelvis are frequently associated with squamous cell carcinoma or adenocarcinoma. MATERIAL AND METHOD: We present the case of a female patient, known for 3 years before with an undefined retroperitoneal lymph node metastasis, being diagnosed at present with a left large cell neuroendocrine renal carcinoma, who initially had lymph node metastasis. RESULTS: Until now, 118 cases of primary neuroendocrine renal carcinomas have been reported. A limited number of poorly differentiated neuroendocrine carcinomas have been reported. DISCUSSION: Due to the clinical and biological findings, the aggressive evolution with early metastasis of lung and bone, the patient is included in the group of poorly differentiated carcinomas. In these cases, multimodal treatment is a gold standard. After surgical treatment and palliative chemotherapy with platinum salts, we obtained a partial remission of the disease and the control of symptoms. CONCLUSIONS: Regarding large cell neuroendocrine carcinoma, the surgical treatment remains the treatment of choice. Chemotherapy can determine limited results, improve the quality of life and enhance the overall survival rate.