Abdominal Radiology

, Volume 42, Issue 5, pp 1472–1484 | Cite as

Neuroendocrine neoplasms of the genitourinary tract in adults: cross-sectional imaging spectrum

  • Venkata S. KatabathinaEmail author
  • Raghu Vikram
  • Adeniran Olaoya
  • Raj Mohan Paspulati
  • Marlo M. Nicolas
  • Priya Rao
  • Atif Zaheer
  • Srinivasa R. Prasad
Pictorial Essay


Neuroendocrine (NE) neoplasms of the genitourinary (GU) tract in adults are rare tumors with distinct histopathology and variable biological behavior and imaging findings. They may be primary or metastatic in origin. The spectrum of primary GU tract NE neoplasms includes carcinoid, small cell carcinoma, large cell NE carcinoma, and paraganglioma. The tumors commonly show positivity to specific immunohistochemical markers and characteristic dense-core granules at the ultra-structural level. Although imaging findings are nonspecific and accurate differentiation from the more common malignancies of the individual organs is not possible, cross-sectional imaging modalities play an important role in the diagnosis, staging, and surveillance of these tumors. Somatostatin receptor scintigraphy (octreotide scan) may be useful in the detection and treatment of metastatic disease in select patients. Knowledge of the various NE tumors of the adult GU tract and familiarity with their pathological and imaging findings permit optimal patient management.


Neuroendocrine Tumors Genitourinary Imaging 


Compliance with ethical standards


No funding was received for this study.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

Statement of informed consent was not applicable since the manuscript does not contain any patient data.


