Renal large cell neuroendocrine carcinoma

Renal large cell neuroendocrine carcinomas are an extremely rare renal tumor, with only six cases reported in the literature (c. 2022) ¹. 

Epidemiology 

Renal large cell neuroendocrine carcinoma represents <1% of the total reported cases of primary renal cancers. The most affected population is male adults with a mean age of 52.5 years ¹. 

Clinical presentation

Patients typically present with non-specific symptoms such as hematuria, flank pain, and palpable abdominal mass ¹. 

Pathology

Renal large cell neuroendocrine carcinoma grossly appears as a solid tan-to-grey cut surface with a large zone of necrosis and increased mitotic activity ^1,3,4.  

Associations

Renal large cell neuroendocrine carcinoma is frequently associated with squamous cell carcinoma or adenocarcinoma ². 

Radiographic features

CT

• heterogenous, exophytic mass with calcification ⁵

Treatment and prognosis

Surgical resection is currently the only curative treatment for renal large cell neuroendocrine carcinoma. The tumor is highly aggressive with a high risk of metastatic development, thus resulting in a poor prognosis ^ref.