Skip to main content
Log in

The spectrum of ectopic thymomas

  • Review and Perspectives
  • Published:
Virchows Archiv Aims and scope Submit manuscript

Abstract

Thymomas are rare tumors that usually manifest in the anterosuperior mediastinum. Occasionally, thymomas may also originate from ectopically dispersed thymic tissue and can arise in locations such as the neck, lung, or pleura or other locations in the thoracic cavity. The occurrence of thymomas in these ectopic locations can cause substantial diagnostic difficulty as the entity is almost never included in the differential diagnosis and its biphasic morphology can cause further complications during the diagnostic process. In this review, we summarize the clinical and pathological spectrum of ectopic thymomas and discuss the histogenesis, treatment, and prognosis of these extraordinary tumors.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Engels EA (2010) Epidemiology of thymoma and associated malignancies. J Thorac Oncol 5:S260–S265

    Article  PubMed  PubMed Central  Google Scholar 

  2. Engels EA, Pfeiffer RM (2003) Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies. Int J Cancer 105:546–551

    Article  CAS  PubMed  Google Scholar 

  3. Rosai J, Levine G. Tumors of the thymus. Atlas of tumor pathology, second series, fascicle 13. Armed Forces Institute of Pathology, Washington, DC. 1976.

  4. Cordier AC, Haumont SM (1980) Development of thymus, parathyroids, and ultimo-branchial bodies in NMRI and nude mice. Am J Anat 157:227–263

    Article  CAS  PubMed  Google Scholar 

  5. Gilmour J (1941) Some developmental abnormalities of the thymus and parathyroids. J Pathol Bacteriol 52:213–218

    Article  Google Scholar 

  6. Loney DA, Bauman NM (1998) Ectopic cervical thymic masses in infants: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 43:77–84

    Article  CAS  PubMed  Google Scholar 

  7. Norris EH (1938) The morphogenesis and histogenesis of the thymus gland in man: in which the origin of the Hassall’s corpuscles of the human thymus is discovered. Contrib Embryol 27:193

    Google Scholar 

  8. Chan JK, Rosai J (1991) Tumors of the neck showing thymic or related branchial pouch differentiation: a unifying concept. Hum Pathol 22:349–367

    Article  CAS  PubMed  Google Scholar 

  9. Rosai J, Limas C, Husband EM (1984) Ectopic hamartomatous thymoma. A distinctive benign lesion of lower neck. Am J Surg Pathol 8:501–513

    Article  CAS  PubMed  Google Scholar 

  10. Fetsch JF, Laskin WB, Michal M et al (2004) Ectopic hamartomatous thymoma: a clinicopathologic and immunohistochemical analysis of 21 cases with data supporting reclassification as a branchial anlage mixed tumor. Am J Surg Pathol 28:1360–1370

    Article  PubMed  Google Scholar 

  11. Moran CA, Suster S, Fishback NF et al (1995) Primary intrapulmonary thymoma. A clinicopathologic and immunohistochemical study of eight cases. Am J Surg Pathol 19:304–312

    Article  CAS  PubMed  Google Scholar 

  12. Myers PO, Kritikos N, Bongiovanni M et al (2007) Primary intrapulmonary thymoma: a systematic review. Eur J Surg Oncol 33:1137–1141

    Article  CAS  PubMed  Google Scholar 

  13. Marchevsky AM (1995) Lung tumors derived from ectopic tissues. Semin Diagn Pathol 12:172–184

    CAS  Google Scholar 

  14. Allen MS Jr, Drash EC (1968) Primary melanoma of the lung. Cancer 21:154–159

    Article  PubMed  Google Scholar 

  15. Drlicek M, Grisold W, Lorber J et al (1991) Pulmonary meningioma. Immunohistochemical and ultrastructural features. Am J Surg Pathol 15:455–459

    Article  CAS  PubMed  Google Scholar 

  16. Tang CK, Toker C, Foris NP et al (1978) Glomangioma of the lung. Am J Surg Pathol 2:103–109

    Article  CAS  PubMed  Google Scholar 

  17. Fukayama M, Maeda Y, Funata N et al (1988) Pulmonary and pleural thymoma. Diagnostic application of lymphocyte markers to the thymoma of unusual site. Am J Clin Pathol 89:617–621

    Article  CAS  PubMed  Google Scholar 

  18. Castleman B, Norris EH (1949) The pathology of the thymus in myasthenia gravis; a study of 35 cases. Medicine (Baltimore) 28:27–58

