Kidney Cancer pp 193-205 | Cite as

Neuroendocrine Kidney Tumors

  • Miao Zhang
  • Charles C. GuoEmail author


Kidney neuroendocrine tumors usually occur in the renal parenchyma and do not include those originate from the urothelial mucosa in the renal pelvis and calyces. Although they are rare, these neuroendocrine tumors demonstrate a wide spectrum of pathologic and clinical features. Several neuroendocrine tumors, including carcinoid, small cell carcinoma, paraganglioma, primitive neuroectodermal tumor, neuroblastoma, and juxtaglomerular cell tumor, are discussed here.


Carcinoid tumor Neuroendocrine carcinoma Primitive neuroectodermal tumor Neuroblastoma Juxtaglomerular cell tumor Metastatic tumor 


  1. 1.
    Yoo J, Park S, Jung Lee H, Jin Kang S, Kee Kim B. Primary carcinoid tumor arising in a mature teratoma of the kidney: a case report and review of the literature. Arch Pathol Lab Med. 2002;126(8):979–81.PubMedGoogle Scholar
  2. 2.
    Rodriguez-Covarrubias F, Gomez X, Valerio JC, Lome-Maldonado C, Gabilondo F. Carcinoid tumor arising in a horseshoe kidney. Int Urol Nephrol. 2007;39(2):373–6.CrossRefGoogle Scholar
  3. 3.
    Krishnan B, Truong LD, Saleh G, Sirbasku DM, Slawin KM. Horseshoe kidney is associated with an increased relative risk of primary renal carcinoid tumor. J Urol. 1997;157(6):2059–66.CrossRefGoogle Scholar
  4. 4.
    McCaffrey JA, Reuter VV, Herr HW, Macapinlac HA, Russo P, Motzer RJ. Carcinoid tumor of the kidney. The use of somatostatin receptor scintigraphy in diagnosis and management. Urol Oncol. 2000;5(3):108–11.CrossRefGoogle Scholar
  5. 5.
    Hansel DE, Epstein JI, Berbescu E, Fine SW, Young RH, Cheville JC. Renal carcinoid tumor: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2007;31(10):1539–44.CrossRefGoogle Scholar
  6. 6.
    el-Naggar AK, Troncoso P, Ordonez NG. Primary renal carcinoid tumor with molecular abnormality characteristic of conventional renal cell neoplasms. Diagn Mol Pathol. 1995;4(1):48–53.CrossRefGoogle Scholar
  7. 7.
    Goldblum JR, Lloyd RV. Primary renal carcinoid. Case report and literature review. Arch Pathol Lab Med. 1993;117(8):855–8.PubMedGoogle Scholar
  8. 8.
    La Rosa S, Bernasconi B, Micello D, Finzi G, Capella C. Primary small cell neuroendocrine carcinoma of the kidney: morphological, immunohistochemical, ultrastructural, and cytogenetic study of a case and review of the literature. Endocr Pathol. 2009;20(1):24–34.CrossRefGoogle Scholar
  9. 9.
    Tetu B, Ro JY, Ayala AG, Ordonez NG, Johnson DE. Small cell carcinoma of the kidney. A clinicopathologic, immunohistochemical, and ultrastructural study. Cancer. 1987;60(8):1809–14.CrossRefGoogle Scholar
  10. 10.
    Capella C, Eusebi V, Rosai J. Primary oat cell carcinoma of the kidney. Am J Surg Pathol. 1984;8(11):855–61.CrossRefGoogle Scholar
  11. 11.
    Casella R, Moch H, Rochlitz C, Meier V, Seifert B, Mihatsch MJ, et al. Metastatic primitive neuroectodermal tumor of the kidney in adults. Eur Urol. 2001;39(5):613–7.CrossRefGoogle Scholar
  12. 12.
    Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, et al. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol. 2002;26(3):320–7.CrossRefGoogle Scholar
  13. 13.
    Cuesta Alcala JA, Solchaga Martinez A, Caballero Martinez MC, Gomez Dorronsoro M, Pascual Piedrola I, Ripa Saldias L, et al. Primary neuroectodermal tumor (PNET) of the kidney: 26 cases. Current status of its diagnosis and treatment. Arch Esp Urol. 2001;54(10):1081–93.PubMedGoogle Scholar
  14. 14.
    Thyavihally YB, Tongaonkar HB, Gupta S, Kurkure PA, Amare P, Muckaden MA, et al. Primitive neuroectodermal tumor of the kidney: a single institute series of 16 patients. Urology. 2008;71(2):292–6.CrossRefGoogle Scholar
  15. 15.
    Parham DM, Roloson GJ, Feely M, Green DM, Bridge JA, Beckwith JB. Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms’ Tumor Study Group Pathology Center. Am J Surg Pathol. 2001;25(2):133–46.CrossRefGoogle Scholar
  16. 16.
    Lagace R, Tremblay M. Non-chromaffin paraganglioma of the kidney with distant metastases. Can Med Assoc J. 1968;99(22):1095–8.PubMedPubMedCentralGoogle Scholar
  17. 17.
    Robertson PW, Klidjian A, Harding LK, Walters G, Lee MR, Robb-Smith AH. Hypertension due to a renin-secreting renal tumour. Am J Med. 1967;43(6):963–76.CrossRefGoogle Scholar
  18. 18.
    Martin SA, Mynderse LA, Lager DJ, Cheville JC. Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature. Am J Clin Pathol. 2001;116(6):854–63.CrossRefGoogle Scholar
  19. 19.
    Hayami S, Sasagawa I, Suzuki H, Kubota Y, Nakada T, Endo Y. Juxtaglomerular cell tumor without hypertension. Scand J Urol Nephrol. 1998;32(3):231–3.CrossRefGoogle Scholar
  20. 20.
    Kuroda N, Moriki T, Komatsu F, Miyazaki E, Hayashi Y, Naruse K, et al. Adult-onset giant juxtaglomerular cell tumor of the kidney. Pathol Int. 2000;50(3):249–54.CrossRefGoogle Scholar
  21. 21.
    Moss AH, Peterson LJ, Scott CW, Winter K, Olin DB, Garber RL. Delayed diagnosis of juxtaglomerular cell tumor hypertension. N C Med J. 1982;43(10):705–7.PubMedGoogle Scholar
  22. 22.
    Haab F, Duclos JM, Guyenne T, Plouin PF, Corvol P. Renin secreting tumors: diagnosis, conservative surgical approach and long-term results. J Urol. 1995;153(6):1781–4.CrossRefGoogle Scholar
  23. 23.
    Msaouel P, Zhang M, Tu SM. Prolonged remission of upper urinary tract urothelial carcinoma with prominent choriocarcinomatous differentiation: a case report. Clin Genitourin Cancer. 2017;15(1):e73–e7.CrossRefGoogle Scholar
  24. 24.
    Govender D, Nteene LM, Chetty R, Hadley GP. Mature renal teratoma and a synchronous malignant neuroepithelial tumour of the ipsilateral adrenal gland. J Clin Pathol. 2001;54(3):253–4.CrossRefGoogle Scholar
  25. 25.
    Huang H, Tamboli P, Karam JA, Vikram R, Zhang M. Secondary malignancies diagnosed using kidney needle core biopsies: a clinical and pathological study of 75 cases. Hum Pathol. 2016;52:55–60.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of PathologyThe University of Texas MD Anderson Cancer CenterHoustonUSA

Personalised recommendations