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Neuroendocrine Neoplasms

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Mediastinal Pathology
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Abstract

Primary mediastinal neuroendocrine neoplasms, as they are referred to in this chapter, encompass several tumors that do not necessarily share the same histogenesis, biologic behavior, or treatment modalities. In addition, some of these tumors may in turn occur as ectopically placed tissues that may be deposited in the mediastinal compartment. In some of these cases, the embryologic development may be the clue to their occurrence. Nevertheless, this group of tumors ranges from the benign tumors to the overtly high-grade malignancy. Basically, the group of tumors that more often present as mediastinal tumors are neuroendocrine carcinomas (well-differentiated neuroendocrine carcinoma (carcinoid tumor), moderately differentiated neuroendocrine carcinoma (atypical carcinoid), poorly differentiated neuroendocrine carcinoma (small cell carcinoma and large cell neuroendocrine carcinoma), paragangliomas, parathyroid tumors (parathyroid adenoma, parathyroid carcinoma), thyroid tumors (intrathoracic goiter, thyroid carcinoma). This chapter will be presented from the practical point of view for the practicing pathologist who may be called upon to interpret these tumors. Clinical, pathological, histopathological, immunohistochemical, radiological, gross, molecular features will be discussed, as well as differential diagnoses and staging.

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References

  1. Oldham HN, Sabiston DC. Primary tumors and cysts of the mediastinum. Arch Surg. 1968;96:71–5.

    Article  CAS  PubMed  Google Scholar 

  2. Wychulis AR, Payne WS, Clagett OT, Woolner LB. Surgical treatment of mediastinal tumors: a 40-year experience. J Thorac Cardiovasc Surg. 1971;62:379–92.

    Article  CAS  PubMed  Google Scholar 

  3. Blegvad S, Lippert H, Simper LB, Dybdahl H. Mediastinal tumours. Scand J Thor Cardiovasc Surg. 1990;24:39–42.

    CAS  Google Scholar 

  4. Hanagiri T, Hatooka S, Oosaki T, Nakanishi R, Mitsudomi T, Shirakusa T. Papillary carcinoma in a huge intrathoracic goiter with tracheal stenosis and invasion. Scand J Thor Cardiovasc Surg. 1993;27:165–8.

    CAS  Google Scholar 

  5. Adkins PB, Maples MD, Hainsworth JD. Primary malignant mediastinal tumors. Ann Thorac Surg. 1984;38:648–59.

    Article  PubMed  Google Scholar 

  6. 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 

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

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  9. Wick MR. Mediastinal cysts and intrathoracic thyroid tumors. Seminar in Diagnostic Pathology. 1990;7:285–94.

    CAS  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  11. 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 

  12. 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 

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

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    Article  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  18. Floros D, Dosios T, Tsourdis A, Yiatromanolakis N. Carcinoid tumor of the thymus with multiple endocrine adenomatosis. Path Res Pract. 1982;175:404–9.

    Article  CAS  PubMed  Google Scholar 

  19. Brown LR, Aughenbaugh GL, Wick MR, Baker BA, Salassa RM. Roentgenologic diagnosis of primary corticotropin-producing carcinoid tumor of the mediastinum. Radiology. 1982;142:143–8.

    Article  CAS  PubMed  Google Scholar 

  20. Thorner MO, Martin WH, Ragan GE, MacLeod RM, Feldman PS, Bruni C, et al. A case of ectopic ACTH syndrome: diagnostic difficulties caused by intermittent secretion. Acta Endcorinol. 1982;99:364–70.

    Article  CAS  Google Scholar 

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

    CAS  Google Scholar 

  22. Vener JD, Zuckerbraun L, Goodman D. Carcinoid tumor of the thymus associated with a parathyroid adenoma. Arch Otoloryngol. 1982;99:324–6.

