Renal Neuroendocrine Tumor: A Case Report with Evaluation of Grading Criteria and Relationship with Carbonic Anhydrase 9 Immunoreactivity

Neuroendocrine tumors (NETs) are only exceptionally primary to the kidney. At present, scant information is known regarding the behavior and prognosis of renal NETs, especially according to the assessment of grading parameters used for NETs originating from other more commonplace sites such as the p...

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Bibliographic Details
Published in:International journal of surgical pathology 2024-05, Vol.32 (3), p.570-577
Main Authors: Monroe, Hunter L., Patadji Santiago, Stell, Williams, H. James, El Naili, Reima
Format: Article
Language:English
Subjects:
Carbonic Anhydrase IX
Carcinoma, Renal Cell - pathology
Humans
Kidney - pathology
Kidney cancer
Kidney Neoplasms - pathology
Metastasis
Neoplasm Grading
Neuroendocrine tumors
Neuroendocrine Tumors - pathology
Pancreatic Neoplasms - pathology
Thymus gland
von Hippel-Lindau Disease - complications
von Hippel-Lindau Disease - pathology
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Summary:Neuroendocrine tumors (NETs) are only exceptionally primary to the kidney. At present, scant information is known regarding the behavior and prognosis of renal NETs, especially according to the assessment of grading parameters used for NETs originating from other more commonplace sites such as the pancreas and lungs. There are only rare reports of grade assessment in renal NETs, with most of these reports relying upon now antiquated World Health Organization gastroenteropancreatic and lung/thymus criteria. As an additional prognostic factor, positive CA9 staining in NETs may correlate with elevated grade, stage and risk of metastasis while serving as a potential target of chemotherapy and immunotherapy and indicator of Von Hippel–Lindau Syndrome. Rarer still are descriptions of renal NETs presenting with renal cell carcinoma in the ipsilateral or contralateral kidney. Thus, we present a patient with a primary renal NET of the right kidney with regional lymphovascular invasion and distant metastasis with an emphasis on grading criteria concordant with the World Health Organization 2022 gastroenteropancreatic and lung/thymus systems. In addition, we discuss unusual staining for CA9 in the patient's tumor and a concomitant left kidney clear cell renal cell carcinoma that may act as a clinicopathologic mimic of Von Hippel–Lindau Syndrome.
ISSN:1066-8969
1940-2465
DOI:10.1177/10668969231186931