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Lymphomas and Other Rare Tumors of the Thymus

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Atlas of Thymic Pathology

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Abstract

Among mediastinal masses, about half are primary tumors in the anterior mediastinum, both benign and malignant. A variety of nonneoplastic lesions (developmental, inflammatory, reactive) can present as a localized mass in this compartment involving the thymus. Ectopic/embryonic remnants also occur in the prevascular compartment of the mediastinum. Rare tumors may develop from each of the multiple tissue types located in this vital area. The purpose of this chapter is to provide a short morphologic/photographic overview of lymphomas and of other rare tumors of mesenchymal type and of germ cell tumors occurring in the thymus or in the anterior mediastinum area. Selected examples of pseudotumors will also be shown. Specific differential diagnostic points are provided, both for “solid” tumors and for hematological malignancies. The main immunohistochemical characteristics of neoplastic/nonneoplastic pathology and updated specific references are discussed.

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References

  1. Azarow KS, Pearl RH, Zurcher R, Edwards FH, Cohen AJ. Primary mediastinal masses. A comparison of adult and pediatric populations. J Thorac Cardiovasc Surg. 1993;106(1):67–72.

    Article  CAS  PubMed  Google Scholar 

  2. Liu T, Al-Kzayer LFY, Xie X, Fan H, Sarsam SN, Nakazawa Y, et al. Mediastinal lesions across the age spectrum: a clinicopathological comparison between pediatric and adult patients. Oncotarget. 2017;8(35):59845–53. https://doi.org/10.18632/oncotarget.17201.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Naeem F, Metzger ML, Arnold SR, Adderson EE. Distinguishing benign mediastinal masses from malignancy in a histoplasmosis-endemic region. J Pediatr. 2015;167(2):409–15. https://doi.org/10.1016/j.jpeds.2015.04.066.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Szolkowska M, Szczepulska-Wojcik E, Maksymiuk B, Burakowska B, Winiarski S, Gatarek J, et al. Primary mediastinal neoplasms: a report of 1,005 cases from a single institution. J Thorac Dis. 2019;11(6):2498–511. https://doi.org/10.21037/jtd.2019.05.42.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Takeda S, Miyoshi S, Akashi A, Ohta M, Minami M, Okumura M, et al. Clinical spectrum of primary mediastinal tumors: a comparison of adult and pediatric populations at a single Japanese institution. J Surg Oncol. 2003a;83(1):24–30. https://doi.org/10.1002/jso.10231.

    Article  PubMed  Google Scholar 

  6. Carter BW, Benveniste MF, Marom EM. Diagnostic approach to the anterior/prevascular mediastinum for radiologists. Mediastinum. 2018;3:18. https://doi.org/10.21037/med.2018.12.03.

    Article  Google Scholar 

  7. Carter BW, Marom EM, Detterbeck FC. Approaching the patient with an anterior mediastinal mass: a guide for clinicians. J Thorac Oncol. 2014;9(9 Suppl 2):S102–9. https://doi.org/10.1097/JTO.0000000000000294.

    Article  PubMed  Google Scholar 

  8. Rashidfarokhi M, Gupta J, Leytin A, Epelbaum O. Ectopic anterior mediastinal pathology in the chest: radiologic-pathologic correlation of unexpected encounters with the “Terrible Ts”. J Clin Imaging Sci. 2016;6:49. https://doi.org/10.4103/2156-7514.197025.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Weissferdt A, Moran CA. The spectrum of ectopic thymomas. Virchows Arch. 2016;469(3):245–54. https://doi.org/10.1007/s00428-016-1967-0.

    Article  PubMed  Google Scholar 

  10. Middleton G. Involvement of the thymus by metastic neoplasms. Br J Cancer. 1966;20(1):41–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Suster S, Moran C. Diagnostic pathology: thoracic. Philadelphia, PA: Elsevier; 2012.

    Google Scholar 

  12. Petersdorf SH, Wood DE. Lymphoproliferative disorders presenting as mediastinal neoplasms. Semin Thorac Cardiovasc Surg. 2000;12(4):290–300.

    Article  CAS  PubMed  Google Scholar 

  13. Addis BJ, Isaacson PG. Large cell lymphoma of the mediastinum: a B-cell tumour of probable thymic origin. Histopathology. 1986;10(4):379–90.

    Article  CAS  PubMed  Google Scholar 

  14. Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG. WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: International Agency for Research on Cancer – IARC Press; 2015.