    Article  Google Scholar 

  23. Huntrakoon M, Lin F, Heitz PU, Tomita T. 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 

  24. Lieske TR, Kincaid J, Sunderrajan JV. Tjhymic carcinoid with cutaneous hyperpigmentation. Ach Int Med. 1985;145:361–3.

    Article  CAS  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  26. Kimura N, Ishikawa T, Sasaki Y, Sasano N, Onodera K, Shimizu 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 

  27. Baker J, Holdaway IM, Jagush M, Kerr AR, Donald RA, Pullan PT. Ectopic secretion of ACTH and met-enkephalin from a thymic carcinoid. J Endocrinol Investig. 1982;5:33–8.

    Article  CAS  Google Scholar 

  28. Yamaji I, Iimura O, Mito T, Yoshida S, Shimamoto K, Minase T. 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 

  29. Zieger MA, Swartz SE, MacGillivray DC, Linnoila I, Shakir M. Thymic carcinoid in association with MEN syndromes. Am Surg. 1992;58:430–4.

    Google Scholar 

  30. Wollensak G, Herbst EW, Beck A, Schaefer HE. Primary thymic carcinoid with Cushing’s syndrome. Virch Arch A Pathol Anat. 1992;420:191–5.

    Article  CAS  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  32. 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 

  33. 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 

  34. 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 

  35. Teh BT, McArdle J, Chan SP, Menon J, Hartley L, Pullan P, et al. Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1. Medicine. 1997;76:21–9.

    Article  CAS  PubMed  Google Scholar 

  36. 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 

  37. 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 

  38. 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 

  39. 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 

  40. Wang DY, Chang DB, Kuo SH, Yang PC, Lee YC, Hsu HC, et al. Carcinoid tumours of the thymus. Thorax. 1994;49:357–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Cardillo G, Treggiari S, Paul MA, Carleo F, De Massimi AR, Remotti D, et al. Primary neuroendocrine tumours of the thymus: a cllinicopathologic and prognostic study of 19 patients. European J Cardio-thoracic Surgery. 2010;37:814–8.

    Article  Google Scholar 

  42. Cardillo G, Rea F, Lucchi M, Paul MA, Margaritora S, Carleo F, 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 

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

    Article  CAS  Google Scholar 

  44. 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 

  45. World Health Organization (WHO) Classification of Tumours. In: Travis WD, Brambilla E, Muller-Hermelink HK, editors. Patholgy & genetics, tumours of the lung, pleura, thymus, and heart. WHO; Lyon, France. 2004. p. 188–95.

    Google Scholar 

  46. Gal AA, Kornstein MJ, Cohen C, Duarte IG, Miller JI, Mansour KA. Neuroendocrine tumors of the thymus: a clinicopathological and prognostic study. Ann Thorac Surg. 2001;72:1179–82.

    Article  CAS  PubMed  Google Scholar 

  47. 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 

  48. Weissferdt A, Moran CA. Staging of primary mediastinal tumors. Adv Anat Pathol. 2013;20(1):1–9.

    Article  PubMed  Google Scholar 

  49. Valli M, Fabris GA, Dewar A, Chikte S, Fisher C, Corrin B, et al. Atypical carcinoid tumour of the thymus: a study of 8 cases. Histopathology. 1994;24:71–375.

    Google Scholar 

  50. Rosai J, editor. WHO Histological typing of tumours of the thymus. 2nd ed; Springer-Verlag Berlin. 1999.

    Google Scholar 

  51. Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG, editors. WHO classification of tumours of the lung, pleura, thymus, and heart; Springer-Verlag Berlin. 2015. p. 234–8.

    Google Scholar 

  52. Tsuta K, Raso MG, Kalhor N, Liu DD, Wistuba II, Moran CA. Histologic features of low- and intermediate-grade neuroendocrine carcinoma (typical and atypical carcinoid tumors) of the lung. Lung Cancer. 2011;71:34–41.

