Sandeep Kaur, Vandana Rana, Neeti Goyal, Divya Shelly, Amit Agrawal
Primary neuroendocrine tumors (NETs) of the kidney are exceedingly rare, with an estimated incidence of only 0.13 per 1 million individuals. Their rarity is attributed to the absence of native neuroendocrine cells within the renal parenchyma. These tumors span the WHO grading spectrum, with well-differentiated lesions reported most frequently. They may also occur in association with congenital anomalies such as horseshoe kidney. In this study, we present a new case of a 33-year-old female diagnosed with a primary renal NET at our hospital. She underwent imaging studies, surgical excision, and histopathological and immunohistochemical analysis. The findings suggest that these tumors are rare and have nonspecific clinical and imaging features. The diagnosis heavily relies on immunohistochemical analysis. Primary renal well-differentiated NETs are associated with low malignant potential and a favorable prognosis. Surgical resection is the preferred treatment, and long-term follow-up is necessary to monitor the patient's condition.