    Google Scholar 

  15. Chen L, Wang M, Fan H, Hu F, Liu T. Comparison of pediatric and adult lymphomas involving the mediastinum characterized by distinctive clinicopathological and radiological features. Sci Rep. 2017;7(1):2577. https://doi.org/10.1038/s41598-017-02720-1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Maeshima AM, Taniguchi H, Suzuki T, Yuda S, Toyoda K, Yamauchi N, et al. Distribution of malignant lymphomas in the anterior mediastinum: a single-institution study of 76 cases in Japan, 1997–2016. Int J Hematol. 2017;106(5):675–80. https://doi.org/10.1007/s12185-017-2331-0.

    Article  PubMed  Google Scholar 

  17. Bassan R, Maino E, Cortelazzo S. Lymphoblastic lymphoma: an updated review on biology, diagnosis, and treatment. Eur J Haematol. 2016;96(5):447–60. https://doi.org/10.1111/ejh.12722.

    Article  PubMed  Google Scholar 

  18. Duwe BV, Sterman DH, Musani AI. Tumors of the mediastinum. Chest. 2005;128(4):2893–909. https://doi.org/10.1378/chest.128.4.2893.

    Article  PubMed  Google Scholar 

  19. Johnson PW. IV. Masses in the mediastinum: primary mediastinal lymphoma and intermediate types. Hematol Oncol. 2015;33(Suppl 1):29–32. https://doi.org/10.1002/hon.2212.

    Article  PubMed  Google Scholar 

  20. Marchevsky A, Marx A, Ströbel P, Suster S, Venuta F, Marino M, et al. Policies and reporting guidelines for small biopsy specimens of mediastinal masses. J Thorac Oncol. 2011;6(7 Suppl 3):S1724–9. https://doi.org/10.1097/JTO.0b013e31821ea57c.

    Article  PubMed  Google Scholar 

  21. Orazi A, Foucar K, Knowles DM, Weiss LE. Knowles neoplastic hematopathology. Philadelphia, PA: Wolters Kluwer Health; 2014.

    Google Scholar 

  22. Pan Y, Li GD, Liu WP, Zhang WY, Tang Y, Li FY. Lymphoblastic lymphoma and acute lymphoblastic leukemia: a clinicopathologic, immunophenotypic and prognostic study in 153 Chinese patients. Zhonghua Bing Li Xue Za Zhi. 2009;38(12):810–5.

    PubMed  Google Scholar 

  23. Swerdlow S, Campo E, Harris N, Jaffe E, Pileri S, Stein H, et al. World Health Organization classification of tumours of haematopoietic and lymphoid tissues. Lyon: International Agency for Research on Cancer – IARC Press; 2017.

    Google Scholar 

  24. Castleman B. Tumors of the thymus gland. Washington, DC: Armed Forces Institute of Pathology; 1955.

    Google Scholar 

  25. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956;9(4):822–30.

    Article  CAS  PubMed  Google Scholar 

  26. Desai SB, Pradhan SA, Chinoy RF. Mediastinal Castleman's disease complicated by follicular dendritic cell tumour. Indian J Cancer. 2000;37(4):129–32.

    CAS  PubMed  Google Scholar 

  27. Kligerman SJ, Auerbach A, Franks TJ, Galvin JR. Castleman disease of the thorax: clinical, radiologic, and pathologic correlation: from the radiologic pathology archives. Radiographics. 2016;36(5):1309–32. https://doi.org/10.1148/rg.2016160076.

    Article  PubMed  Google Scholar 

  28. O’Reilly PE, Joshi VV, Holbrook CT, Weisenburger DD. Multicentric Castleman’s disease in a child with prominent thymic involvement: a case report and brief review of the literature. Mod Pathol. 1993;6(6):776–80.

    PubMed  Google Scholar 

  29. Detlefsen S. IgG4-related disease: a systemic condition with characteristic microscopic features. Histol Histopathol. 2013;28(5):565–84. https://doi.org/10.14670/hh-28.565.

    Article  PubMed  Google Scholar 

  30. Simonetti S, Perez Munoz N, Lopez Vivancos J, Sanchez Sitjes L, Herranz Perez JC, Leal Bohorquez N, et al. IgG4-related disease in thymus. A very rare case of chronic fibrosis mimicking sarcoidosis. Tumori. 2017;103(Suppl. 1):e19–24. https://doi.org/10.5301/tj.5000687.