  1. 1.
    Eble JN, Sauter G, Epstein JI, Sesterhenn IA (eds) (2004) World Health Organization classification of tumours:pathology and genetics of tumours of the urinary system and male genital organs. Lyon: IARCGoogle Scholar
  2. 2.
    Fine SW (2007) Neuroendocrine lesions of the genitourinary tract. Adv Anat Pathol 14(4):286–296. doi: 10.1097/PAP.0b013e3180ca8a89 CrossRefPubMedGoogle Scholar
  3. 3.
    Jimenez RE, Folpe AL, Lapham RL, et al. (2002) Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol 26(3):320–327CrossRefGoogle Scholar
  4. 4.
    Dickersin GR, Kline IW, Scully RE (1982) Small cell carcinoma of the ovary with hypercalcemia: a report of eleven cases. Cancer 49(1):188–197CrossRefGoogle Scholar
  5. 5.
    Witkowski L, Goudie C, Foulkes WD, McCluggage WG (2016) Small-cell carcinoma of the ovary of hypercalcemic type (malignant rhabdoid tumor of the ovary): a review with recent developments on pathogenesis. Surg Pathol Clin 9(2):215–226CrossRefGoogle Scholar
  6. 6.
    Mazzucchelli R, Morichetti D, Lopez-Beltran A, et al. (2009) Neuroendocrine tumours of the urinary system and male genital organs: clinical significance. BJU Int 103(11):1464–1470. doi: 10.1111/j.1464-410X.2009.08451.x CrossRefPubMedGoogle Scholar
  7. 7.
    Lane BR, Chery F, Jour G, et al. (2007) Renal neuroendocrine tumours: a clinicopathological study. BJU Int 100(5):1030–1035. doi: 10.1111/j.1464-410X.2007.07116.x CrossRefPubMedGoogle Scholar
  8. 8.
    Lane BR, Jour G, Zhou M (2009) Renal neuroendocrine tumors. Indian J Urol 25(2):155–160. doi: 10.4103/0970-1591.52905 CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Murali R, Kneale K, Lalak N, Delprado W (2006) Carcinoid tumors of the urinary tract and prostate. Arch Pathol Lab Med 130(11):1693–1706PubMedGoogle Scholar
  10. 10.
    Omiyale AO, Venyo AK (2013) Primary carcinoid tumour of the kidney: a review of the literature. Adv Urol 2013:579396. doi: 10.1155/2013/579396 CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Moulopoulos A, DuBrow R, David C, Dimopoulos MA (1991) Primary renal carcinoid: computed tomography, ultrasound, and angiographic findings. J Comput Assist Tomogr 15(2):323–325CrossRefGoogle Scholar
  12. 12.
    McKeown DK, Nguyen GK, Rudrick B, Johnson MA (1988) Carcinoid of the kidney: radiologic findings. AJR Am J Roentgenol 150(1):143–144CrossRefGoogle Scholar
  13. 13.
    Tanaka T, Yamamoto H, Imai A, et al. (2015) A case of primary renal carcinoid tumor. Case Rep Urol 2015:736213PubMedPubMedCentralGoogle Scholar
  14. 14.
    Romero FR, Rais-Bahrami S, Permpongkosol S, et al. (2006) Primary carcinoid tumors of the kidney. J Urol 176(6 Pt 1):2359–2366. doi: 10.1016/j.juro.2006.07.129 CrossRefPubMedGoogle Scholar
  15. 15.
    Mufarrij P, Varkarakis IM, Studeman KD, Jarrett TW (2005) Primary renal carcinoid tumor with liver metastases detected with somatostatin receptor imaging. Urology 65(5):1002. doi: 10.1016/j.urology.2004.11.042 CrossRefPubMedGoogle Scholar
  16. 16.
    La Rosa S, Bernasconi B, Micello D, Finzi G, Capella C (2009) Primary small cell neuroendocrine carcinoma of the kidney: morphological, immunohistochemical, ultrastructural, and cytogenetic study of a case and review of the literature. Endocr Pathol 20(1):24–34. doi: 10.1007/s12022-008-9054-y CrossRefPubMedGoogle Scholar
  17. 17.
    Dundr P, Pesl M, Povysil C, Bauerova L, Soukup V (2010) Primary large cell neuroendocrine carcinoma of the kidney. Pathol Oncol Res 16(1):139–142. doi: 10.1007/s12253-009-9180-y CrossRefPubMedGoogle Scholar
  18. 18.
    Lee H, Cho JY, Kim SH, et al. (2009) Imaging findings of primitive neuroectodermal tumors of the kidney. J Comput Assist Tomogr 33(6):882–886. doi: 10.1097/RCT.0b013e31819e938b CrossRefPubMedGoogle Scholar
  19. 19.
    Chea YW, Agrawal R, Poh AC (2008) Primitive neuroectodermal tumour of the kidney: radiologic-pathological correlations. Hong Kong Med J 14(3):240–243PubMedGoogle Scholar
  20. 20.