    Article  CAS  Google Scholar 

  19. Kinoshita T, Yoshida J, Ishii G et al (2012) Pulmonary metastasis from encapsulated cervical ectopic type a thymoma. Ann Thorac Surg 94:e141–e142

    Article  PubMed  Google Scholar 

  20. Yamanaka S, Yamamoto T, Watanabe K et al (2009) Metastatic ectopic cervical thymoma with prolonged survival; report of a case. Kyobu Geka 62:594–597

    CAS  PubMed  Google Scholar 

  21. Martin JM, Randhawa G, Temple WJ (1986) Cervical thymoma. Arch Pathol Lab Med 110:354–357

    CAS  PubMed  Google Scholar 

  22. Oh YL, Ko YH, Ree HJ (1998) Aspiration cytology of ectopic cervical thymoma mimicking a thyroid mass. A case report. Acta Cytol 42:1167–1171

    Article  CAS  PubMed  Google Scholar 

  23. Mourra N, Duron F, Parc R et al (2005) Cervical ectopic thymoma: a diagnostic pitfall on frozen section. Histopathology 46:583–585

    Article  CAS  PubMed  Google Scholar 

  24. Taweevisit M, Sampatanukul P, Thorner PS (2013) Ectopic thymoma can mimic benign and malignant thyroid lesions on fine needle aspiration cytology: a case report and literature review. Acta Cytol 57:213–220

    Article  PubMed  Google Scholar 

  25. Ridenhour CE, Henzel JH, DeWeese MS et al (1970) Thymoma arising from undescended cervical thymus. Surgery 67:614–619

    CAS  PubMed  Google Scholar 

  26. Bakshi J, Ghosh S, Pragache G et al (2005) Ectopic cervical thymoma in the submandibular region. J Otolaryngol 34:223–226

    Article  PubMed  Google Scholar 

  27. Mende S, Moschopulos M, Marx A et al (2004) Ectopic micronodular thymoma with lymphoid stroma. Virchows Arch 444:397–399

    Article  PubMed  Google Scholar 

  28. Jung JI, Kim HH, Park SH et al (1999) Malignant ectopic thymoma in the neck: a case report. AJNR Am J Neuroradiol 20:1747–1749

    CAS  PubMed  Google Scholar 

  29. Boman K (1941) On thymusfoeraendringar via myasternier. Nord Med 10:1625–1634

    Google Scholar 

  30. Noh JM, Ha SY, Ahn YC et al (2015) Potential role of adjuvant radiation therapy in cervical thymic neoplasm involving thyroid gland or neck. Cancer Res Treat 47:436–440

    Article  CAS  PubMed  Google Scholar 

  31. Yan B, Lim D, Petersson F (2010) Ectopic cervical thymoma: a report of two cases of a rare entity frequently misdiagnosed on fine needle aspiration cytology and frozen section. Head Neck Pathol 4:152–156

    Article  PubMed  PubMed Central  Google Scholar 

  32. Chang ST, Chuang SS (2003) Ectopic cervical thymoma: a mimic of T-lymphoblastic lymphoma. Pathol Res Pract 199:633–635

    Article  PubMed  Google Scholar 

  33. Choi H, Koh SH, Park MH et al (2008) Myasthenia gravis associated with ectopic cervical thymoma. J Clin Neurosci 15:1393–1395

    Article  PubMed  Google Scholar 

  34. Milde P, Sidawy MK (1999) Thymoma presenting as a palpable thyroid nodule: a pitfall in fine needle aspiration (FNA) of the neck. Cytopathology 10:415–419

    Article  CAS  PubMed  Google Scholar 

  35. Yokoyama S, Hayashida R, Yoshiyama K et al (2015) Ectopic cervical thymoma excised through a transcervical approach combined with video-assisted thoracoscopic surgery: a case report. Ann Thorac Cardiovasc Surg 21:293–297

    Article  PubMed  Google Scholar 

  36. McBurney R, Clagett OT, McDonald J (1951) Primary intrapulmonary neoplasm (thymoma?) associated with myasthenia gravis: report of case. Proc Staff Meet Mayo Clin 26:345–353

    CAS  PubMed  Google Scholar 

  37. Stefani A, Boulenger E, Mehaut S et al (2007) Primary intrapulmonary thymoma associated with congenital hyperhomocysteinemia. J Thorac Cardiovasc Surg 134:799–801

    Article  PubMed  Google Scholar 

  38. Gunaldi M, Oguz Kara I, Duman BB et al (2012) Primary intrapulmonary thymoma associated with myasthenia gravis. Gen Thorac Cardiovasc Surg 60:610–613