    Article  PubMed  Google Scholar 

  31. Laurent C, Do C, Gourraud PA, de Paiva GR, Valmary S, Brousset P. Prevalence of common non-hodgkin lymphomas and subtypes of hodgkin lymphoma by nodal site of involvement: a systematic retrospective review of 938 cases. Medicine. 2015;94(25):e987. https://doi.org/10.1097/MD.0000000000000987.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Marino M, Ascani S. An overview on the differential diagnostics of tumors of the anterior-superior mediastinum: the pathologist’s perspective. Mediastinum. 2019;3(6) https://doi.org/10.21037/med.2018.12.01.

  33. Hale LP. Histologic and molecular assessment of human thymus. Ann Diagn Pathol. 2004;8(1):50–60.

    Article  PubMed  Google Scholar 

  34. Jevremovic D, Roden AC, Ketterling RP, Kurtin PJ, McPhail ED. LMO2 is a specific marker of T-lymphoblastic leukemia/lymphoma. Am J Clin Pathol. 2016;145(2):180–90. https://doi.org/10.1093/ajcp/aqv024.

    Article  CAS  PubMed  Google Scholar 

  35. Menter T, Gasser A, Juskevicius D, Dirnhofer S, Tzankov A. Diagnostic Utility of the Germinal Center-associated Markers GCET1, HGAL, and LMO2 in Hematolymphoid Neoplasms. Appl Immunohistochem Mol Morphol. 2015;23(7):491–8. https://doi.org/10.1097/PAI.0000000000000107.

    Article  CAS  PubMed  Google Scholar 

  36. Chilosi M, Doglioni C, Yan Z, Lestani M, Menestrina F, Sorio C, et al. Differential expression of cyclin-dependent kinase 6 in cortical thymocytes and T-cell lymphoblastic lymphoma/leukemia. Am J Pathol. 1998;152(1):209–17.

    CAS  PubMed  PubMed Central  Google Scholar 

  37. Kakuta N, Sumitani M, Sugitani A, Nakahama K, Miki Y, Syoji S. Mediastinal peripheral T-cell lymphoma diagnosed by repeated biopsies after an initial diagnosis of fibrosing mediastinitis. Respirol Case Rep. 2017;5(6):e00272. https://doi.org/10.1002/rcr2.272.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Fend F, Nachbaur D, Oberwasserlechner F, Kreczy A, Huber H, Müller-Hermelink HK. Phenotype and topography of human thymic B cells. An immunohistologic study. Virchows Arch B Cell Pathol Incl Mol Pathol. 1991;60(6):381–8.

    Article  CAS  PubMed  Google Scholar 

  39. Isaacson PG, Norton AJ, Addis BJ. The human thymus contains a novel population of B lymphocytes. Lancet. 1987;2(8574):1488–91.

    Article  CAS  PubMed  Google Scholar 

  40. Perera J, Huang H. The development and function of thymic B cells. Cell Mol Life Sci. 2015;72(14):2657–63. https://doi.org/10.1007/s00018-015-1895-1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Yamano T, Nedjic J, Hinterberger M, Steinert M, Koser S, Pinto S, et al. Thymic B cells are licensed to present self antigens for central T Cell tolerance induction. Immunity. 2015;42(6):1048–61. https://doi.org/10.1016/j.immuni.2015.05.013.

    Article  CAS  PubMed  Google Scholar 

  42. Piña-Oviedo S, Moran CA. Primary mediastinal classical hodgkin lymphoma. Adv Anat Pathol. 2016;23(5):285–309. https://doi.org/10.1097/PAP.0000000000000119.

    Article  CAS  PubMed  Google Scholar 

  43. Rauthe S, Rosenwald A. Mediastinal lymphomas. Pathologe. 2016;37(5):457–64. https://doi.org/10.1007/s00292-016-0199-z.

    Article  CAS  PubMed  Google Scholar 

  44. Marino M, Roden A. The evolution of the histopathologic classification of thymic epithelial tumors (Review). Mediastinum. 2018;2(9) https://doi.org/10.21037/med.2018.01.04.

  45. Suster S, Moran CA. Malignant thymic neoplasms that may mimic benign conditions. Semin Diagn Pathol. 1995;12(1):98–104.

    CAS  PubMed  Google Scholar 

  46. den Bakker MA, Oosterhuis JW. Tumours and tumour-like conditions of the thymus other than thymoma; a practical approach. Histopathology. 2009;54(1):69–89. https://doi.org/10.1111/j.1365-2559.2008.03177.x.

