Primary Large Cell Neuroendocrine Carcinoma of the Kidney

Abstract

We report a case of a 56-year-old male with a primary large cell neuroendocrine renal carcinoma. Grossly, the left kidney was enlarged by a solid tumor that measured 145 x 125 x 100 mm. Histologically, the tumor consisted of large cells with a moderate to abundant amount of eosinophilic cytoplasm. The nuclei were irregular, some of them with finely or coarsely granular chromatin, others with vesicular chromatin and prominent nucleoli. The tumor cells showed multiple mitotic figures (up to 32 mitoses/10 HPF). In some areas, the tumor cells were arranged in solid sheets; however, the predominant pattern was solid-alveolar, trabecular and cribriform. Large areas of tumor necrosis were found. Immunohistochemically, the tumor cells were positive for synaptophysin, CD56 and CD57. Cytokeratin AE1/AE3, vimentin and CD10 were positive only focally. Chromogranin showed weak cytoplasmic positivity in rare tumor cells. Cytokeratin CAM5.2, cytokeratin 34betaE12, BerEP 4, EMA, TTF-1, cytokeratin 7, cytokeratin 20, calretinin, serotonin, somatostatin, gastrin, calcitonin, glukagon and insulin were negative. Primary large cell neuroendocrine carcinoma of the kidney is a rare tumor. To the best of our knowledge, only 3 cases of a tumor of this type have been reported to date.

Primary Large Cell Neuroendocrine Carcinoma of the Renal Pelvis: A Case Report

“…Neuroendocrine tumors of the kidney are subdivided into epithelial and neural types (4). Neural types of renal NE tumors include primitive neuroectodermal tumor, neuroblastoma and paraganglioma and usually arise in the renal parenchyma.…”

Primary neuroendocrine carcinoma of the kidney

“…One such cavity occupied a considerable part of the tumor. Less frequently, large cell neuroendocrine carcinoma is seen 21,22 . Some authors 23,24 claim that neuroendocrine carcinoma of the renal pelvis is more frequently associated with transitional cell carcinoma, adenocarcinoma or squamous cell carcinoma than with the same tumors arising in the renal parenchyma.…”

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

“…Death is seen in around 75% of patients within a year of diagnosis. Primary renal large cell NEC is an extremely rare malignancy; fewer than 10 cases have been reported in the literature [14,[19][20][21][22][23][24]. Renal paragangliomas are rare, and to date, very few cases have been reported in the literature [25].…”