    Article  PubMed  Google Scholar 

  53. Hughes JP, Ancalmo N, Leonard GL, Ochsner JL. Carcinoid tumour of the thymus gland: report of a case. Thorax. 1975;30:470–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. Tanaka T, Tanaka S, Kimura H, Ito J. Mediastinal tumor of thymic origin and related to carcinoid tumor. Acta Path Jpn. 1974;24:413–26.

    CAS  Google Scholar 

  55. Sundstrom C, Wilander E. Thymic carcinoid: a case report. Acta Pathol Microbiol Scand Sect A. 1976;84:311–6.

    CAS  Google Scholar 

  56. Blom P, Johannessen JV. Mediastinal mass in a young male. Ultrastruct Pathol. 1983;4:391–5.

    Article  CAS  PubMed  Google Scholar 

  57. Llaverias S, Valls C, Picas E. Carcinoid tumor of the thymus. AJR. 1984;163:478.

    Article  Google Scholar 

  58. Kogan J. Carcinoid tumor of the thymus. Postgrad Med. 1984;75:291–6.

    Article  CAS  PubMed  Google Scholar 

  59. Chalk S, Donald KJ. Carcinoid tumor of the thymus. A case report including discussion of the morphological diagnosis and the cell or origin. Virchows Arch A Pathol Anat Histol. 1977;377:91–6.

    Article  CAS  PubMed  Google Scholar 

  60. Viebahn R, Hiddemann W, Klinke F, Bassewitz DB. Thymus carcinoid. Pathol Res Pract. 1985;180:445–8.

    Article  CAS  PubMed  Google Scholar 

  61. Salyer WR, Salyer DC, Eggleston JC. Carcinoid tumors of the thymus. Cancer. 1976;37:958–73.

    Article  CAS  PubMed  Google Scholar 

  62. Rao U, Takita H. Carcinoid tumour of possible thymic origin: case report. Thorax. 1977;32:771–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  63. Paties C, Zangrandi A, Vasallo G, Rinda G, Solcia E. Multidirectional carcinoma of the thymus with neuroendocrine and sarcomatoid components and carcinoid syndrome. Pathol Res Pract. 1991;187:170–7.

    Article  CAS  PubMed  Google Scholar 

  64. Kuo TT. Carcinoid tumor of the thymus with divergent sarcomatoid differentiation: report of a case with histogenetic considerations. Hum Pathol. 1994;25:319–23.

    Article  CAS  PubMed  Google Scholar 

  65. Mizuno T, Masaoka A, Hashimoto T, Shibata K, Yamakawa Y, Torii K, et al. Coexisting thymic carcinoid tumor and thymoma. Ann Thorac Surg. 1990;50:650–2.

    Article  CAS  PubMed  Google Scholar 

  66. 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 

  67. 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 

  68. Lagrange W, Dahm HH, Karstens J, Feichtinger J, Mittermayer C. Melanocytic neuroendocrine carcinoma of the thymus. Cancer. 1987;59:484–8.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  70. 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 

  71. 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 

  72. 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 

  73. 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 

  74. 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 

  75. 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 

  76. Wick MR, Scheithauer BW. Oat cell carcinoma of the thymus. Cancer. 1982;49:1652–7.

    Article  CAS  PubMed  Google Scholar 

  77. Chetty R, Batitang S, Govender D. Large cell neuroendocrine carcinoma of the thymus. Histopathology. 1997;31:274.

    Article  CAS  PubMed  Google Scholar 

  78. Miettinen M. Synpatophysin and neurofilament protein as markers for neuroendocrine tumors. Arch Pathol Lab Med. 1987;111:813–8.

    CAS  PubMed  Google Scholar 

  79. Herbst WM, Kummer W, Hofmann W, Otto H, Heym C. Carcinoid tumors of the thymus: an immunohistochemical study. Cancer. 1987;60:2465–70.

