Skip to main content

Neuroendocrine Neoplasms

  • Chapter
  • First Online:
The Thorax

Abstract

Neuroendocrine neoplasms represent an important group of tumors; however, the histological characteristics of these neoplasms are similar regardless of the site of origin. It is well known that these neoplasms may occur virtually in any anatomical site; therefore, clinical and radiological information becomes an important tool in the assessment of these neoplasms. From the immunohistochemical point of view, all these neoplasms will show similar immunophenotype using the more conventional and daily neuroendocrine markers such as chromogranin, synaptophysin, and CD56. In some cases adding TTF-1, CDX-2 may aid in the assessment of primary versus metastatic disease. However, the most important element in assigning site of origin should be based on the overall information of clinical, radiological, and pathological characteristics. More specifically regarding thoracic neoplasm, these tumors can be either primary neoplasm of the lung or of the thymus. Therefore, the use of radiological information in the assessment plays a crucial role, as the histology alone cannot determine primary site.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Pachter MR, Lattes R. Uncommon mediastinal tumors. Dis Chest. 1963;43:519–28.

    Article  CAS  PubMed  Google Scholar 

  2. Rosai J, Higa E. Mediastinal endocrine neoplasm of probable thymic origin related to carcinoid tumor. Cancer. 1972;29:1061–74.

    Article  CAS  PubMed  Google Scholar 

  3. Wick MR, Rosai J. Neuroendocrine neoplasms of the mediastinum. Semin Diagn Pathol. 1991;8:35–51.

    CAS  PubMed  Google Scholar 

  4. Wick MR. Mediastinal cysts and intrathoracic thyroid tumors. Semin Diagn Pathol. 1990;7:285–94.

    CAS  PubMed  Google Scholar 

  5. Klemm KM, Moran CA. Primary neuroendocrine carcinomas of the thymus. Semin Diagn Pathol. 1999;16:32–41.

    CAS  PubMed  Google Scholar 

  6. Moran CA. Primary neuroendocrine carcinomas of the mediastinum: review of current criteria for histopathologic diagnosis and classification. Semin Diagn Pathol. 2005;22:223–9.

    Article  PubMed  Google Scholar 

  7. Moran CA, Suster S. Neuroendocrine carcinomas (carcnoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma): current concepts. Hematol Oncol Clin North Am. 2007;21:395–407.

    Article  PubMed  Google Scholar 

  8. Manes JL, Taylor HB. Thymic carcnoid in familial multiple endocrine adenomatosis. Arch Pathol. 1973;95:252–5.

    CAS  PubMed  Google Scholar 

  9. Lockich JJ, Li F. Carcinoid of the thymus with hereditary hyperparathyroidism. Ann Intern Med. 1978;89:364–5.

    Article  Google Scholar 

  10. Marchevsky AM, Dikman SH. Mediastinal carcinoid with incomplete Sipple’s syndrome. Cancer. 1979;43:2497–501.

    Article  CAS  PubMed  Google Scholar 

  11. Stewart CA, Kingston CW. Carcinoid tumour of the thymus with Cushing’s syndrome. Pathology. 1980;12:487–94.

    Article  CAS  PubMed  Google Scholar 

  12. Floros D, Dosios T, Tsourdis A, et al. Carcinoid tumor of the thymus with multiple endocrine adenomatosis. Pathol Res Pract. 1982;175:404–9.

    Article  CAS  PubMed  Google Scholar 

  13. Brown LR, Aughenbaugh GL, Wick MR, et al. Roentgenologic diagnosis of primary corticotropin-producing carcinoid tumor of the mediastinum. Radiology. 1982;142:143–8.

    Article  CAS  PubMed  Google Scholar 

  14. Thorner MO, Martin WH, Ragan GE, et al. A case of ectopic ACTH syndrome: diagnostic difficulties caused by intermittent secretion. Acta Endcorinol. 1982;99:364–70.

    CAS  Google Scholar 

  15. Birnberg FA, Webb WR, Selch MT, et al. Thymic carcinoid tumors with hyperparathyroidism. Am J Radiol. 1982;139:1001–4.

