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.
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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.