    Article  CAS  PubMed  Google Scholar 

  80. DeLellis RA, Wolfe HJ. Calcitonin in spindle cell thymic carcinoid tumors. Arch Pathol Lab Med. 1976;100:340.

    CAS  PubMed  Google Scholar 

  81. Wick MR, Scheithauer BW, Kovacs K. Neuron-specific enolase in neuroendocrine tumors of the thymus, bronchus, and skin. Am J Clin Pathol. 1983;79:703–7.

    Article  CAS  PubMed  Google Scholar 

  82. Weissferdt A, Tang X, Wistuba II, Moran CA. Comparative immunohistochemical analysis of pulmonary and thymic neuroendocrine carcinomas using PAX8 and TTF1. Mod Pathol. 2013;26:1554–60.

    Article  CAS  PubMed  Google Scholar 

  83. Weissferdt A, Kalhor N, Liu H, Rodriguez J, Fujimoto J, Tang X, 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 

  84. Kujari H, Joensuu H, Klemi P, Asola R, Nordman E. A flow cytometridc analysis of 23 carcinoid tumors. Cancer. 1988;61:2517–20.

    Article  CAS  PubMed  Google Scholar 

  85. Fetissof F, Boivin F, Jobar P, Arbeille-Brassart B, Romet L, Maillet M. Microfilamentous carcinoid of the thymus: correlation of ultratructural study with Grimelius stain. Ultrastruct Pathol. 1982;3:9–15.

    Article  CAS  PubMed  Google Scholar 

  86. Kay S, Willson MA. Ultrastructural studies of an ACTH-secreting thymic tumor. Cancer. 1970 Aug;26(2):445–52.

    Article  CAS  PubMed  Google Scholar 

  87. Moran CA, Rush W, Mena H. Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of 30 cases. Histopathology. 1997;31:167–73.

    Article  CAS  PubMed  Google Scholar 

  88. Moran CA, Albores-Saavedra J, Wenig BM, Mena H. Pigmented Extradrenal paragangliomas: a clinicopathologic and immunohistochemical study of five cases. Cancer. 1997;79:398–402.

    Article  CAS  PubMed  Google Scholar 

  89. 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 

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

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Article  Google Scholar 

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

    Article  Google Scholar 

  94. 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 

  95. 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 

  96. Enquist RW, Tormey DC, Jenis EH, Warkel RL. Malignant chemodectoma of the superior mediastinum with elevated urinary homovanillic acid. Chest. 1974;66:209–11.

    Article  CAS  PubMed  Google Scholar 

  97. Victor S, Anand KV, Andappan P, Bharathy S, Rao S, Reddy KC. Malignant mediastinal chemodectoma. Chest. 1975;68(4):583–4.

    Article  CAS  PubMed  Google Scholar 

  98. Arom V, Nicoloff DM. Intrathoracic paraganglioma arising from aorticosympathetic paraganglion. Arch Surg. 1976;111:275–9.

    Article  CAS  PubMed  Google Scholar 

  99. Lacquet LK, Moulijn AC, Jongerius CM, Rensing JB. Intrathoracic chemodectoma with multiple localisations. Thorax. 1977;32:203–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  100. Olson JL, Salyer WR. Mediastinal paragangliomas (Aortic Body Tumor): a report of four cases and a review of the literature. Cancer. 1978;41:2405–12.

    Article  CAS  PubMed  Google Scholar 

  101. Lack EE, Stillinger RA, Colvin DB, Groves RM, Burnette DG. Aortico-pulmonary paraganglioma: report of a case with ultrastructural study and review of the literature. Cancer. 1979;43:269–78.

    Article  CAS  PubMed  Google Scholar 

  102. Gallivan MVE, Chun B, Rowden G, Lack EE. Intrathoracic paravertebral malignant paraganglioma. Arch Pathol Lab Med. 1980;104:46–51.

    CAS  PubMed  Google Scholar 

  103. Grace MP, Batist G, Grace WR, Gillooley JF. Aorticopulmonary paraganglioma and gastric leiomyoblastoma in a young woman. Am J Med. 1981;70:1288–92.

