Single institutional experience on primary neuroendocrine neoplasms of the kidney: a rare distinct entity

Primary pure renal neuroendocrine neoplasms (R-NENs) are a distinct and rare entity. Not much is known about the histopathology and biologic behavior of these tumors. We attempted to review the clinicopathologic aspects of these neoplasms encountered at our institution. We performed a retrospective chart review to identify primary pure (not admixed with any other tumor component) R-NENs from institutional Cancer Registry database. Pathologic review of the diagnostic archival slides was done for detailed assessment of the histologic features. R-NENs were classified according to the current WHO system for gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs). Eight pure R-NEN cases were identified, all unifocal, and most (6/8) involved the right kidney. Three patients had poorly differentiated neuroendocrine carcinoma (NEC), and five had well-differentiated neuroendocrine tumor (NET). All tumors were located near the renal hilum, stained diffusely with synaptophysin, variably with chromogranin, and were negative for renal site-specific marker PAX8 or for markers of renal cell carcinoma. We identified two distinct patterns of growth: one of sheets with interspersed rosettes and the other of large nests with low proliferative crowded centers and peripheral cells with higher proliferation and prominent palisading. Based on Ki-67 proliferative index, the tumors were classifiable into WHO grade 1 or grade 2 (based on GEP-NEN). All three NECs characteristically showed cytologic features intermediate between classic large and small cell type. This is the first comprehensive clinicopathologic study involving the rare group of R-NEN. Classifying and grading them according to the GEP-NEN system is of prognostic significance.