    Article  PubMed  Google Scholar 

  39. Kung IT, Loke SL, So SY et al (1985) Intrapulmonary thymoma: report of two cases. Thorax 40:471–474

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Katsura M, Kouso H, Shikada Y et al (2015) Primary intrapulmonary thymoma. Gen Thorac Cardiovasc Surg 63:56–59

    Article  PubMed  Google Scholar 

  41. Moran CA, Travis WD, Rosado-de-Christenson M et al (1992) Thymomas presenting as pleural tumors. Report of eight cases. Am J Surg Pathol 16:138–144

    Article  CAS  PubMed  Google Scholar 

  42. Hofmann W, Möller P, Manke H et al (1985) Thymoma: a clinicopathologic study of 98 cases with special reference to three unusual cases. Pathology-Research and Practice 179:337–353

    Article  CAS  Google Scholar 

  43. Honma K, Shimada K (1986) Metastasizing ectopic thymoma arising in the right thoracic cavity and mimicking diffuse pleural mesothelioma—an autopsy study of a case with review of literature. Wien Klin Wochenschr 98:14–20

    CAS  PubMed  Google Scholar 

  44. Payne CB Jr, Morningstar WA, Chester EH (1966) Thymoma of the pleura masquerading as diffuse mesothelioma. Am Rev Respir Dis 94:441–446

    PubMed  Google Scholar 

  45. Filosso PL, Delsedime L, Cristofori RC et al (2012) Ectopic pleural thymoma mimicking a giant solitary fibrous tumour of the pleura. Interact Cardiovasc Thorac Surg 15:930–932

    Article  PubMed  PubMed Central  Google Scholar 

  46. Kitada M, Sato K, Matsuda Y et al (2011) Ectopic thymoma presenting as a giant intrathoracic tumor: a case report. World J Surg Oncol 9:66

    Article  PubMed  PubMed Central  Google Scholar 

  47. Higashiyama M, Doi O, Kodama K et al (1996) Ectopic primary pleural thymoma: report of a case. Surg Today 26:747–750

    Article  CAS  PubMed  Google Scholar 

  48. Fushimi H, Tanio Y, Kotoh K (1998) Ectopic thymoma mimicking diffuse pleural mesothelioma: a case report. Hum Pathol 29:409–410

    Article  CAS  PubMed  Google Scholar 

  49. Kim HS, Lee HJ, Cho SY et al (2007) Myasthenia gravis in ectopic thymoma presenting as pleural masses. Lung Cancer 57:115–117

    Article  PubMed  Google Scholar 

  50. Yamazaki K, Yoshino I, Oba T et al (2007) Ectopic pleural thymoma presenting as a giant mass in the thoracic cavity. Ann Thorac Surg 83:315–317

    Article  PubMed  Google Scholar 

  51. Skoutelis K, Nikolopoulos DD, Markopoulos K et al (2009) Ectopic thymoma of the lung; a rare case report and review of the literature. Cases J 2:9149

    Article  PubMed  PubMed Central  Google Scholar 

  52. Asa SL, Dardick I, Van Nostrand AW et al (1988) Primary thyroid thymoma: a distinct clinicopathologic entity. Hum Pathol 19:1463–1467

    Article  CAS  PubMed  Google Scholar 

  53. Lewis JE, Wick MR, Scheithauer BW et al (1987) Thymoma. A clinicopathologic review. Cancer 60:2727–2743

    Article  CAS  PubMed  Google Scholar 

  54. Cohen JB, Troxell M, Kong CS et al (2003) Ectopic intrathyroidal thymoma: a case report and review. Thyroid 13:305–308

    Article  PubMed  Google Scholar 

  55. Weissferdt A, Moran CA (2016) Ectopic primary intrathyroidal thymoma: a clinicopathological and immunohistochemical analysis of 3 cases. Hum Pathol 49:71–76

    Article  CAS  PubMed  Google Scholar 

  56. Mirra M, Zanella M, Bussani R et al (1997) Intrapericardial thymoma: report of two incidental autopsy cases and review of the literature. Arch Pathol Lab Med 121:59–63

    CAS  PubMed  Google Scholar 

  57. Iliceto S, Quagliara D, Calabrese P et al (1984) Visualization of pericardial thymoma and evaluation of chemotherapy by two-dimensional echocardiography. Am Heart J 107:605–606

    Article  CAS  PubMed  Google Scholar 

  58. Ben Hami B, Caulet-Maugendre S, Valla J et al (1996) Primary pericardial thymoma: an unusual etiology of neoplastic pericarditis. Ann Pathol 16:445–448