    Article  CAS  PubMed  Google Scholar 

  104. Evora PRB, Bongiovani HL, Sgarbieri RN, Ribeiro PJ, Pereira LC, Moraes MM, et al. Nonfunctioning paraganglioma of the posterior mediastinum. Scand J Thorac Cardiovasc Surg. 1988;22:295–7.

    Article  CAS  PubMed  Google Scholar 

  105. Odze R, Begin LR. Malignant paraganglioma of the posterior mediastinum: a case report and review of the literature. Cancer. 1990;65:564–9.

    Article  CAS  PubMed  Google Scholar 

  106. Assaf HM, Al-Momen AA, Martin JG. Aorticopulmonary paraganglioma: a case report with immunohistochemical studies and literature review. Arch Pathol Lab Med. 1992;116:1085–7.

    CAS  PubMed  Google Scholar 

  107. 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 

  108. Plaza JA, Wakely PE, Moran CA, Fletcher CD, Suster S. Sclerosing paraganglioma: report of 19 cases of an unusual variant of neuroendocrine tumor that may be mistaken for an aggressive malignant neoplasm. Am J Surg Pathol. 2006;30:7–12.

    Article  PubMed  Google Scholar 

  109. Kimani S, Young WF. Hereditary paraganglioma-phechromocytoma syndromes. In: Pagaon RA, et al., editors. GeneReviews [internet]. Seattle: University of Washington, Seattle 1993–2015. 2008 May 21 [updated 2014 Nov 06].

    Google Scholar 

  110. Sato H, Shoji S, Kajiwara H, Itoh J, Osamura RY. A novel mutation (P236S) in the succinate dehydrogenase subunit B gene in a Japanese patient with posterior mediastinal paraganglioma. Endocr Pathol. 2013;24:144–8.

    Article  PubMed  Google Scholar 

  111. Garibaldi E, Bresciani S, Panaia R, Delmastro E, Malinverni G, Gabriele P. Hereditary paraganglioma syndrome associated with SDHD gene mutations: a patient with multicentric presentation treated with radiotherapy. Case report. Tumori. 2011;97:214–20.

    Article  CAS  PubMed  Google Scholar 

  112. Prodanov T, Havekes B, Nathanson KL, Adams KT, Pacak K. Malignant paraganglioma associated with succinate dehydrogenase subunit B in an 8-year-old child: the age of first screening? Pediatr Nephrol. 2009;24:1239–42.

    Article  PubMed  PubMed Central  Google Scholar 

  113. Ortega PF, Sosa LA, Patel M, Zambrano E. Cystic paraganglioma of the anterior mediastinum. Ann Diagn Pathol. 2010;14:341–6.

    Article  PubMed  Google Scholar 

  114. Flieder DB, Moran CA, Suster S. Primary alveolar soft part sarcoma of the mediastinum: a clinicopathological and immunohistochemical study of two cases. Histopathology. 1997;31:469–73.

    Article  CAS  PubMed  Google Scholar 

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

    Article  Google Scholar 

  116. Cope O. Surgery of hyperparathyroidism: the occurrence of parathryoids in the anterior mediastinum and the division of the operation into two stages. Ann Surg. 1941;114:706–33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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

    Google Scholar 

  118. 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 

  119. 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 

  120. Thacker WC, Wells VH, Hall ER. Parathryoid cyst of the mediastinum. Ann Surg. 1971;174:969–75.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  121. Margolis IB, Wayne R, Organ CH. Parathryoid cysts: functional and mediastinal. Surgery. 1975;77:462–6.

    CAS  PubMed  Google Scholar 

  122. Simkin EP. Hyperparathyroidism associated with a parathyroid cyst: an unusual presentation. Br J Surg. 1976;63:927–8.

    Article  CAS  PubMed  Google Scholar 

  123. Calandra DB, Shah KH, Prinz RA, Sullivan H, Hofmann C, Oslapas R, et al. Parathyroid cyst: a report of eleven cases including two associated with hyperparathyroid crisis. Surgery. 1983;94:887–92.