    CAS  Google Scholar 

  16. Huntrakoon M, Lin F, Heitz PU, et al. Thymic carcinoid tumor with Cushing’s syndrome: report of a case with electron microscopic and immunoperoxidase studies for neuron-specific enolase and corticotropin. Arch Pathol Lab Med. 1984;108:551–4.

    CAS  PubMed  Google Scholar 

  17. Lieske TR, Kincaid J, Sunderrajan JV. Tjhymic carcinoid with cutaneous hyperpigmentation. Arch Intern Med. 1985;145:361–3.

    Article  CAS  PubMed  Google Scholar 

  18. Gartner LA, Voorhess ML. Adrenocorticotropin hormone-producing thymic carcinoid in a teenager. Cancer. 1993;71:106–11.

    Article  CAS  PubMed  Google Scholar 

  19. Kimura N, Ishikawa T, Sasaki Y, et al. Expression of prohormone convertase, PC2, in adrenocorticotropin-producing thymic carcinoid with elevated plasma corticotropin-releasing hormone. J Clin Endocrinol Metab. 1996;81:390–5.

    CAS  PubMed  Google Scholar 

  20. Baker J, Holdaway IM, Jagush M, et al. Ectopic secretion of ACTH and met-enkephalin from a thymic carcinoid. J Endocrinol Investig. 1982;5:33–8.

    Article  CAS  Google Scholar 

  21. Yamaji I, Iimura O, Mito T, et al. An ectopic ACTH producing oncocytic carcinoid tumor of the thymus: report of a case. Jpn J Med. 1984;23:62–6.

    Article  CAS  PubMed  Google Scholar 

  22. Zieger MA, Swartz SE, MacGillivray DC, et al. Thymic carcinoid in association with MEN syndromes. Am Surg. 1992;58:430–4.

    Google Scholar 

  23. Wollensak G, Herbst EW, Beck A, et al. Primary thymic carcinoid with Cushing’s syndrome. Virchcows Arch A Pathol Anat. 1992;420:191–5.

    Article  CAS  Google Scholar 

  24. Zahner J, Borchard F, Schmitz U, et al. Thymus carcinoid in multiple endocrine neoplasms type I. Dtsch Med Wochenschr. 1994;119:135–40.

    Article  CAS  PubMed  Google Scholar 

  25. Gelfand ET, Basualdo CA, Callagham JC. Carcinoid tumor of the thymus associated with recurrent pericarditis. Chest. 1981;79:350–1.

    Article  CAS  PubMed  Google Scholar 

  26. Pimstone BL, Uys CJ, Vogelpoel L. Studies in a case of Cushing’s syndrome due to an ACTH-producing thymic tumour. Am J Med. 1972;53:521–8.

    Article  CAS  PubMed  Google Scholar 

  27. Lowenthal RM, Gumpel JM, Kreel L, McLaughlin JE, Skeggs BL. Carcinoid tumour of the thymus with systemic manifestations: a radiological and pathological study. Thorax. 1974;29:553–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Teh BT, McArdle J, Chan SP, et al. Cliicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1. Medicine. 1997;76:21–9.

    Article  CAS  PubMed  Google Scholar 

  29. Rosai J, HIga E, Davie J. Mediastinal endocrine neoplasm in patients with multiple endocrine adenomatosis: a previously unrecognized association. Cancer. 1972;29:1075–83.

    Article  CAS  PubMed  Google Scholar 

  30. Wick MR, Scheithauer BW. Thymic carcinoid. A histologic, immunohistochemical, and ultrastructural study of 12 cases. Cancer. 1984;53:475–84.

    Article  CAS  PubMed  Google Scholar 

  31. Wick MR, Scott RE, Li CY, Carney JA. Carcinoid tumor of the thyus. A clinicopathologic report of seven cases with a review of the literature. Mayo Clin Proc. 1980;55:246–54.

    CAS  PubMed  Google Scholar 

  32. De Montpreville VT, Macchiarini P, Dulmet E. Thymic neuroendocrine carcinoma (carcinoid): a clinicopathologic study of fourteen cases. J Thorac Cardiovasc Surg. 1996;111:134–41.