    CAS  PubMed  Google Scholar 

  59. Salih Deveci M, Ceyhan K, Deveci G et al (2001) Pericardial rhabdomyomatous spindle cell thymoma with mucinous cystic degeneration. Histopathology 38:479–481

    Article  CAS  PubMed  Google Scholar 

  60. Theodore S, Parija C, Neelakandhan KS (2004) Giant ectopic pericardial thymoma. Indian Journal of Thoracic and Cardiovascular Surgery 20:102–103

    Article  Google Scholar 

  61. Azoulay S, Adem C, Gatineau M et al (2005) Pericardial ectopic thymoma. Virchows Arch 446:185–188

    Article  PubMed  Google Scholar 

  62. Arai H, Rino Y, Fushimi K et al (2015) Pericardial ectopic thymoma presenting with cardiac tamponade: report of a case. Surg Today 45:1200–1204

    Article  PubMed  Google Scholar 

  63. Kanzaki M, Oyama K, Ikeda T et al (2004) Noninvasive thymoma in the middle mediastinum. Ann Thorac Surg 77:2209–2210

    Article  PubMed  Google Scholar 

  64. Kojima K, Yokoi K, Matsuguma H et al (2002) Middle mediastinal thymoma. J Thorac Cardiovasc Surg 124:639–640

    Article  PubMed  Google Scholar 

  65. Holt BA, Varadarajulu S, Krall K, et al. Unusual case of a stage I thymoma of the posterior mediastinum: endoscopic ultrasound-guided fine needle aspiration alone clinches the diagnosis. Endoscopy. 2014;46 Suppl 1 UCTN:E577-578.

  66. Sakurai H, Kaji M, Suemasu K (2009) Thymoma of the middle mediastinum: 11C-acetate positron emission tomography imaging. Ann Thorac Surg 87:1271–1274

    Article  PubMed  Google Scholar 

  67. Huang TW, Cheng YL, Tzao C et al (2007) Middle mediastinal thymoma. Respirology 12:934–936

    Article  PubMed  Google Scholar 

  68. Akiba T, Nakada T, Inagaki T (2015) A three-dimensional mediastinal model created with rapid prototyping in a patient with ectopic thymoma. Ann Thorac Cardiovasc Surg 21:87–89

    Article  PubMed  Google Scholar 

  69. Kuzdzal J, Zielinski M, Papla B et al (2006) Middle mediastinal thymoma of unusual pathologic type. Ann Thorac Cardiovasc Surg 12:200–202

    PubMed  Google Scholar 

  70. Nakamura H, Adachi Y, Fujioka S et al (2007) Thoracoscopic resection of middle mediastinal noninvasive thymoma: report of a case. Surg Today 37:787–789

    Article  PubMed  Google Scholar 

  71. Chung SR, Kim IS, Kim J (2012) Thymoma of the middle mediastinum. Korean J Thorac Cardiovasc Surg 45:267–268

    Article  PubMed  PubMed Central  Google Scholar 

  72. Orki A, Patlakoglu MS, Tahaoglu C et al (2009) Malignant invasive thymoma in the posterior mediastinum. Ann Thorac Surg 87:1274–1275

    Article  PubMed  Google Scholar 

  73. Von Wadon A (1934) Thymoma intracheale. Zentralbl Allg Pathol 60:308

    Google Scholar 

  74. Jansen JD, Johnson FE (1990) Fatal ectopic thymoma. Ann Thorac Surg 50:469–470

    Article  CAS  PubMed  Google Scholar 

  75. MacLean G, Guberman A, Giulivi A (1990) Late pseudo-exacerbation of myasthenia gravis due to ectopic thymoma invading lower cranial nerves. Can J Neurol Sci 17:46–48

    Article  CAS  PubMed  Google Scholar 

  76. Ko SF, Tsai YH, Huang HY et al (2005) Retrotracheal thymoma masquerading as esophageal submucosal tumor. World J Gastroenterol 11:3165–3166

    Article  PubMed  PubMed Central  Google Scholar 

  77. Miller DV, Tazelaar HD, Handy JR et al (2005) Thymoma arising within cardiac myxoma. Am J Surg Pathol 29:1208–1213

    Article  PubMed  Google Scholar 

  78. Marandino F, Zoccali C, Salducca N et al (2010) Ectopic primary type A thymoma located in two thoracic vertebras: a case report. BMC Cancer 10:322

    Article  PubMed  PubMed Central  Google Scholar 

  79. Suster S, Moran CA (1999) Thymoma, atypical thymoma, and thymic carcinoma. A novel conceptual approach to the classification of thymic epithelial neoplasms. Am J Clin Pathol 111:826–833