    CAS  PubMed  Google Scholar 

  124. Ramos-Gabatini A, Mallette LE, Bringhurst FR, Draper MW. Functional mediastinal parathyroid cyst: dynamics of parathyroid hormone secreting during cyst aspirations and surgery. Am J Med. 1985;79:633–9.

    Article  Google Scholar 

  125. Linos DA, Schoretsanitis G, Carvounis E. Parathyroid cysts of the neck and mediastinum. Acta Chir Scand. 1989;155:211–6.

    CAS  PubMed  Google Scholar 

  126. Petri N, Holten I. Parathyroid cyst: report of case in the mediastinum. J Laryngol Otol. 1990;104:56–7.

    Article  CAS  PubMed  Google Scholar 

  127. Downey RJ, Cerfolio RJ, Deschamps C, Grant CS, Pairolero PC. Mediastinal parathyroid cyst. Mayo Clin Proc. 1995;70:946–50.

    Article  CAS  PubMed  Google Scholar 

  128. 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 

  129. Scholz DA, Purnell DC, Woolner LB, Clagett OT. Mediastinal hyperfunctioning parathyroid tumors. Ann Surg. 1973;178:173–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  130. Wang C, Mahaffey JE, Axelrod L, Perlman JA. Hyperfunctioning supernumerary parathyroid glands. Surg Gynecol Obstet. 1979;148:711–4.

    CAS  PubMed  Google Scholar 

  131. Russell CF, Edis AJ, Scholz DA, Sheedy PF, van Heerden JA. Mediastinal parathyroid tumors: experience with 38 tumors requiring mediastinotomy for removal. Ann Surg. 1981;193:805–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  132. Clark OH. Mediastinal parathyroid tumors. Arch Surg. 1988;123:1096–100.

    Article  CAS  PubMed  Google Scholar 

  133. Conn JM, Goncalves MA, Mansour KA, McGarity WC. The mediastinal parathyroid. Am Surg. 1991;57:62–6.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  135. Schlinkert RT, Whitaker MD, Argueta R. Resection of select mediastinal parathyroid adenomas through an anterior mediastinotomy. Mayo Clin Proc. 1991;66:1110–3.

    Article  CAS  PubMed  Google Scholar 

  136. Murphy MN, Glennon PG, Diocee MS, Wick MR, Cavers DJ. 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 

  137. 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 

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

    Article  PubMed  Google Scholar 

  139. Avramides A, Papamargaritis K, Antoniadis A, Gakis D, Leontsini M. Large parathyroid functioning carcinoma (1,200 g) presenting as substernal goiter. J Endocrinol Investig. 1992;15:39–42.

    Article  CAS  Google Scholar 

  140. 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 

  141. Yoshiike F, Koizumi T, Yoneyama A, Komatu M, Yamaguchi S, Hanaoka M, et al. Thymic squamous cell carcinoma producing parathyroid hormone-related protein and CYFRA 21-1. Intern Med. 2004;43(6):493–5.

    Article  PubMed  Google Scholar 

  142. Hsiao LT, Chiou TJ, Yu IT, Chen PM. Superior vena cava syndrome and hypercalcaemia in a patient with primary mediastinal B-cell lymphoma secreting parathyroid hormone-related protein. Br J Haematol. 2002;119(1):1.

    Article  PubMed  Google Scholar 

  143. 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 

  144. Becker FO, Tausk K. Radiologically evident functioning mediastinal parathyroid adenoma. Chest. 1970;58:79–81.

    Article  CAS  PubMed  Google Scholar 

  145. College D, Rohati PK. Mediastinal parathyroid cyst. J Comput Assist Tomogr. 1983;7(1):140–2.

    Article  CAS  PubMed  Google Scholar 

  146. Hargreaves HK, Wright TC. A large functioning parathyroid lipoadenoma found in the posterior mediastinum. Am J Clin Pathol. 1981;76:89–93.