    Article  PubMed  Google Scholar 

  33. Economopoulos GC, Lewis JW, Lee MW, Silverman NA. Carcinoid tumors of the thymus. Ann Thorac Surg. 1990;50:58–61.

    Article  CAS  PubMed  Google Scholar 

  34. Wang DY, Chang DB, Kuo SH, et al. Carcinoid tumours of the thymus. Thorax. 1994;49:357–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Cardillo G, Treggiari S, Paul MA, et al. Primary neuroendocrine tumours of the thymus: a cllinicopathologic and prognostic study of 19 patients. Eur J Cardiothorac Surg. 2010;37:814–8.

    Article  PubMed  Google Scholar 

  36. Cardillo G, Rea F, Lucchi M, et al. Primary neuroendocrine tumors of the thymus: a multicenter experience of 35 patients. Ann Thorac Surg. 2012;94:241–6.

    Article  PubMed  Google Scholar 

  37. Neary NM, Lopez-Chavez A, Abel BS, et al. Neuroendocrine ACTH-producing tumor of the thymus – experience with 12 patients over 25 years. J Clin Endocrinol Metab. 2012;97:2223–30.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Moran CA, Suster S. Neuroendocrine carcinomas (Carcinoid Tumor) of the thymus. A clinicopathologic analysis of 80 cases. Am J Clin Pathol. 2000;114:100–10.

    Article  CAS  PubMed  Google Scholar 

  39. Rosado de Christenson ML, Abbott GF, Kirejczyk WM, Galvin JR, Travis WD. Thoracic carcinoids: radiologic-pathologic correlation. Radiographics. 1999;19:707–36.

    Article  CAS  PubMed  Google Scholar 

  40. Valli M, Fabris GA, Dewar A, et al. Atypical carcinoid tumour of the thymus: a study of 8 cases. Histopathology. 1994;24:71–375.

    Google Scholar 

  41. Weissferdt A, Moran CA. Thymic carcinoma, part 2: a clinicopathologic correlation of 33 cases with a proposed staging system. Am J Clin Pathol. 2012;138:115–21.

    Article  PubMed  Google Scholar 

  42. Moran CA, Lindholm KE, Brunnstrom H, et al. Typical and atypical carcinoid tumors of the lung: a clinicopathological correlation of 783 cases with emphasis on histological features. Hum Pathol. 2020;98:98–109.

    Article  PubMed  Google Scholar 

  43. Suster S, Moran CA. Thymic carcinoid with prominent mucinous stroma: report of a distinctive morphologic variant of thymic neuroendocrine neoplasm. Am J Surg Pathol. 1995;19:1277–85.

    Article  CAS  PubMed  Google Scholar 

  44. Moran CA, Suster S. Angiomatoid neuroendocrine carcinoma of the thymus: report of a distinctive morphological variant of neuroendocrine tumor of te thymus resembling a vascular neoplasm. Hum Pathol. 1999;30:635–9.

    Article  CAS  PubMed  Google Scholar 

  45. Lagrange W, Dahm HH, Karstens J, et al. Melanocytic neuroendocrine carcinoma of the thymus. Cancer. 1987;59:484–8.

    Article  CAS  PubMed  Google Scholar 

  46. Ho FCS, Ho JCI. Pigmented carcinoid tumour of the thymus. Histopathology. 1977;1:363–9.

    Article  CAS  PubMed  Google Scholar 

  47. Klemm KM, Moran CA, Suster S. Pigmented thymic carcinoids: a clinicopathological and immunohistochemical study of two cases. Mod Pathol. 1999;12:946–8.

    CAS  PubMed  Google Scholar 

  48. Moran CA, Suster S. Spindle cell neuroendocrine carcinomas of the thymus: (spindle cell thymic carcinoid): a clinicopathologic and immunohistochemical study of seven cases. Mod Pathol. 1999;12:587–91.

    CAS  PubMed  Google Scholar 

  49. Levine G, Rosai J. A spindle cell variant of thymic carcinoid tumor. A clinical, histologic, and fine structural study with emphasis on its distinction from spindle cell thymoma. Arch Pathol Lab Med. 1976;100:293–300.