    Chu WC, Reznikov B, Lee EY, et al. (2008) Primitive neuroectodermal tumour (PNET) of the kidney: a rare renal tumour in adolescents with seemingly characteristic radiological features. Pediatr Radiol 38(10):1089–1094. doi: 10.1007/s00247-008-0971-1 CrossRefPubMedGoogle Scholar
  21. 21.
    Hari S, Jain TP, Thulkar S, Bakhshi S (2008) Imaging features of peripheral primitive neuroectodermal tumours. Br J Radiol 81(972):975–983. doi: 10.1259/bjr/30073320 CrossRefPubMedGoogle Scholar
  22. 22.
    Wu CF, Wang LJ, Chen CS (2000) Images in clinical urology. Magnetic resonance imaging of primitive neuroectodermal tumor of the kidney. Urology 55(2):284–285CrossRefGoogle Scholar
  23. 23.
    Thyavihally YB, Tongaonkar HB, Gupta S, et al. (2008) Primitive neuroectodermal tumor of the kidney: a single institute series of 16 patients. Urology 71(2):292–296. doi: 10.1016/j.urology.2007.09.051 CrossRefPubMedGoogle Scholar
  24. 24.
    Pant-Purohit M, Lopez-Beltran A, Montironi R, MacLennan GT, Cheng L (2010) Small cell carcinoma of the urinary bladder. Histol Histopathol 25(2):217–221PubMedGoogle Scholar
  25. 25.
    Kim JC, Kim KH, Jung S (2003) Small cell carcinoma of the urinary bladder: cT and MR imaging findings. Korean J Radiol 4(2):130–135CrossRefGoogle Scholar
  26. 26.
    Blunt DM, Sansom HE, King DM (1996) Imaging of small cell carcinoma of the male urogenital tract. Clin Radiol 51(10):724–727CrossRefGoogle Scholar
  27. 27.
    Wong-You-Cheong JJ, Woodward PJ, Manning MA, Sesterhenn IA (2006) From the Archives of the AFIP: neoplasms of the urinary bladder: radiologic-pathologic correlation. Radiographics 26(2):553–580. doi: 10.1148/rg.262055172 CrossRefPubMedGoogle Scholar
  28. 28.
    Asbury WL Jr, Hatcher PA, Gould HR, Reeves WA, Wilson DD (1996) Bladder pheochromocytoma with ring calcification. Abdom Imaging 21(3):275–277CrossRefGoogle Scholar
  29. 29.
    Wang H, Ye H, Guo A, et al. (2010) Bladder paraganglioma in adults: MR appearance in four patients. Eur J Radiol . doi: 10.1016/j.ejrad.2010.09.020 CrossRefPubMedGoogle Scholar
  30. 30.
    Qiao HS, Feng XL, Yong L, et al. (2007) The MRI of extraadrenal pheochromocytoma in the abdominal cavity. Eur J Radiol 62(3):335–341. doi: 10.1016/j.ejrad.2007.02.041 CrossRefPubMedGoogle Scholar
  31. 31.
    Zhou M, Epstein JI, Young RH (2004) Paraganglioma of the urinary bladder: a lesion that may be misdiagnosed as urothelial carcinoma in transurethral resection specimens. Am J Surg Pathol 28(1):94–100CrossRefGoogle Scholar
  32. 32.
    Wang W, Epstein JI (2008) Small cell carcinoma of the prostate. A morphologic and immunohistochemical study of 95 cases. Am J Surg Pathol 32(1):65–71. doi: 10.1097/PAS.0b013e318058a96b CrossRefPubMedGoogle Scholar
  33. 33.
    Schwartz LH, LaTrenta LR, Bonaccio E, et al. (1998) Small cell and anaplastic prostate cancer: correlation between CT findings and prostate-specific antigen level. Radiology 208(3):735–738CrossRefGoogle Scholar
  34. 34.
    Chang JM, Lee HJ, Lee SE, et al. (2008) Pictorial review: Unusual tumours involving the prostate: radiological-pathological findings. Br J Radiol 81(971):907–915. doi: 10.1259/bjr/68294775 CrossRefPubMedGoogle Scholar
  35. 35.
    Yilmaz M, Celen Z, Sevinc A, Karakok M (2009) Widespread metastases in small cell carcinoma of the prostate on FDG PET/CT. Clin Nucl Med 34(9):598–600. doi: 10.1097/RLU.0b013e3181b06aed CrossRefPubMedGoogle Scholar
  36. 36.
    Furtado P, Lima MV, Nogueira C, Franco M, Tavora F (2011) Review of small cell carcinomas of the prostate. Prostate Cancer 2011:543272. doi: 10.1155/2011/543272 CrossRefPubMedPubMedCentralGoogle Scholar
  37. 37.
    Yao JL, Madeb R, Bourne P, et al. (2006) Small cell carcinoma of the prostate: an immunohistochemical study. Am J Surg Pathol 30(6):705–712CrossRefGoogle Scholar
  38. 38.
    Ulbright TM, Young RH (2004) Carcinoid tumor of the testis. Am J Clin Pathol 121(2):297 (author reply 298)PubMedGoogle Scholar
  39. 39.
    Wang WP, Guo C, Berney DM, et al. (2010) Primary carcinoid tumors of the testis: a clinicopathologic study of 29 cases. Am J Surg Pathol 34(4):519–524. doi: 10.1097/PAS.0b013e3181d31f33 CrossRefPubMedGoogle Scholar