    Article  CAS  PubMed  Google Scholar 

  147. Hajhosseini B, Montazeri V, Hajhosseini L, Nezami N, Beygui RE. Mediastinal goiter: a comprehensive study of 60 consecutive cases with special emphasis on identifying predictors of malignancy and sternotomy. Am J Surg. 2012;203:442–7.

    Article  PubMed  Google Scholar 

  148. Zapatero J, Baamonde C, Gonzalez F, Orusco E, Gallardo MP, Folque E. Ectopic goiters of the mediastinum: presentation of two cases and review of the literature. Jpn J Surg. 1988;18:105–9.

    Article  CAS  PubMed  Google Scholar 

  149. Raffaelli M, De Crea C, Ronti S, Bellantone R, Lombardi CP. Substernal goiters: incidence, surgical approach, and complications in a tertiary care referral center. Head Neck. 2011;33:1420–5.

    Article  PubMed  Google Scholar 

  150. Erbil Y, Bozbora A, Barbaros U, Ozarmagan S, Azezli A, Movalilar S. Surgical management of substernal goiters: clilnical experience of 170 cases. Surg Today. 2004;34:732–4.

    Article  PubMed  Google Scholar 

  151. Gao B, Zhang X, Zhao J, Zhao J, He Y, Wen Y, et al. Surgical treatment of large substernal thyroid goiter: analysis of 12 patients. Int J Clin Exp Med. 2013;6:488–96.

    PubMed  PubMed Central  Google Scholar 

  152. Shasha AR. Substernal goiter: what is in a definition? Surgery. 2010;147:239–40.

    Article  Google Scholar 

  153. Huins CT, Georgalas C, Mehrzad H, Tolley NS. A new classification system for retrosternal goiter based on a systematic review of its complications and management. Int J Surg. 2008;6:71–6.

    Article  PubMed  Google Scholar 

  154. Barker TA, Daultrey CR, Trotter SE, Kalkat M. Intrathymic primary intrathoracic goiter in a patient with breast malignancy. Ann Thorac Surg. 2012;93:35–6.

    Article  Google Scholar 

  155. Landeta F, Hoffmeier A, Fuchs M, Scheld H, Maintz D, Stypmann J, et al. Ectopic thyroid mass in the heart. Lancet. 2012;379:1762.

    Article  PubMed  Google Scholar 

  156. Rios A, Rodriguez JM, Canteras M, Galindo PJ, Balsalobre MD, Parrilla P. Risk factors for malignancy in multinodular goiters. EJSO. 2004;30:58–62.

    Article  CAS  PubMed  Google Scholar 

  157. Bellafonte R, Lombardi CP, Bossola M, Fadda G, Salvatori M, Princi P. Posterior mediastinal hyperfunctioning insular thyroid carcinoma. Thorax. 2005;95:358–60.

    Google Scholar 

  158. Nervi M, Iacconi P, Spinelli C, Janni A, Miccoli P. Thyroid carcinoma in intrthoracic goiter. Langenbeck's Arch Surg. 1998;383:383–7.

    Article  Google Scholar 

  159. Oikonomou A, Astrinakis E, Giatromanolaki A, Karros P, Margaritis D, Prassopoulos P. Intrathoracic papillary thyroid carcinoma from occult primary disease. Gen Thorac Cardiovasc Surg. 2014;62:198–201.

    Article  PubMed  Google Scholar 

  160. Sand J, Pehkonen E, Mattila J, Seppănen S, Salmi J. Pulsating mass at the sternum: a primary carcinoma of ectopic mediastinal thyroid. J Thorac Cardiovasc Surg. 1996;112:833–5.

    Article  CAS  PubMed  Google Scholar 

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Kalhor, N., Moran, C. (2019). Neuroendocrine Neoplasms. In: Mediastinal Pathology . Springer, Cham. https://doi.org/10.1007/978-3-319-98980-8_9

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