    CAS  PubMed  Google Scholar 

  50. Moran CA, Suster S. Primary neuroendocrine carcinoma (thymic carcinoid) of the thymus with prominent oncocytic features: a clinicopathologic study of 22 cases. Mod Pathol. 2000;13:489–94.

    Article  CAS  PubMed  Google Scholar 

  51. Moran CA, Suster S. Cystic well-differentiated neuroendocrine carcinoma (carcinoid tumor): a clinicopathologic and immunohistochemical study of two cases. Am J Clin Pathol. 2006;126:377–80.

    Article  PubMed  Google Scholar 

  52. Moran CA, Suster S. Thymic neuroendocrine carcinomas with combined features ranging from well differentiated (carcinoid) to small cell carcinoma. Am J Clin Pathol. 2000;113:345–50.

    Article  CAS  PubMed  Google Scholar 

  53. Weissferdt A, Kalhor N, Liu H, et al. Thymic neuroendocrine tumors (paraganglioma and carcinoid tumors): a comparative immunohistochemical study of 46 cases. Hum Pathol. 2014;45:2463–70.

    Article  PubMed  Google Scholar 

  54. Phillips B. Intrathoracic pheochromocytoma. Arch Pathol. 1940;30:916–21.

    Google Scholar 

  55. Lattes R. Nonchromaffin paraganglioma of ganglion nodosum, carotid body, and aortic arch bodies. Cancer. 1950;3:667–94.

    Article  CAS  PubMed  Google Scholar 

  56. Marshall RB, Horn RC. Nonchromaffin paraganglioma: a comparative study. Cancer. 1961;4:779–87.

    Article  Google Scholar 

  57. Green WO, Bassett FH. Extrathoracic pheochromocytoma: report of a case. Am J Clin Pathol. 1965;35(2):142–6.

    Article  Google Scholar 

  58. Tama L, Ellis H, Hodgson CH, Dockerty MB. Chemodectoma of the mediastinum: report on a patient with superior vena caval obstruction treated by a shunt from the right innominate vein to the right atrium. J Thorac Cardiovasc Surg. 1962;43:585–94.

    Article  CAS  PubMed  Google Scholar 

  59. Pachter RM. Mediastinal nonchromaffin paraganglioma: a clinicopathologic study based on eight cases. J Thorac Cardiovasc Surg. 1963;45:152–9.

    Article  CAS  PubMed  Google Scholar 

  60. Dajee A, Dajee H, Hinrichs S, Lillington G. Pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma. Carney’s tirad. J Thorac Cardiovasc Surg. 1982;84:377–81.

    Article  CAS  PubMed  Google Scholar 

  61. Moran CA, Suster S, Fishback N, Koss MN. Mediastinal paragangliomas: a clinicopathologic and immunohistochemical study of 16 cases. Cancer. 1993;72:2358–64.

    Article  CAS  PubMed  Google Scholar 

  62. Daye D, Ackman JB. Characterization of mediastinal masses by MRI: techniques and applications. Appl Radiol. 2017;46:10–22.

    Article  Google Scholar 

  63. Nakazono T, White CS, Yamasaki F, et al. MRI findings of mediastinal neurogenic tumors. AJR Am J Roentgenol. 2011;197:W643–52.

    Article  PubMed  Google Scholar 

  64. Olsen WL, Dillon WP, Kelly WM, Norman D, Brant-Zawadzki M, Newton TH. MR imaging of paragangliomas. AJR Am J Roentgenol. 1987;148:201–4.

    Article  CAS  PubMed  Google Scholar 

  65. Brewer LA. The occurrence of parathyroid tissue within the thymus: report of four cases. Endocrinology. 1934;18(3):397–408.

    Article  Google Scholar 

  66. Bauer W, Federman DD. Hyperparathryoidism epitomized: the case of captain Charles E. Martell. Metabolism. 1962;XI(1):21–9.

    Google Scholar 

  67. Hardy JD, Snavely JR, Langford HG. Low intrathoracic parathyroid adenoma: large functioning tumor representing fifth parathyroid, opposite eight dorsal vertebra with independent arterial supply and opacified at operation with arteriogram. Ann Surg. 1964;159:310–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Nathaniels EK, Nathaniels AM, Wang C. Mediastinal parathyroid tumors: a clinical and pathological study of 84 cases. Ann Surg. 1970;171(2):165–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Downey RJ, Cerfolio RJ, Deschamps C, et al. Mediastinal parathyroid cyst. Mayo Clin Proc. 1995;70:946–50.