    Article  Google Scholar 

  47. Rosenwald A, Wright G, Leroy K, Yu X, Gaulard P, Gascoyne RD, et al. Molecular diagnosis of primary mediastinal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell lymphoma related to Hodgkin lymphoma. J Exp Med. 2003;198(6):851–62. https://doi.org/10.1084/jem.20031074.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Savage KJ, Monti S, Kutok JL, Cattoretti G, Neuberg D, De Leval L, et al. The molecular signature of mediastinal large B-cell lymphoma differs from that of other diffuse large B-cell lymphomas and shares features with classical Hodgkin lymphoma. Blood. 2003;102(12):3871–9. https://doi.org/10.1182/blood-2003-06-1841.

    Article  CAS  PubMed  Google Scholar 

  49. Menestrina F, Chilosi M, Bonetti F, Lestani M, Scarpa A, Novelli P, et al. Mediastinal large-cell lymphoma of B-type, with sclerosis: histopathological and immunohistochemical study of eight cases. Histopathology. 1986;10(6):589–600.

    Article  CAS  PubMed  Google Scholar 

  50. Paulli M, Strater J, Gianelli U, Rousset MT, Gambacorta M, Orlandi E, et al. Mediastinal B-cell lymphoma: a study of its histomorphologic spectrum based on 109 cases. Hum Pathol. 1999;30(2):178–87. https://doi.org/10.1016/s0046-8177(99)90273-3.

    Article  CAS  PubMed  Google Scholar 

  51. Copie-Bergman C, Plonquet A, Alonso MA, Boulland ML, Marquet J, Divine M, et al. MAL expression in lymphoid cells: further evidence for MAL as a distinct molecular marker of primary mediastinal large B-cell lymphomas. Mod Pathol. 2002;15(11):1172–80. https://doi.org/10.1097/01.MP.0000032534.81894.B3.

    Article  PubMed  Google Scholar 

  52. Aladily TN, Mansour A, Alsughayer A, Sughayer M, Medeiros LJ. The utility of CD83, fascin and CD23 in the differential diagnosis of primary mediastinal large B-cell lymphoma versus classic Hodgkin lymphoma. Ann Diagn Pathol. 2019;40:72–6. https://doi.org/10.1016/j.anndiagpath.2019.04.009.

    Article  PubMed  Google Scholar 

  53. Hoeller S, Zihler D, Zlobec I, Obermann EC, Pileri SA, Dirnhofer S, et al. BOB.1, CD79a and cyclin E are the most appropriate markers to discriminate classical Hodgkin's lymphoma from primary mediastinal large B-cell lymphoma. Histopathology. 2010;56(2):217–28. https://doi.org/10.1111/j.1365-2559.2009.03462.x.

    Article  PubMed  Google Scholar 

  54. O’Malley DP, Fedoriw Y, Weiss LM. Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A proposed scoring system. Appl Immunohistochem Mol Morphol. 2016;24(8):535–40. https://doi.org/10.1097/PAI.0000000000000236.

    Article  CAS  PubMed  Google Scholar 

  55. Eberle FC, Rodriguez-Canales J, Wei L, Hanson JC, Killian JK, Sun HW, et al. Methylation profiling of mediastinal gray zone lymphoma reveals a distinctive signature with elements shared by classical Hodgkin’s lymphoma and primary mediastinal large B-cell lymphoma. Haematologica. 2011;96(4):558–66. https://doi.org/10.3324/haematol.2010.033167.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Traverse-Glehen A, Pittaluga S, Gaulard P, Sorbara L, Alonso MA, Raffeld M, et al. Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. Am J Surg Pathol. 2005;29(11):1411–21.

    Article  PubMed  Google Scholar 

  57. Hutchinson CB, Wang E. Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma. Arch Pathol Lab Med. 2011;135(3):394–8. https://doi.org/10.1043/2009-0463-RSR.1.

    Article  PubMed  Google Scholar 

  58. Pilichowska M, Pittaluga S, Ferry JA, Hemminger J, Chang H, Kanakry JA, et al. Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL. Blood Adv. 2017;1(26):2600–9. https://doi.org/10.1182/bloodadvances.2017009472.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Sarkozy C, Copie-Bergman C, Damotte D, Ben-Neriah S, Burroni B, Cornillon J, et al. Gray-zone lymphoma between cHL and large B-cell lymphoma: a histopathologic series from the LYSA. Am J Surg Pathol. 2019;43(3):341–51. https://doi.org/10.1097/pas.0000000000001198.

    Article  PubMed  Google Scholar 

  60. Sarkozy C, Molina T, Ghesquières H, Michallet AS, Dupuis J, Damotte D, et al. Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association. Haematologica. 2017;102(1):150–9. https://doi.org/10.3324/haematol.2016.152256.

