Abstract/Scope of Chapter
This chapter provides an overview of lymphomas and related tumors presenting at extranodal locations. Commonalities and differences in the approach to diagnosis, staging, and their pathogenesis are summarized. Intrinsic tumors of the thymus are also discussed.
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References
Burkhardt B, Zimmermann M, Oschlies I, et al. The impact of age and gender on biology, clinical features and treatment outcome of non-Hodgkin lymphoma in childhood and adolescence. Br J Haematol 2005;131:39-49.
Jaffe ES, Harris NL, Stein H, Isaacson PG. Classification of lymphoid neoplasms: the microscope as a tool for disease discovery. Blood 2008;112:4384-99.
Sokolowska-Wojdylo M, Wenzel J, Gaffal E, et al. Circulating clonal CLA(+) and CD4(+) T cells in Sezary syndrome express the skin-homing chemokine receptors CCR4 and CCR10 as well as the lymph node-homing chemokine receptor CCR7. Br J Dermatol 2005;152:258-64.
Magro CM, Dyrsen ME. Cutaneous lymphocyte antigen expression in benign and neoplastic cutaneous B- and T-cell lymphoid infiltrates. J Cutan Pathol 2008;35:1040-9.
Dogan A, Du M, Koulis A, Briskin MJ, Isaacson PG. Expression of lymphocyte homing receptors and vascular addressins in low-grade gastric B-cell lymphomas of mucosa-associated lymphoid tissue. Am J Pathol 1997;151:1361-9.
Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.
Bogle MA, Riddle CC, Triana EM, Jones D, Duvic M. Primary cutaneous B-cell lymphoma. J Am Acad Dermatol 2005;53:479-84.
Dijkman R, Tensen CP, Jordanova ES, et al. Array-based comparative genomic hybridization analysis reveals recurrent chromosomal alterations and prognostic parameters in primary cutaneous large B-cell lymphoma. J Clin Oncol 2006;24:296-305.
Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L. Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients. Blood 2005;106:2491-7.
Senff NJ, Hoefnagel JJ, Jansen PM, et al. Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. J Clin Oncol 2007;25:1581-7.
Jahnke K, Korfel A, O’Neill BP, et al. International study on low-grade primary central nervous system lymphoma. Ann Neurol 2006;59:755-62.
Shenkier TN, Blay JY, O’Neill BP, et al. Primary CNS lymphoma of T-cell origin: a descriptive analysis from the international primary CNS lymphoma collaborative group. J Clin Oncol 2005;23:2233-9.
Ferreri AJ, Blay JY, Reni M, et al. Prognostic scoring system for primary CNS lymphomas: the International Extranodal Lymphoma Study Group experience. J Clin Oncol 2003;21:266-72.
Salzburg J, Burkhardt B, Zimmermann M, et al. Prevalence, clinical pattern, and outcome of CNS involvement in childhood and adolescent non-Hodgkin’s lymphoma differ by non-Hodgkin’s lymphoma subtype: a Berlin-Frankfurt-Munster Group Report. J Clin Oncol 2007;25:3915-22.
Ambrosetti A, Zanotti R, Pattaro C, et al. Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjoegren syndrome or hepatitis C virus infection. Br J Haematol 2004;126:43-9.
Essadi I, Ismaili N, Tazi E, et al. Primary lymphoma of the head and neck: two case reports and review of the literature. Cases J 2008;1:426.
Epstein JB, Epstein JD, Le ND, Gorsky M. Characteristics of oral and paraoral malignant lymphoma: a population-based review of 361 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92:519-25.
Derringer GA, Thompson LD, Frommelt RA, Bijwaard KE, Heffess CS, Abbondanzo SL. Malignant lymphoma of the thyroid gland: a clinicopathologic study of 108 cases. Am J Surg Pathol 2000;24:623-39.
Koss MN. Pulmonary lymphoid disorders. Semin Diagn Pathol 1995;12:158-71.
Solomonov A, Zuckerman T, Goralnik L, Ben-Arieh Y, Rowe JM, Yigla M. Non-Hodgkin’s lymphoma presenting as an endobronchial tumor: report of eight cases and literature review. Am J Hematol 2008;83:416-9.
Kurtin PJ, Myers JL, Adlakha H, et al. Pathologic and clinical features of primary pulmonary extranodal marginal zone B-cell lymphoma of MALT type. Am J Surg Pathol 2001;25:997-1008.
Chng WJ, Remstein ED, Fonseca R, et al. Gene expression profiling of pulmonary mucosa-associated lymphoid tissue lymphoma identifies new biologic insights with potential diagnostic and therapeutic applications. Blood 2009;113:635-45.
Zinzani PL, Poletti V, Zompatori M, et al. Bronchus-associated lymphoid tissue lymphomas: an update of a rare extranodal maltoma. Clin Lymphoma Myeloma 2007;7:566-72.
Satani T, Yokose T, Kaburagi T, Asato Y, Itabashi M, Amemiya R. Amyloid deposition in primary pulmonary marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Pathol Int 2007;57:746-50.
Rawal A, Finn WG, Schnitzer B, Valdez R. Site-specific morphologic differences in extranodal marginal zone B-cell lymphomas. Arch Pathol Lab Med 2007;131:1673-8.
Jones D, Renshaw AA. Recurrent crystal-storing histiocytosis of the lung in a patient without a clonal lymphoproliferative disorder. Arch Pathol Lab Med 1996;120:978-80.
Kojima M, Nakamura N, Otuski Y, et al. Pulmonary lesion of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia appears to be a cause of lymphoplasmacytic proliferation of the lung: a report of five cases. Pathol Res Pract 2008;204:185-90.
Mollejo M, Rodriguez-Pinilla MS, Montes-Moreno S, et al. Splenic Follicular Lymphoma: Clinicopathologic Characteristics of a Series of 32 Cases. Am J Surg Pathol 2009;33:730-8.
Zhu Q, Xu B, Xu K, Li J, Jin XL. Primary non-Hodgkin’s lymphoma in the esophagus. J Dig Dis 2008;9:241-4.
Krugmann J, Dirnhofer S, Gschwendtner A, et al. Primary gastrointestinal B-cell lymphoma. A clincopathological and immunohistochemical study of 61 cases with an evaluation of prognostic parameters. Pathol Res Pract 2001;197:385-93.
Bende RJ, Smit LA, Bossenbroek JG, et al. Primary follicular lymphoma of the small intestine: alpha4beta7 expression and immunoglobulin configuration suggest an origin from local antigen-experienced B cells. Am J Pathol 2003;162:105-13.
Afolayan EA, Anjorin AS. Incidence of primary extranodal lymphoma involving gastrointestinal tract by histological type at Ilorin. Niger J Med 2001;10:135-8.
Schmitt-Graff A, Hummel M, Zemlin M, et al. Intestinal T-cell lymphoma: a reassessment of cytomorphological and phenotypic features in relation to patterns of small bowel remodelling. Virchows Arch 1996;429:27-36.
Katoh A, Ohshima K, Kanda M, et al. Gastrointestinal T cell lymphoma: predominant cytotoxic phenotypes, including alpha/beta, gamma/delta T cell and natural killer cells. Leuk Lymphoma 2000;39:97-111.
Zettl A, Ott G, Makulik A, et al. Chromosomal gains at 9q characterize enteropathy-type T-cell lymphoma. Am J Pathol 2002;161:1635-45.
Salmon JS, Thompson MA, Arildsen RC, Greer JP. Non-Hodgkin’s lymphoma involving the liver: clinical and therapeutic considerations. Clin Lymphoma Myeloma 2006;6:273-80.
Ostrowski ML, Inwards CY, Strickler JG, Witzig TE, Wenger DE, Unni KK. Osseous Hodgkin disease. Cancer 1999;85:1166-78.
Ostrowski ML, Unni KK, Banks PM, et al. Malignant lymphoma of bone. Cancer 1986;58:2646-55.
Jones D, Kraus MD, Dorfman DM. Lymphoma presenting as a solitary bone lesion. Am J Clin Pathol 1999;111:171-8.
Jones D. Large Cell lymphoma presenting in the bone marrow. Case Rev Pathol 2000;5:281-6.
Kajiura D, Yamashita Y, Mori N. Diffuse large B-cell lymphoma initially manifesting in the bone marrow. Am J Clin Pathol 2007;127:762-9.
Talwalkar SS, Miranda RN, Valbuena JR, Routbort MJ, Martin AW, Medeiros LJ. Lymphomas involving the breast: a study of 106 cases comparing localized and disseminated neoplasms. Am J Surg Pathol 2008;32:1299-309.
Validire P, Capovilla M, Asselain B, et al. Primary breast non-Hodgkin’s lymphoma: A large single center study of initial characteristics, natural history, and prognostic factors. Am J Hematol 2009;84:133-9.
Ottaviani G, Matturri L, Rossi L, Jones D. Sudden death due to lymphomatous infiltration of the cardiac conduction system. Cardiovasc Pathol 2003;12:77-81.
Ban-Hoefen M, Zeglin MA, Bisognano JD. Diffuse large B cell lymphoma presenting as a cardiac mass and odynophagia. Cardiol J 2008;15:471-4.
Stakos DA, Xatseras DI, Boudoulas H. Cardiac lymphoma. Eur Heart J 2006;27:1538.
Fujisaki J, Tanaka T, Kato J, et al. Primary cardiac lymphoma presenting clinically as restrictive cardiomyopathy. Circ J 2005;69:249-52.
Schubert S, Abdul-Khaliq H, Lehmkuhl HB, et al. Diagnosis and treatment of post-transplantation lymphoproliferative disorder in pediatric heart transplant patients. Pediatr Transplant 2009;13:54-62.
Aversa SM, Stragliotto S, Marino D, et al. Post-transplant lymphoproliferative disorders after heart or kidney transplantation at a single centre: presentation and response to treatment. Acta Haematol 2008;120:36-46.
Singh D, Kumar L, Sharma A, Vijayaraghavan M, Thulkar S, Tandon N. Adrenal involvement in non-Hodgkin’s lymphoma: four cases and review of literature. Leuk Lymphoma 2004;45:789-94.
Kraus MD, Jones D, Bartlett NL. Intravascular lymphoma associated with endocrine dysfunction: a report of four cases and a review of the literature. Am J Med 1999;107:169-76.
Elharroudi T, Ismaili N, Errihani H, Jalil A. Primary lymphoma of the ovary. J Cancer Res Ther 2008;4:195-6.
Vang R, Medeiros LJ, Ha CS, Deavers M. Non-Hodgkin’s lymphomas involving the uterus: a clinicopathologic analysis of 26 cases. Mod Pathol 2000;13:19-28.
Garcia MG, Deavers MT, Knoblock RJ, et al. Myeloid sarcoma involving the gynecologic tract: a report of 11 cases and review of the literature. Am J Clin Pathol 2006;125:783-90.
Visco C, Medeiros LJ, Mesina OM, et al. Non-Hodgkin’s lymphoma affecting the testis: is it curable with doxorubicin-based therapy? Clin Lymphoma 2001;2:40-6.
Zouhair A, Weber D, Belkacemi Y, et al. Outcome and patterns of failure in testicular lymphoma: a multicenter Rare Cancer Network study. Int J Radiat Oncol Biol Phys 2002;52:652-6.
Gomez Garcia I, Rodriguez Patron R, Sanz Mayayo E. et al [Primary testicular lymphoma. Report of a new case and review of the literature]. Actas Urol Esp 2004;28:141-6.
Mandal M, Crusio KM, Meng F, et al. Regulation of lymphocyte progenitor survival by the proapoptotic activities of Bim and Bid. Proc Natl Acad Sci U S A 2008;105:20840-5.
Meager A, Peterson P, Willcox N. Hypothetical review: thymic aberrations and type-I interferons; attempts to deduce autoimmunizing mechanisms from unexpected clues in monogenic and paraneoplastic syndromes. Clin Exp Immunol 2008;154:141-51.
Leite MI, Jones M, Strobel P, et al. Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status. Am J Pathol 2007;171:893-905.
Roxanis I, Micklem K, McConville J, Newsom-Davis J, Willcox N. Thymic myoid cells and germinal center formation in myasthenia gravis; possible roles in pathogenesis. J Neuroimmunol 2002;125:185-97.
Verstandig AG, Epstein DM, Miller WT Jr, Aronchik JA, Gefter WB, Miller WT. Thymoma-report of 71 cases and a review. Crit Rev Diagn Imaging 1992;33:201-30.
Sperling B, Marschall J, Kennedy R, Pahwa P, Chibbar R. Thymoma: a review of the clinical and pathological findings in 65 cases. Can J Surg 2003;46:37-42.
Flores KG, Li J, Hale LP. B cells in epithelial and perivascular compartments of human adult thymus. Hum Pathol 2001;32:926-34.
Spencer J, Choy M, Hussell T, Papadaki L, Kington JP, Isaacson PG. Properties of human thymic B cells. Immunology 1992;75:596-600.
Punnonen J, de Vries JE. Characterization of a novel CD2+ human thymic B cell subset. J Immunol 1993;151:100-10.
Copie-Bergman C, Plonquet A, Alonso MA, 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;15:1172-80.
Meraouna A, Cizeron-Clairac G, Panse RL, et al. The chemokine CXCL13 is a key molecule in autoimmune myasthenia gravis. Blood 2006;108:432-40.
Thangarajh M, Masterman T, Helgeland L, et al. The thymus is a source of B-cell-survival factors-APRIL and BAFF-in myasthenia gravis. J Neuroimmunol 2006;178:161-6.
Le Panse R, Cizeron-Clairac G, Bismuth J, Berrih-Aknin S. Microarrays reveal distinct gene signatures in the thymus of seropositive and seronegative myasthenia gravis patients and the role of CC chemokine ligand 21 in thymic hyperplasia. J Immunol 2006;177:7868-79.
Sugihara A, Inaba M, Mori SI, et al. Differentiation from thymic B cell progenitors to mature B cells in vitro. Immunobiology 2000;201:515-26.
Berzi A, Ayata CK, Cavalcante P, et al. BDNF and its receptors in human myasthenic thymus: implications for cell fate in thymic pathology. J Neuroimmunol 2008;197:128-39.
Annunziato F, Romagnani P, Cosmi L, et al. Macrophage-derived chemokine and EBI1-ligand chemokine attract human thymocytes in different stage of development and are produced by distinct subsets of medullary epithelial cells: possible implications for negative selection. J Immunol 2000;165:238-46.
Hoffacker V, Schultz A, Tiesinga JJ, et al. Thymomas alter the T-cell subset composition in the blood: a potential mechanism for thymoma-associated autoimmune disease. Blood 2000;96:3872-9.
Masaoka A, Monden Y, Nakahara K, Tanioka T. Follow-up study of thymomas with special reference to their clinical stages. Cancer 1981;48:2485-92.
Engel P, Marx A, Muller-Hermelink HK. Thymic tumours in Denmark. A retrospective study of 213 cases from 1970-1993. Pathol Res Pract 1999;195:565-70.
Zettl A, Strobel P, Wagner K, et al. Recurrent genetic aberrations in thymoma and thymic carcinoma. Am J Pathol 2000;157:257-66.
Lequaglie C, Giudice G, Brega Massone PP, Conti B, Cataldo I. Clinical and pathologic predictors of survival in patients with thymic tumors. J Cardiovasc Surg (Torino) 2002;43:269-74.
Thomas DA, O’Brien S, Cortes J, et al. Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma. Blood 2004;104:1624-30.
Hunault M, Truchan-Graczyk M, Caillot D, et al. Outcome of adult T-lymphoblastic lymphoma after acute lymphoblastic leukemia-type treatment: a GOELAMS trial. Haematologica 2007;92:1623-30.
Onciu M, Lai R, Vega F, Bueso-Ramos C, Medeiros LJ. Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets. Am J Clin Pathol 2002;117:252-8.
Onishi Y, Matsuno Y, Tateishi U, et al. Two entities of precursor T-cell lymphoblastic leukemia/lymphoma based on radiologic and immunophenotypic findings. Int J Hematol 2004;80:43-51.
Ballerini P, Landman-Parker J, Cayuela JM, et al. Impact of genotype on survival of children with T-cell acute lymphoblastic leukemia treated according to the French protocol FRALLE-93: the effect of TLX3/HOX11L2 gene expression on outcome. Haematologica 2008;93:1658-65.
Baleydier F, Decouvelaere AV, Bergeron J, et al. T cell receptor genotyping and HOXA/TLX1 expression define three T lymphoblastic lymphoma subsets which might affect clinical outcome. Clin Cancer Res 2008;14:692-700.
Gorczyca W, Tugulea S, Liu Z, Li X, Wong JY, Weisberger J. Flow cytometry in the diagnosis of mediastinal tumors with emphasis on differentiating thymocytes from precursor T-lymphoblastic lymphoma/leukemia. Leuk Lymphoma 2004;45:529-38.
Sakuraba M, Motoji T, Nitta S, Mizoguchi H, Ohnuki T. Increased thymocyte CD4+CD8+ cells and T-cell receptor beta gene rearrangements in thymoma. Jpn J Thorac Cardiovasc Surg 2003;51:481-7.
Macon WR, Rynalski TH, Swerdlow SH, Cousar JB. T-cell lymphoblastic leukemia/lymphoma presenting in a recurrent thymoma. Mod Pathol 1991;4:524-8.
Johnson PW, Davies AJ. Primary mediastinal B-cell lymphoma. Hematology Am Soc Hematol Educ Program 2008;2008:349-58.
Bishop PC, Wilson WH, Pearson D, Janik J, Jaffe ES, Elwood PC. CNS involvement in primary mediastinal large B-cell lymphoma. J Clin Oncol 1999;17:2479-85.
Iqbal J, Greiner TC, Patel K, et al. Distinctive patterns of BCL6 molecular alterations and their functional consequences in different subgroups of diffuse large B-cell lymphoma. Leukemia 2007;21:2332-43.
Pileri SA, Gaidano G, Zinzani PL, et al. Primary mediastinal B-cell lymphoma: high frequency of BCL-6 mutations and consistent expression of the transcription factors OCT-2, BOB.1, and PU.1 in the absence of immunoglobulins. Am J Pathol 2003;162:243-53.
Meier C, Hoeller S, Bourgau C, et al. Recurrent numerical aberrations of JAK2 and deregulation of the JAK2-STAT cascade in lymphomas. Mod Pathol 2009;22:476-87.
Roberts RA, Wright G, Rosenwald AR, et al. Loss of major histocompatibility class II gene and protein expression in primary mediastinal large B-cell lymphoma is highly coordinated and related to poor patient survival. Blood 2006;108:311-8.
Rosenwald A, Wright G, Leroy K, 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;198:851-62.
Krugmann J, Feichtinger H, Greil R, Fend F. Thymic Hodgkin’s disease - a histological and immunohistochemical study of three cases. Pathol Res Pract 1999;195:681-7.
Magro CM, Crowson AN, Kovatich AJ, Burns F. Drug-induced reversible lymphoid dyscrasia: a clonal lymphomatoid dermatitis of memory and activated T cells. Hum Pathol 2003;34:119-29.
Carlson JA, Mihm MC Jr, LeBoit PE. Cutaneous lymphocytic vasculitis: a definition, a review, and a proposed classification. Semin Diagn Pathol 1996;13:72-90.
Camilleri-Broet S, Martin A, Moreau A, et al. Primary central nervous system lymphomas in 72 immunocompetent patients: pathologic findings and clinical correlations. Groupe Ouest Est d’etude des Leucenies et Autres Maladies du Sang (GOELAMS). Am J Clin Pathol 1998;110:607-12.
Nuckols JD, Liu K, Burchette JL, McLendon RE, Traweek ST. Primary central nervous system lymphomas: a 30-year experience at a single institution. Mod Pathol 1999;12:1167-73.
Iwamoto FM, Abrey LE. Primary dural lymphomas: a review. Neurosurg Focus 2006;21:E5.
Weber AL, Rahemtullah A, Ferry JA. Hodgkin and non-Hodgkin lymphoma of the head and neck: clinical, pathologic, and imaging evaluation. Neuroimaging Clin N Am 2003;13:371-92.
Papouliakos S, Karkos PD, Korres G, Karatzias G, Sastry A, Riga M. Comparison of clinical and histopathological evaluation of tonsils in pediatric and adult patients. Eur Arch Otorhinolaryngol 2008;266:1309-13.
Arber DA, Rappaport H, Weiss LM. Non-Hodgkin’s lymphoproliferative disorders involving the spleen. Mod Pathol 1997;10:18-32.
Stancu M, Jones D, Vega F, Medeiros LJ. Peripheral T-cell lymphoma arising in the liver. Am J Clin Pathol 2002;118:574-81.
Cirillo M, Federico M, Curci G, Tamborrino E, Piccinini L, Silingardi V. Primary gastrointestinal lymphoma: a clinicopathological study of 58 cases. Haematologica 1992;77:156-61.
Banks PM. Gastrointestinal lymphoproliferative disorders. Histopathology 2007;50:42-54.
Bernatz PE, Harrison EG, Clagett OT. Thymoma: a clinicopathologic study. J Thorac Cardiovasc Surg 1961;42:424-44.
Muller-Hermelink HK, Marx A. Thymoma. Curr Opin Oncol 2000;12:426-33.
Suster S, Moran CA. Thymoma classification: current status and future trends. Am J Clin Pathol 2006;125:542-54.
Müller-Hermelink K, Moller P, Engel P, et al. Tumors of the thymus: Introduction. In: Travis WD, Brambilla E, Müller-Hermelink HK, Harris CC, eds. Pathology and genetics of tumours of the lung, pleura, thymus and heart (WHO classification of tumours series). Lyon, France: IARC Press, 2004:148-51.
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Stewart, B.D., Manning, J.T., Jones, D. (2010). Extranodal Lymphomas and Tumors of the Thymus. In: Jones, D. (eds) Neoplastic Hematopathology. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-384-8_27
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