    Article  CAS  PubMed  Google Scholar 

  80. Ring NP, Addis BJ (1986) Thymoma: an integrated clinicopathological and immunohistochemical study. J Pathol 149:327–337

    Article  CAS  PubMed  Google Scholar 

  81. Pan CC, Chen PC, Chou TY et al (2003) Expression of calretinin and other mesothelioma-related markers in thymic carcinoma and thymoma. Hum Pathol 34:1155–1162

    Article  CAS  PubMed  Google Scholar 

  82. Kodama T, Watanabe S, Sato Y et al (1986) An immunohistochemical study of thymic epithelial tumors. I. Epithelial component. Am J Surg Pathol 10:26–33

    Article  CAS  PubMed  Google Scholar 

  83. Wick MR (1990) Assessing the prognosis of thymomas. Ann Thorac Surg 50:521–522

    Article  CAS  PubMed  Google Scholar 

  84. Wick MR, Rosai J. Epithelial tumors. Surgery of the Thymus: Springer; 1990:79–107.

  85. Battifora H, Sun TT, Bahu RM et al (1980) The use of antikeratin antiserum as a diagnostic tool: thymoma versus lymphoma. Hum Pathol 11:635–641

    Article  CAS  PubMed  Google Scholar 

  86. Dotto J, Pelosi G, Rosai J (2007) Expression of p63 in thymomas and normal thymus. Am J Clin Pathol 127:415–420

    Article  PubMed  Google Scholar 

  87. Weissferdt A, Moran CA (2011) Pax8 expression in thymic epithelial neoplasms: an immunohistochemical analysis. Am J Surg Pathol 35:1305–1310

    Article  PubMed  Google Scholar 

  88. Wu M, Sun K, Gil J et al (2009) Immunohistochemical detection of p63 and XIAP in thymic hyperplasia and thymomas. Am J Clin Pathol 131:689–693

    Article  PubMed  Google Scholar 

  89. Tacha D, Zhou D, Cheng L (2011) Expression of PAX8 in normal and neoplastic tissues: a comprehensive immunohistochemical study. Appl Immunohistochem Mol Morphol 19:293–299

    Article  CAS  PubMed  Google Scholar 

  90. Ozcan A, Shen SS, Hamilton C et al (2011) PAX 8 expression in non-neoplastic tissues, primary tumors, and metastatic tumors: a comprehensive immunohistochemical study. Mod Pathol 24:751–764

    Article  CAS  PubMed  Google Scholar 

  91. Laury AR, Hornick JL, Perets R et al (2010) PAX8 reliably distinguishes ovarian serous tumors from malignant mesothelioma. Am J Surg Pathol 34:627–635

    PubMed  Google Scholar 

  92. Nonaka D, Tang Y, Chiriboga L et al (2008) Diagnostic utility of thyroid transcription factors Pax8 and TTF-2 (FoxE1) in thyroid epithelial neoplasms. Mod Pathol 21:192–200

    CAS  PubMed  Google Scholar 

  93. Pan CC, Chen WY, Chiang H (2001) Spindle cell and mixed spindle/lymphocytic thymomas: an integrated clinicopathologic and immunohistochemical study of 81 cases. Am J Surg Pathol 25:111–120

    Article  CAS  PubMed  Google Scholar 

  94. Suster S, Rosai J (1990) Histology of the normal thymus. Am J Surg Pathol 14:284–303

    Article  CAS  PubMed  Google Scholar 

  95. Chan JK, Tsang WY, Seneviratne S et al (1995) The MIC2 antibody 013. Practical application for the study of thymic epithelial tumors. Am J Surg Pathol 19:1115–1123

    Article  CAS  PubMed  Google Scholar 

  96. Casey EM, Kiel PJ, Loehrer PJ Sr (2008) Clinical management of thymoma patients. Hematol Oncol Clin North Am 22:457–473

    Article  PubMed  Google Scholar 

  97. Kim ES, Putnam JB, Komaki R et al (2004) Phase II study of a multidisciplinary approach with induction chemotherapy, followed by surgical resection, radiation therapy, and consolidation chemotherapy for unresectable malignant thymomas: final report. Lung Cancer 44:369–379

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Annikka Weissferdt.

Ethics declarations

Funding

The authors have no funding to disclose.

Conflict of interest

The authors have no conflict of interest to disclose.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Weissferdt, A., Moran, C.A. The spectrum of ectopic thymomas. Virchows Arch 469, 245–254 (2016). https://doi.org/10.1007/s00428-016-1967-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00428-016-1967-0

Keywords

Navigation