    Article  PubMed  PubMed Central  Google Scholar 

  61. McCluggage WG, McManus K, Qureshi R, McAleer S, Wotherspoon AC. Low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) of thymus. Hum Pathol. 2000;31(2):255–9.

    Article  CAS  PubMed  Google Scholar 

  62. Kim JM. Primary extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue-type in the thymus of a patient with Sjögren's syndrome and rheumatoid arthritis. J Korean Med Sci. 2003;18(6):897–900. https://doi.org/10.3346/jkms.2003.18.6.897.

    Article  PubMed  PubMed Central  Google Scholar 

  63. Kang LY, Ho SP, Chou YP. Primary thymic mucosa-associated lymphoid tissue lymphoma with multiple thin walled lung cysts: case report and literature review. Chin J Cancer Res. 2013;25(3):354–7. https://doi.org/10.3978/j.issn.1000-9604.2013.06.07.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Maitra A, McKenna RW, Weinberg AG, Schneider NR, Kroft SH. Precursor B-cell lymphoblastic lymphoma. A study of nine cases lacking blood and bone marrow involvement and review of the literature. Am J Clin Pathol. 2001;115(6):868–75. https://doi.org/10.1309/Q5GV-3K00-WAC6-BBUB.

    Article  CAS  PubMed  Google Scholar 

  65. Sander CA, Jaffe ES, Gebhardt FC, Yano T, Medeiros LJ. Mediastinal lymphoblastic lymphoma with an immature B-cell immunophenotype. Am J Surg Pathol. 1992;16(3):300–5.

    Article  CAS  PubMed  Google Scholar 

  66. Lin O, Frizzera G. Angiomyoid and follicular dendritic cell proliferative lesions in Castleman's disease of hyaline-vascular type: a study of 10 cases. Am J Surg Pathol. 1997;21(11):1295–306.

    Article  CAS  PubMed  Google Scholar 

  67. Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646–57. https://doi.org/10.1182/blood-2016-10-746933.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Soumerai JD, Sohani AR, Abramson JS. Diagnosis and management of Castleman disease. Cancer Control. 2014;21(4):266–78.

    Article  PubMed  Google Scholar 

  69. Wu YL, Wu F, Xu CP, Chen GL, Zhang Y, Chen W, et al. Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease. Diagn Pathol. 2019;14(1):5. https://doi.org/10.1186/s13000-019-0779-3.

    Article  PubMed  PubMed Central  Google Scholar 

  70. Busch J, Seidel C, Zengerling F. Male extragonadal germ cell tumors of the adult. Oncol Res Treat. 2016;39(3):140–4. https://doi.org/10.1159/000444271.

    Article  CAS  PubMed  Google Scholar 

  71. Kalhor N, Moran CA. Primary germ cell tumors of the mediastinum: a review. Mediastinum. 2018;2:4. https://doi.org/10.21037/med.2017.12.01.

    Article  Google Scholar 

  72. Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007;21(2):340–50. https://doi.org/10.1038/sj.leu.2404491.

    Article  CAS  PubMed  Google Scholar 

  73. Wong WS, Loong F, Ooi GC, Tse TC, Chim CS. Primary granulocytic sarcoma of the mediastinum. Leuk Lymphoma. 2004;45(9):1931–3. https://doi.org/10.1080/10428190310001645898.

    Article  CAS  PubMed  Google Scholar 

  74. Bremmer F, Ströbel P. Mediastinal germ cell tumors. Der Pathologe. 2016;37(5):441–8. https://doi.org/10.1007/s00292-016-0196-2.

    Article  CAS  PubMed  Google Scholar 

  75. Ikdahl T, Josefsen D, Jakobsen E, Delabie J, Fossa SD. Concurrent mediastinal germ-cell tumour and haematological malignancy: case report and short review of literature. Acta Oncol. 2008;47(3):466–9. https://doi.org/10.1080/02841860701636272.

    Article  PubMed  Google Scholar 

  76. Zhao GQ, Dowell JE. Hematologic malignancies associated with germ cell tumors. Expert Rev Hematol. 2012;5(4):427–37. https://doi.org/10.1586/ehm.12.24.

    Article  CAS  PubMed  Google Scholar 

  77. Masunaga A, Ishibashi F, Koh E, Oide T, Sekine Y, Hiroshima K. Possible relationship between fibrosis of IgG4-related thymitis and the profibrotic cytokines, transforming growth factor beta 1, interleukin 1 beta and interferon gamma: a case report. Diagn Pathol. 2018;13(1):6. https://doi.org/10.1186/s13000-018-0684-1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  78. Ishimoto H, Yatera K, Shimabukuro I, Matsuki Y, Hanaka T, Oda K, et al. Case of immunoglobulin G4 (IgG4)-related disease diagnosed by transbronchial lung biopsy and endobronchial ultrasound-guided transbronchial needle aspiration. J UOEH. 2014;36(4):237–42.