    Article  CAS  PubMed  Google Scholar 

  70. Gurbuz AT, Peetz ME. Giant mediastinal parathyroid cyst: an unusual cause of hypercalcemic crisis—case report and review of the literature. Surgery. 1996;120:795–800.

    Article  CAS  PubMed  Google Scholar 

  71. Roslyn JJ, Gordon HE, Mulder DG. Mediastinal parathyroid adenomas: a cause of persistent hyperparathyroidism. Am Surg. 1983;49:523–7.

    CAS  PubMed  Google Scholar 

  72. Murphy MN, Glennon PG, Diocee MS, et al. Nonsecretory parathyroid carcinoma of the mediastinum: light microscopic, immunohistochemical, and ultrastructural features of a case, and review of the literature. Cancer. 1986;58:2468–76.

    Article  CAS  PubMed  Google Scholar 

  73. Kastan DJ, Kottamasu SR, Frame B, Greenwald KA. Carcinoma in a mediastinal fifth parathyroid gland. JAMA. 1987;257(9):1218–9.

    Article  CAS  PubMed  Google Scholar 

  74. Putnam JB, Schantz SP, Pugh WC, et al. Extended en bloc resection of a primary mediastinal parathyroid carcinoma. Ann Thorac Surg. 1990;50:138–40.

    Article  PubMed  Google Scholar 

  75. Avramides A, Papamargaritis K, Antoniadis A, et al. Large parathyroid functioning carcinoma (1,200 g) presenting as substernal goiter. J Endocrinol Investig. 1992;15:39–42.

    Article  CAS  Google Scholar 

  76. Kelly MD, Sheridan BF, Farnsworth AE, Palfreeman S. Parathyroid carcinoma in a mediastinal sixth parathyroid gland. Aust N Z J Surg. 1994;64:446–9.

    Article  CAS  PubMed  Google Scholar 

  77. Moran CA, Suster S. Primary parathyroid tumors of the mediastinum: a clinicopathologic and immunohistochemical study of 17 cases. Am J Clin Pathol. 2005;124:749–54.

    Article  PubMed  Google Scholar 

  78. Carter BW, Benveniste MF, Madan R, et al. ITMIG classification of mediastinal compartments and multidisciplinary approach to mediastinal masses. Radiographics. 2017;37:413–36.

    Article  PubMed  Google Scholar 

  79. Carter BW, Okumura M, Detterbeck FC, Marom EM. Approaching the patient with an anterior mediastinal mass: a guide for radiologists. J Thorac Oncol. 2014;9:S110–8.

    Article  PubMed  Google Scholar 

  80. Bahl M, Muzaffar M, Vij G, Sosa JA, Choudhury KR, Hoang JK. Prevalence of the polar vessel sign in parathyroid adenomas on the arterial phase of 4D CT. AJNR Am J Neuroradiol. 2014;35:578–81.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  81. Kukar M, Platz TA, Schaffner TJ, et al. The use of modified four-dimensional computed tomography in patients with primary hyperparathyroidism: an argument for the abandonment of routine sestamibi single-positron emission computed tomography (SPECT). Ann Surg Oncol. 2015;22:139–45.

    Article  PubMed  Google Scholar 

  82. Rodgers SE, Hunter GJ, Hamberg LM, et al. Improved preoperative planning for directed parathyroidectomy with 4-dimensional computed tomography. Surgery. 2006;140:932–40; discussion 40-1

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Cesar A. Moran .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Strange, C.D., de Groot, P.M., Truong, M.T., Moran, C.A. (2023). Neuroendocrine Neoplasms. In: Moran, C.A., Truong, M.T., de Groot, P.M. (eds) The Thorax. Springer, Cham. https://doi.org/10.1007/978-3-031-21040-2_5

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-21040-2_5

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-21039-6

  • Online ISBN: 978-3-031-21040-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics