U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Extranodal Lymphoma

MedGen UID:
1807589
•Concept ID:
C0694571
•
Neoplastic Process

Definition

A non-Hodgkin or Hodgkin lymphoma that arises from an anatomic site other than a lymph node. [from NCI]

Professional guidelines

PubMed

Review of lymphoma in the duodenum: An update of diagnosis and management.
Iwamuro M, Tanaka T, Okada H
World J Gastroenterol 2023 Mar 28;29(12):1852-1862. doi: 10.3748/wjg.v29.i12.1852. PMID: 37032723Free PMC Article
Primary testicular lymphoma.
Cheah CY, Wirth A, Seymour JF
Blood 2014 Jan 23;123(4):486-93. Epub 2013 Nov 26 doi: 10.1182/blood-2013-10-530659. PMID: 24282217
Gela histological scoring system for post-treatment biopsies of patients with gastric MALT lymphoma is feasible and reliable in routine practice.
Copie-Bergman C, Wotherspoon AC, Capella C, Motta T, Pedrinis E, Pileri SA, Bertoni F, Conconi A, Zucca E, Ponzoni M, Ferreri AJ
Br J Haematol 2013 Jan;160(1):47-52. Epub 2012 Oct 9 doi: 10.1111/bjh.12078. PMID: 23043300

Recent clinical studies

Etiology

Marginal-Zone Lymphomas.
Rossi D, Bertoni F, Zucca E
N Engl J Med 2022 Feb 10;386(6):568-581. doi: 10.1056/NEJMra2102568. PMID: 35139275
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free PMC Article
Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial.
Ferreri AJ, Cwynarski K, Pulczynski E, Ponzoni M, Deckert M, Politi LS, Torri V, Fox CP, Rosée PL, Schorb E, Ambrosetti A, Roth A, Hemmaway C, Ferrari A, Linton KM, Rudà R, Binder M, Pukrop T, Balzarotti M, Fabbri A, Johnson P, Gørløv JS, Hess G, Panse J, Pisani F, Tucci A, Stilgenbauer S, Hertenstein B, Keller U, Krause SW, Levis A, Schmoll HJ, Cavalli F, Finke J, Reni M, Zucca E, Illerhaus G; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2016 May;3(5):e217-27. Epub 2016 Apr 6 doi: 10.1016/S2352-3026(16)00036-3. PMID: 27132696
Primary breast lymphoma.
Cheah CY, Campbell BA, Seymour JF
Cancer Treat Rev 2014 Sep;40(8):900-8. Epub 2014 Jun 6 doi: 10.1016/j.ctrv.2014.05.010. PMID: 24953564
First-line treatment for primary testicular diffuse large B-cell lymphoma with rituximab-CHOP, CNS prophylaxis, and contralateral testis irradiation: final results of an international phase II trial.
Vitolo U, Chiappella A, Ferreri AJ, Martelli M, Baldi I, Balzarotti M, Bottelli C, Conconi A, Gomez H, Lopez-Guillermo A, Martinelli G, Merli F, Novero D, Orsucci L, Pavone V, Ricardi U, Storti S, Gospodarowicz MK, Cavalli F, Sarris AH, Zucca E
J Clin Oncol 2011 Jul 10;29(20):2766-72. Epub 2011 Jun 6 doi: 10.1200/JCO.2010.31.4187. PMID: 21646602

Diagnosis

Marginal-Zone Lymphomas.
Rossi D, Bertoni F, Zucca E
N Engl J Med 2022 Feb 10;386(6):568-581. doi: 10.1056/NEJMra2102568. PMID: 35139275
Genetic and phenotypic attributes of splenic marginal zone lymphoma.
Bonfiglio F, Bruscaggin A, Guidetti F, Terzi di Bergamo L, Faderl M, Spina V, Condoluci A, Bonomini L, Forestieri G, Koch R, Piffaretti D, Pini K, Pirosa MC, Cittone MG, Arribas A, Lucioni M, Ghilardi G, Wu W, Arcaini L, Baptista MJ, Bastidas G, Bea S, Boldorini R, Broccoli A, Buehler MM, Canzonieri V, Cascione L, Ceriani L, Cogliatti S, Corradini P, Derenzini E, Devizzi L, Dietrich S, Elia AR, Facchetti F, Gaidano G, Garcia JF, Gerber B, Ghia P, Gomes da Silva M, Gritti G, Guidetti A, Hitz F, Inghirami G, Ladetto M, Lopez-Guillermo A, Lucchini E, Maiorana A, Marasca R, Matutes E, Meignin V, Merli M, Moccia A, Mollejo M, Montalban C, Novak U, Oscier DG, Passamonti F, Piazza F, Pizzolitto S, Rambaldi A, Sabattini E, Salles G, Santambrogio E, Scarfò L, Stathis A, Stüssi G, Geyer JT, Tapia G, Tarella C, Thieblemont C, Tousseyn T, Tucci A, Vanini G, Visco C, Vitolo U, Walewska R, Zaja F, Zenz T, Zinzani PL, Khiabanian H, Calcinotto A, Bertoni F, Bhagat G, Campo E, De Leval L, Dirnhofer S, Pileri SA, Piris MA, Traverse-Glehen A, Tzankov A, Paulli M, Ponzoni M, Mazzucchelli L, Cavalli F, Zucca E, Rossi D
Blood 2022 Feb 3;139(5):732-747. doi: 10.1182/blood.2021012386. PMID: 34653238
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free PMC Article
Pediatric Extranodal Lymphoma.
Chung EM, Pavio M
Radiol Clin North Am 2016 Jul;54(4):727-46. doi: 10.1016/j.rcl.2016.03.004. PMID: 27265605
Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial.
Ferreri AJ, Cwynarski K, Pulczynski E, Ponzoni M, Deckert M, Politi LS, Torri V, Fox CP, Rosée PL, Schorb E, Ambrosetti A, Roth A, Hemmaway C, Ferrari A, Linton KM, Rudà R, Binder M, Pukrop T, Balzarotti M, Fabbri A, Johnson P, Gørløv JS, Hess G, Panse J, Pisani F, Tucci A, Stilgenbauer S, Hertenstein B, Keller U, Krause SW, Levis A, Schmoll HJ, Cavalli F, Finke J, Reni M, Zucca E, Illerhaus G; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2016 May;3(5):e217-27. Epub 2016 Apr 6 doi: 10.1016/S2352-3026(16)00036-3. PMID: 27132696

Therapy

Long-term efficacy, safety and neurotolerability of MATRix regimen followed by autologous transplant in primary CNS lymphoma: 7-year results of the IELSG32 randomized trial.
Ferreri AJM, Cwynarski K, Pulczynski E, Fox CP, Schorb E, Celico C, Falautano M, Nonis A, La Rosée P, Binder M, Fabbri A, Ilariucci F, Krampera M, Roth A, Hemmaway C, Johnson PW, Linton KM, Pukrop T, Gørløv JS, Balzarotti M, Hess G, Keller U, Stilgenbauer S, Panse J, Tucci A, Orsucci L, Pisani F, Zanni M, Krause SW, Schmoll HJ, Hertenstein B, Rummel M, Smith J, Thurner L, Cabras G, Pennese E, Ponzoni M, Deckert M, Politi LS, Finke J, Ferranti A, Cozens K, Burger E, Ielmini N, Cavalli F, Zucca E, Illerhaus G; IELSG32 study investigators
Leukemia 2022 Jul;36(7):1870-1878. Epub 2022 May 13 doi: 10.1038/s41375-022-01582-5. PMID: 35562406
MATRix-RICE therapy and autologous haematopoietic stem-cell transplantation in diffuse large B-cell lymphoma with secondary CNS involvement (MARIETTA): an international, single-arm, phase 2 trial.
Ferreri AJM, Doorduijn JK, Re A, Cabras MG, Smith J, Ilariucci F, Luppi M, Calimeri T, Cattaneo C, Khwaja J, Botto B, Cellini C, Nassi L, Linton K, McKay P, Olivieri J, Patti C, Re F, Fanni A, Singh V, Bromberg JEC, Cozens K, Gastaldi E, Bernardi M, Cascavilla N, Davies A, Fox CP, Frezzato M, Osborne W, Liberati AM, Novak U, Zambello R, Zucca E, Cwynarski K; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2021 Feb;8(2):e110-e121. doi: 10.1016/S2352-3026(20)30366-5. PMID: 33513372Free PMC Article
Whole-brain radiotherapy or autologous stem-cell transplantation as consolidation strategies after high-dose methotrexate-based chemoimmunotherapy in patients with primary CNS lymphoma: results of the second randomisation of the International Extranodal Lymphoma Study Group-32 phase 2 trial.
Ferreri AJM, Cwynarski K, Pulczynski E, Fox CP, Schorb E, La Rosée P, Binder M, Fabbri A, Torri V, Minacapelli E, Falautano M, Ilariucci F, Ambrosetti A, Roth A, Hemmaway C, Johnson P, Linton KM, Pukrop T, Sønderskov Gørløv J, Balzarotti M, Hess G, Keller U, Stilgenbauer S, Panse J, Tucci A, Orsucci L, Pisani F, Levis A, Krause SW, Schmoll HJ, Hertenstein B, Rummel M, Smith J, Pfreundschuh M, Cabras G, Angrilli F, Ponzoni M, Deckert M, Politi LS, Finke J, Reni M, Cavalli F, Zucca E, Illerhaus G; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2017 Nov;4(11):e510-e523. Epub 2017 Oct 17 doi: 10.1016/S2352-3026(17)30174-6. PMID: 29054815
Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial.
Ferreri AJ, Cwynarski K, Pulczynski E, Ponzoni M, Deckert M, Politi LS, Torri V, Fox CP, Rosée PL, Schorb E, Ambrosetti A, Roth A, Hemmaway C, Ferrari A, Linton KM, Rudà R, Binder M, Pukrop T, Balzarotti M, Fabbri A, Johnson P, Gørløv JS, Hess G, Panse J, Pisani F, Tucci A, Stilgenbauer S, Hertenstein B, Keller U, Krause SW, Levis A, Schmoll HJ, Cavalli F, Finke J, Reni M, Zucca E, Illerhaus G; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2016 May;3(5):e217-27. Epub 2016 Apr 6 doi: 10.1016/S2352-3026(16)00036-3. PMID: 27132696
First-line treatment for primary testicular diffuse large B-cell lymphoma with rituximab-CHOP, CNS prophylaxis, and contralateral testis irradiation: final results of an international phase II trial.
Vitolo U, Chiappella A, Ferreri AJ, Martelli M, Baldi I, Balzarotti M, Bottelli C, Conconi A, Gomez H, Lopez-Guillermo A, Martinelli G, Merli F, Novero D, Orsucci L, Pavone V, Ricardi U, Storti S, Gospodarowicz MK, Cavalli F, Sarris AH, Zucca E
J Clin Oncol 2011 Jul 10;29(20):2766-72. Epub 2011 Jun 6 doi: 10.1200/JCO.2010.31.4187. PMID: 21646602

Prognosis

Marginal-Zone Lymphomas.
Rossi D, Bertoni F, Zucca E
N Engl J Med 2022 Feb 10;386(6):568-581. doi: 10.1056/NEJMra2102568. PMID: 35139275
Genetic and phenotypic attributes of splenic marginal zone lymphoma.
Bonfiglio F, Bruscaggin A, Guidetti F, Terzi di Bergamo L, Faderl M, Spina V, Condoluci A, Bonomini L, Forestieri G, Koch R, Piffaretti D, Pini K, Pirosa MC, Cittone MG, Arribas A, Lucioni M, Ghilardi G, Wu W, Arcaini L, Baptista MJ, Bastidas G, Bea S, Boldorini R, Broccoli A, Buehler MM, Canzonieri V, Cascione L, Ceriani L, Cogliatti S, Corradini P, Derenzini E, Devizzi L, Dietrich S, Elia AR, Facchetti F, Gaidano G, Garcia JF, Gerber B, Ghia P, Gomes da Silva M, Gritti G, Guidetti A, Hitz F, Inghirami G, Ladetto M, Lopez-Guillermo A, Lucchini E, Maiorana A, Marasca R, Matutes E, Meignin V, Merli M, Moccia A, Mollejo M, Montalban C, Novak U, Oscier DG, Passamonti F, Piazza F, Pizzolitto S, Rambaldi A, Sabattini E, Salles G, Santambrogio E, Scarfò L, Stathis A, Stüssi G, Geyer JT, Tapia G, Tarella C, Thieblemont C, Tousseyn T, Tucci A, Vanini G, Visco C, Vitolo U, Walewska R, Zaja F, Zenz T, Zinzani PL, Khiabanian H, Calcinotto A, Bertoni F, Bhagat G, Campo E, De Leval L, Dirnhofer S, Pileri SA, Piris MA, Traverse-Glehen A, Tzankov A, Paulli M, Ponzoni M, Mazzucchelli L, Cavalli F, Zucca E, Rossi D
Blood 2022 Feb 3;139(5):732-747. doi: 10.1182/blood.2021012386. PMID: 34653238
Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free PMC Article
Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial.
Ferreri AJ, Cwynarski K, Pulczynski E, Ponzoni M, Deckert M, Politi LS, Torri V, Fox CP, Rosée PL, Schorb E, Ambrosetti A, Roth A, Hemmaway C, Ferrari A, Linton KM, Rudà R, Binder M, Pukrop T, Balzarotti M, Fabbri A, Johnson P, Gørløv JS, Hess G, Panse J, Pisani F, Tucci A, Stilgenbauer S, Hertenstein B, Keller U, Krause SW, Levis A, Schmoll HJ, Cavalli F, Finke J, Reni M, Zucca E, Illerhaus G; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2016 May;3(5):e217-27. Epub 2016 Apr 6 doi: 10.1016/S2352-3026(16)00036-3. PMID: 27132696
First-line treatment for primary testicular diffuse large B-cell lymphoma with rituximab-CHOP, CNS prophylaxis, and contralateral testis irradiation: final results of an international phase II trial.
Vitolo U, Chiappella A, Ferreri AJ, Martelli M, Baldi I, Balzarotti M, Bottelli C, Conconi A, Gomez H, Lopez-Guillermo A, Martinelli G, Merli F, Novero D, Orsucci L, Pavone V, Ricardi U, Storti S, Gospodarowicz MK, Cavalli F, Sarris AH, Zucca E
J Clin Oncol 2011 Jul 10;29(20):2766-72. Epub 2011 Jun 6 doi: 10.1200/JCO.2010.31.4187. PMID: 21646602

Clinical prediction guides

Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of Central Nervous System Recurrence.
Ollila TA, Olszewski AJ
Curr Treat Options Oncol 2018 Jun 21;19(8):38. doi: 10.1007/s11864-018-0555-8. PMID: 29931605Free PMC Article
Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial.
Ferreri AJ, Cwynarski K, Pulczynski E, Ponzoni M, Deckert M, Politi LS, Torri V, Fox CP, Rosée PL, Schorb E, Ambrosetti A, Roth A, Hemmaway C, Ferrari A, Linton KM, Rudà R, Binder M, Pukrop T, Balzarotti M, Fabbri A, Johnson P, Gørløv JS, Hess G, Panse J, Pisani F, Tucci A, Stilgenbauer S, Hertenstein B, Keller U, Krause SW, Levis A, Schmoll HJ, Cavalli F, Finke J, Reni M, Zucca E, Illerhaus G; International Extranodal Lymphoma Study Group (IELSG)
Lancet Haematol 2016 May;3(5):e217-27. Epub 2016 Apr 6 doi: 10.1016/S2352-3026(16)00036-3. PMID: 27132696
Gela histological scoring system for post-treatment biopsies of patients with gastric MALT lymphoma is feasible and reliable in routine practice.
Copie-Bergman C, Wotherspoon AC, Capella C, Motta T, Pedrinis E, Pileri SA, Bertoni F, Conconi A, Zucca E, Ponzoni M, Ferreri AJ
Br J Haematol 2013 Jan;160(1):47-52. Epub 2012 Oct 9 doi: 10.1111/bjh.12078. PMID: 23043300
Prognostic scoring system for primary CNS lymphomas: the International Extranodal Lymphoma Study Group experience.
Ferreri AJ, Blay JY, Reni M, Pasini F, Spina M, Ambrosetti A, Calderoni A, Rossi A, Vavassori V, Conconi A, Devizzi L, Berger F, Ponzoni M, Borisch B, Tinguely M, Cerati M, Milani M, Orvieto E, Sanchez J, Chevreau C, Dell'Oro S, Zucca E, Cavalli F
J Clin Oncol 2003 Jan 15;21(2):266-72. doi: 10.1200/JCO.2003.09.139. PMID: 12525518
MALT Lymphomas.
Cavalli F, Isaacson PG, Gascoyne RD, Zucca E
Hematology Am Soc Hematol Educ Program 2001:241-58. doi: 10.1182/asheducation-2001.1.241. PMID: 11722987

Recent systematic reviews

Advanced Ann Arbor stage and age over 60 years as prognostic predictors in patients with primary cervical lymphoma: a retrospective cohort study and systematic review.
Gao L, Chen X, Zhao J, Xu A, Liu M, Yu H, Kong S, Li S
BMC Cancer 2023 Jan 27;23(1):95. doi: 10.1186/s12885-023-10548-4. PMID: 36707772Free PMC Article
Systematic review reveals urgent need to homogenize endpoints choices and definitions in marginal zone lymphomas trials.
Bommier C, Ruggiu M, Monégier A, Zucca E, Thieblemont C, Lambert J
Leuk Lymphoma 2022 Jul;63(7):1544-1555. Epub 2022 Feb 24 doi: 10.1080/10428194.2022.2032038. PMID: 35201907
Prevalence of Chlamydia psittaci, Chlamydia pneumoniae, and Chlamydia trachomatis Determined by Molecular Testing in Ocular Adnexa Lymphoma Specimens.
Travaglino A, Pace M, Varricchio S, Della Pepa R, Iuliano A, Picardi M, Pane F, Staibano S, Mascolo M
Am J Clin Pathol 2020 Mar 9;153(4):427-434. doi: 10.1093/ajcp/aqz181. PMID: 31755895
Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review.
van den Brand M, van Krieken JH
Haematologica 2013 Jul;98(7):1003-13. doi: 10.3324/haematol.2012.083386. PMID: 23813646Free PMC Article

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...