    Article  PubMed  Google Scholar 

  79. den Bakker MA, Marx A, Mukai K, Strobel P. Mesenchymal tumours of the mediastinum-part I. Virchows Arch. 2015a;467(5):487–500. https://doi.org/10.1007/s00428-015-1830-8.

    Article  CAS  Google Scholar 

  80. den Bakker MA, Marx A, Mukai K, Strobel P. Mesenchymal tumours of the mediastinum--part II. Virchows Arch. 2015b;467(5):501–17. https://doi.org/10.1007/s00428-015-1832-6.

    Article  CAS  Google Scholar 

  81. den Bakker M, Marx A, Ströbel P. The pathology of mesenchymal tumors of the mediastinum. Mediastinum. 2018;2:42. https://doi.org/10.21037/med.2018.05.01.

    Article  Google Scholar 

  82. Makdisi G, Roden AC, Shen KR. Successful resection of giant mediastinal lipofibroadenoma of the thymus by video-assisted thoracoscopic surgery. Ann Thorac Surg. 2015;100(2):698–700.

    Google Scholar 

  83. Hui M, Paul TR, Uppin SG, Jyothi N. Lipofibroadenoma with B1 thymoma: A case report of a rare thymic tumor. Indian J Pathol Microbiol. 2018;61(4):630–32.

    Google Scholar 

  84. Doyle LA, Vivero M, Fletcher CD, Mertens F, Hornick JL. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol. 2014;27(3):390–5. https://doi.org/10.1038/modpathol.2013.164.

    Article  CAS  PubMed  Google Scholar 

  85. Tsubochi H, Endo T, Sogabe M, Endo S, Morinaga S, Dobashi Y. Solitary fibrous tumor of the thymus with variegated epithelial components. Int J Clin Exp Pathol. 2014;7(11):7477–84.

    PubMed  PubMed Central  Google Scholar 

  86. Fletcher C, Bridge J, Hogendoorn P, Mertens F. World Health Organization classification of tumours of soft tissue and bone. Lyon: International Agengy for Research on Cancer – IARC Press; 2013.

    Google Scholar 

  87. Yoshida A, Tsuta K, Ohno M, Yoshida M, Narita Y, Kawai A, et al. STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol. 2014;38(4):552–9. https://doi.org/10.1097/pas.0000000000000137.

    Article  PubMed  Google Scholar 

  88. Lee JH, Jeong JS, Kim SR, Jin GY, Chung MJ, Kuh JH, et al. Mediastinal desmoid tumor with remarkably rapid growth: a case report. Medicine. 2015;94(52):e2370. https://doi.org/10.1097/md.0000000000002370.

    Article  PubMed  PubMed Central  Google Scholar 

  89. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268–74. https://doi.org/10.1097/cco.0000000000000374.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  90. Mullen JT, DeLaney TF, Rosenberg AE, Le L, Iafrate AJ, Kobayashi W, et al. Beta-catenin mutation status and outcomes in sporadic desmoid tumors. Oncologist. 2013;18(9):1043–9. https://doi.org/10.1634/theoncologist.2012-0449.

    Article  PubMed  PubMed Central  Google Scholar 

  91. Moran CA, Suster S. Mediastinal hemangiomas: a study of 18 cases with emphasis on the spectrum of morphological features. Hum Pathol. 1995;26(4):416–21. https://doi.org/10.1016/0046-8177(95)90143-4.

    Article  CAS  PubMed  Google Scholar 

  92. Mansour Z, Neuville A, Massard G. Mediastinal epithelioid haemangioendothelioma: a rare mediastinal tumour. Interact Cardiovasc Thorac Surg. 2010;10(1):122–4. https://doi.org/10.1510/icvts.2009.216978.

    Article  PubMed  Google Scholar 

  93. Suster S, Moran CA, Koss MN. Epithelioid hemangioendothelioma of the anterior mediastinum. Clinicopathologic, immunohistochemical, and ultrastructural analysis of 12 cases. Am J Surg Pathol. 1994;18(9):871–81.

    Article  CAS  PubMed  Google Scholar 

  94. Hart J, Mandavilli S. Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med. 2011;135(2):268–72. https://doi.org/10.1043/1543-2165-135.2.268.

    Article  PubMed  Google Scholar 

  95. Anderson T, Zhang L, Hameed M, Rusch V, Travis WD, Antonescu CR. Thoracic epithelioid malignant vascular tumors: a clinicopathologic study of 52 cases with emphasis on pathologic grading and molecular studies of WWTR1-CAMTA1 fusions. Am J Surg Pathol. 2015;39(1):132–9. https://doi.org/10.1097/PAS.0000000000000346.

    Article  PubMed  PubMed Central  Google Scholar 

  96. Weissferdt A, Kalhor N, Suster S, Moran CA. Primary angiosarcomas of the anterior mediastinum: a clinicopathologic and immunohistochemical study of 9 cases. Hum Pathol. 2010;41(12):1711–7. https://doi.org/10.1016/j.humpath.2010.05.003.

    Article  CAS  PubMed  Google Scholar 

  97. Licci S, Puma F, Sbaraglia M, Ascani S. Primary intrathymic lymphangioma. Am J Clin Pathol. 2014;142(5):683–8. https://doi.org/10.1309/AJCPGDRPIMYSSX7U.

    Article  PubMed  Google Scholar 

  98. Zhou H, Zhong C, Fu Q, Tang S, Luo Q, Yu L, et al. Thoracoscopic resection of a huge mediastinal cystic lymphangioma. J Thorac Dis. 2017;9(10):E887–9. https://doi.org/10.21037/jtd.2017.09.97.

    Article  PubMed  PubMed Central  Google Scholar 

  99. Ji Y, Chen S, Peng S, Xia C, Li L. Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 2019;14(1):165. https://doi.org/10.1186/s13023-019-1147-9.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Ozeki M, Nozawa A, Kawamoto N, Fujino A, Hirakawa S, Fukao T. Potential biomarkers of kaposiform lymphangiomatosis. Pediatr Blood Cancer. 2019;66(9):e27878. https://doi.org/10.1002/pbc.27878.

    Article  CAS  PubMed  Google Scholar 

  101. Bishop JA, Alaggio R, Zhang L, Seethala RR, Antonescu CR. Adamantinoma-like Ewing family tumors of the head and neck: a pitfall in the differential diagnosis of basaloid and myoepithelial carcinomas. Am J Surg Pathol. 2015;39(9):1267–74. https://doi.org/10.1097/pas.0000000000000460.

    Article  PubMed  PubMed Central  Google Scholar 

  102. Reali A, Mortellaro G, Allis S, Trevisiol E, Anglesio SM, Bartoncini S, et al. A case of primary mediastinal Ewing's sarcoma/primitive neuroectodermal tumor presenting with initial compression of superior vena cava. Ann Thorac Med. 2013;8(3):121–3. https://doi.org/10.4103/1817-1737.109834.

    Article  PubMed  PubMed Central  Google Scholar 

  103. Bae SH, Hwang JH, Da Nam B, Kim HJ, Kim KU, Kim DW, et al. Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum: A Case Report and Literature Review. Medicine (Baltimore). 2016;95(7):e2725. https://doi.org/10.1097/md.0000000000002725.

    Article  Google Scholar 

  104. Takeda S, Miyoshi S, Ohta M, Minami M, Masaoka A, Matsuda H. Primary germ cell tumors in the mediastinum: a 50-year experience at a single Japanese institution. Cancer. 2003b;97(2):367–76. https://doi.org/10.1002/cncr.11068.

    Article  PubMed  Google Scholar 

  105. Oosterhuis JW, Stoop H, Honecker F, Looijenga LH. Why human extragonadal germ cell tumours occur in the midline of the body: old concepts, new perspectives. Int J Androl. 2007;30(4):256–63.; discussion 263-4. https://doi.org/10.1111/j.1365-2605.2007.00793.x.

    Article  PubMed  Google Scholar 

  106. Oosterhuis JW, Looijenga LHJ. Human germ cell tumours from a developmental perspective. Nat Rev Cancer. 2019;19:522–37. https://doi.org/10.1038/s41568-019-0178-9.

    Article  CAS  PubMed  Google Scholar 

  107. Malagón HD, Montiel DP. Mediastinal germ cell tumours. Diagn Histopathol. 2010;16(5):228–36. https://doi.org/10.1016/j.mpdhp.2010.03.002.

    Article  Google Scholar 

  108. Moran CA, Suster S. Primary germ cell tumors of the mediastinum: I. Analysis of 322 cases with special emphasis on teratomatous lesions and a proposal for histopathologic classification and clinical staging. Cancer. 1997;80(4):681–90.

    Article  CAS  PubMed  Google Scholar 

  109. Alkan M, Karnak I, Ciftci AO, Tanyel FC. Thymic teratoma or thymic remnant attached to mediastinal teratoma? The cellular origin of mediastinal teratomas revisited. Eur J Pediatr Surg. 2004;14(2):117–9. https://doi.org/10.1055/s-2004-817842.

    Article  CAS  PubMed  Google Scholar 

  110. Paradies G, Zullino F, Orofino A, Leggio S. Mediastinal teratomas in children. Case reports and review of the literature. Ann Ital Chir. 2013;84(4):395–403.

    PubMed  Google Scholar 

  111. Moran CA, Suster S, Przygodzki RM, Koss MN. Primary germ cell tumors of the mediastinum: II. Mediastinal seminomas--a clinicopathologic and immunohistochemical study of 120 cases. Cancer. 1997;80(4):691–8. https://doi.org/10.1002/(sici)1097-0142(19970815)80:4<691::aid-cncr7>3.0.co;2-q.

    Article  CAS  PubMed  Google Scholar 

  112. Sung MT, Maclennan GT, Lopez-Beltran A, Zhang S, Montironi R, Cheng L. Primary mediastinal seminoma: a comprehensive assessment integrated with histology, immunohistochemistry, and fluorescence in situ hybridization for chromosome 12p abnormalities in 23 cases. Am J Surg Pathol. 2008;32(1):146–55. https://doi.org/10.1097/PAS.0b013e3181379edf.

    Article  PubMed  Google Scholar 

  113. Weissferdt A, Rodriguez-Canales J, Liu H, Fujimoto J, Wistuba II, Moran CA. Primary mediastinal seminomas: a comprehensive immunohistochemical study with a focus on novel markers. Hum Pathol. 2015;46(3):376–83. https://doi.org/10.1016/j.humpath.2014.11.009.

    Article  CAS  PubMed  Google Scholar 

  114. Zhang K, Deng H, Cagle PT. Utility of immunohistochemistry in the diagnosis of pleuropulmonary and mediastinal cancers: a review and update. Arch Pathol Lab Med. 2014;138(12):1611–28. https://doi.org/10.5858/arpa.2014-0092-RA.

    Article  PubMed  Google Scholar 

  115. Alli PM, Crain BJ, Heitmiller R, Argani P. Malignant melanoma presenting as an intrathymic tumor: a primary thymic melanoma? Arch Pathol Lab Med. 2000;124(1):130–4. https://doi.org/10.1043/0003-9985(2000)124<0130:mmpaai>2.0.co;2.

    Article  CAS  PubMed  Google Scholar 

  116. Naik D, Jebasingh KF, Cherian AJ, Dasgupta R. Ectopic thymic parathyroid adenoma. BMJ Case Rep. 2014;2014 https://doi.org/10.1136/bcr-2014-206634.

  117. Spinelli C, Liserre J, Pucci V, Massart F, Grosso M, Brandi ML. Primary hyperparathyroidism: fifth parathyroid intrathymic adenoma in a young patient. J Pediatr Endocrinol Metab. 2012;25(7–8):781–4. https://doi.org/10.1515/jpem-2012-0101.

    Article  PubMed  Google Scholar 

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Acknowledgments

The authors thank Dr Enzo Gallo for the photographical assistance and Mrs Arianna Papadantonakis for her technical assistance.

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The authors have no conflicts of interest to declare.

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Funding The authors received no funding for this project.

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Authors and Affiliations

  1. Department of Pathology, IRCCS Regina Elena National Cancer Institute, Rome, Italy

    Mirella Marino

  2. Department of Pathology, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland

    Malgorzata Szolkowska

  3. Pathology Unit, Perugia University, Terni, Italy

    Stefano Ascani

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  1. Mirella Marino
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  2. Malgorzata Szolkowska
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  3. Stefano Ascani
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Correspondence to Mirella Marino .

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Editors and Affiliations

  1. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Deepali Jain

  2. Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, TX, USA

    Justin A. Bishop

  3. University of Virginia Medical Center, Charlottesville, VA, USA

    Mark R. Wick

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Marino, M., Szolkowska, M., Ascani, S. (2020). Lymphomas and Other Rare Tumors of the Thymus. In: Jain, D., Bishop, J.A., Wick, M.R. (eds) Atlas of Thymic Pathology. Springer, Singapore. https://doi.org/10.1007/978-981-15-3164-4_13

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