  40. 40.
    Stroosma OB, Delaere KP (2008) Carcinoid tumours of the testis. BJU Int 101(9):1101–1105. doi: 10.1111/j.1464-410X.2007.07360.x CrossRefPubMedGoogle Scholar
  41. 41.
    Hodzic J, Golka K, Schulze H (2004) Primary testicular carcinoid. Med Sci Monit 10(8):CS46–CS48PubMedGoogle Scholar
  42. 42.
    Frederick LR, Ballek NK, Esplin JA, Kohler TS (2010) Primary carcinoid tumor presenting as a nonpalpable testicular mass. J Clin Oncol 28(31):e637–e639. doi: 10.1200/jco.2010.29.7457 CrossRefPubMedGoogle Scholar
  43. 43.
    Son HY, Ra SW, Jeong JO, et al. (2004) Primary carcinoid tumor of the bilateral testis associated with carcinoid syndrome. Int J Urol 11(11):1041–1043. doi: 10.1111/j.1442-2042.2004.00925.x CrossRefPubMedGoogle Scholar
  44. 44.
    Park SB, Kim JK, Cho KS (2006) Imaging findings of a primary bilateral testicular carcinoid tumor associated with carcinoid syndrome. J Ultrasound Med 25(3):413–416CrossRefGoogle Scholar
  45. 45.
    Modlin IM, Shapiro MD, Kidd M (2005) An analysis of rare carcinoid tumors: clarifying these clinical conundrums. World J Surg 29(1):92–101. doi: 10.1007/s00268-004-7443-z CrossRefPubMedGoogle Scholar
  46. 46.
    Davis KP, Hartmann LK, Keeney GL, Shapiro H (1996) Primary ovarian carcinoid tumors. Gynecol Oncol 61(2):259–265. doi: 10.1006/gyno.1996.0136 CrossRefPubMedGoogle Scholar
  47. 47.
    Strosberg J, Nasir A, Cragun J, Gardner N, Kvols L (2007) Metastatic carcinoid tumor to the ovary: a clinicopathologic analysis of seventeen cases. Gynecol Oncol 106(1):65–68. doi: 10.1016/j.ygyno.2007.02.034 CrossRefPubMedGoogle Scholar
  48. 48.
    Chaowalit N, Connolly HM, Schaff HV, Webb MJ, Pellikka PA (2004) Carcinoid heart disease associated with primary ovarian carcinoid tumor. Am J Cardiol 93(10):1314–1315. doi: 10.1016/j.amjcard.2004.01.075 CrossRefPubMedGoogle Scholar
  49. 49.
    Outwater EK, Siegelman ES, Hunt JL (2001) Ovarian teratomas: tumor types and imaging characteristics. Radiographics 21(2):475–490CrossRefGoogle Scholar
  50. 50.
    Takeuchi M, Matsuzaki K, Uehara H (2011) Primary carcinoid tumor of the ovary: MR imaging characteristics with pathologic correlation. Magn Reson Med Sci: MRM 10(3):205–209CrossRefGoogle Scholar
  51. 51.
    Young RH, Oliva E, Scully RE (1994) Small cell carcinoma of the ovary, hypercalcemic type. A clinicopathological analysis of 150 cases. Am J Surg Pathol 18(11):1102–1116CrossRefGoogle Scholar
  52. 52.
    Eichhorn JH, Young RH, Scully RE (1992) Primary ovarian small cell carcinoma of pulmonary type. A clinicopathologic, immunohistologic, and flow cytometric analysis of 11 cases. Am J Surg Pathol 16(10):926–938CrossRefGoogle Scholar
  53. 53.
    Eichhorn JH, Young RH (2001) Neuroendocrine tumors of the genital tract. Am J Clin Pathol 115(Suppl):S94–112PubMedGoogle Scholar
  54. 54.
    Schleef J, Wagner A, Kleta R, et al. (1999) Small-cell carcinoma of the ovary of the hypercalcemic type in an 8-year-old girl. Pediatr Surg Int 15(5–6):431–434CrossRefGoogle Scholar
  55. 55.
    Bakhru A, Liu JR, Lagstein A (2012) A case of small cell carcinoma of the ovary hypercalcemic variant in a teenager. Gynecol Oncol Case Rep 2(4):139–142. doi: 10.1016/j.gynor.2012.09.001 CrossRefPubMedPubMedCentralGoogle Scholar
  56. 56.
    Dykgraaf RH, de Jong D, van Veen M, et al. (2009) Clinical management of ovarian small-cell carcinoma of the hypercalcemic type: a proposal for conservative surgery in an advanced stage of disease. Int J Gynecol Cancer 19(3):348–353. doi: 10.1111/IGC.0b013e3181a1a116 CrossRefPubMedGoogle Scholar
  57. 57.
    Distelmaier F, Calaminus G, Harms D, et al. (2006) Ovarian small cell carcinoma of the hypercalcemic type in children and adolescents: a prognostically unfavorable but curable disease. Cancer 107(9):2298–2306. doi: 10.1002/cncr.22213 CrossRefPubMedGoogle Scholar
  58. 58.
    Estel R, Hackethal A, Kalder M, Munstedt K (2011) Small cell carcinoma of the ovary of the hypercalcaemic type: an analysis of clinical and prognostic aspects of a rare disease on the basis of cases published in the literature. Arch Gynecol Obstet 284(5):1277–1282. doi: 10.1007/s00404-011-1846-5 CrossRefPubMedGoogle Scholar
  59. 59.
    Kurasaki A, Sakurai N, Yamamoto Y, et al. (2013) Ovarian pulmonary-type small cell carcinoma: case report and review of the literature. Int J Gynecol Pathol 32(5):464–470. doi: 10.1097/PGP.0b013e31826d7ea8 CrossRefPubMedGoogle Scholar
  60. 60.
    Suzuki N, Kameyama K, Hirao T, et al. (2007) A case of pulmonary type of ovarian small cell carcinoma. J Obstet Gynaecol Res 33(2):203–206. doi: 10.1111/j.1447-0756.2007.00494.x CrossRefPubMedGoogle Scholar
  61. 61.
    Matsumoto H, Takai N, Nasu K, Narahara H (2011) Small cell carcinoma of the endometrium: a report of two cases. J Obstet Gynaecol Res 37(11):1739–1743. doi: 10.1111/j.1447-0756.2011.01593.x CrossRefPubMedGoogle Scholar
  62. 62.
    Tavassoli FA, Devilee P (2003) World Health Organization classification of tumours: pathology and genetics of tumours of the breast and female genital organs. Lyon: IARCGoogle Scholar
  63. 63.
    Tognini G, Ferrozzi F, Bini P, et al. (2002) Small cell carcinoma of the uterine corpus: CT appearance. Clin Imaging 26(2):133–135CrossRefGoogle Scholar
  64. 64.
    Tamai K, Koyama T, Saga T, et al. (2007) Small cell carcinoma of the uterine corpus: MR imaging and pathological correlation. J Comput Assist Tomogr 31(3):485–489. doi: 10.1097/01.rct.0000243452.33610.d4 CrossRefPubMedGoogle Scholar
  65. 65.
    van Hoeven KH, Hudock JA, Woodruff JM, Suhrland MJ (1995) Small cell neuroendocrine carcinoma of the endometrium. Int J Gynecol Pathol 14(1):21–29CrossRefGoogle Scholar
  66. 66.
    Conner MG, Richter H, Moran CA, Hameed A, Albores-Saavedra J (2002) Small cell carcinoma of the cervix: a clinicopathologic and immunohistochemical study of 23 cases. Ann Diagn Pathol 6(6):345–348. doi: 10.1053/adpa.2002.36661 CrossRefPubMedGoogle Scholar
  67. 67.
    Stoler MH, Mills SE, Gersell DJ, Walker AN (1991) Small-cell neuroendocrine carcinoma of the cervix. A human papillomavirus type 18-associated cancer. Am J Surg Pathol 15(1):28–32CrossRefGoogle Scholar
  68. 68.
    Abeler VM, Holm R, Nesland JM, Kjorstad KE (1994) Small cell carcinoma of the cervix. A clinicopathologic study of 26 patients. Cancer 73(3):672–677CrossRefGoogle Scholar
  69. 69.
    Yang DH, Kim JK, Kim KW, et al. (2004) MRI of small cell carcinoma of the uterine cervix with pathologic correlation. AJR Am J Roentgenol 182(5):1255–1258. doi: 10.2214/ajr.182.5.1821255 CrossRefPubMedGoogle Scholar
  70. 70.
    Gardner GJ, Reidy-Lagunes D, Gehrig PA (2011) Neuroendocrine tumors of the gynecologic tract: a Society of Gynecologic Oncology (SGO) clinical document. Gynecol Oncol 122(1):190–198. doi: 10.1016/j.ygyno.2011.04.011 CrossRefPubMedGoogle Scholar
  71. 71.
    Collinet P, Lanvin D, Declerck D, et al. (2000) Neuroendocrine tumors of the uterine cervix. Clinicopathologic study of five patients. Eur J Obstet Gynecol Reprod Biol 91(1):51–57CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Venkata S. Katabathina
    • 1
    Email author
  • Raghu Vikram
    • 2
  • Adeniran Olaoya
    • 5
  • Raj Mohan Paspulati
    • 3
  • Marlo M. Nicolas
    • 6
  • Priya Rao
    • 7
  • Atif Zaheer
    • 4
  • Srinivasa R. Prasad
    • 2
  1. 1.Department of RadiologyUniversity of Texas Health Science Center at San AntonioSan AntonioUSA
  2. 2.Department of RadiologyUniversity of Texas MD Anderson Cancer CenterHoustonUSA
  3. 3.Department of RadiologyCase Western Reserve UniversityClevelandUSA
  4. 4.Department of RadiologyJohns Hopkins UniversityBaltimoreUSA
  5. 5.University of Texas School of Public HealthHoustonUSA
  6. 6.Department of PathologyUniversity of Texas Health Science Center at San AntonioSan AntonioUSA
  7. 7.Department of PathologyUniversity of Texas MD Anderson Cancer CenterHoustonUSA

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