eMedicine Specialties > Hematology > Stem Cells and Disorders

Lymphoma, Mediastinal

Author: Sonali M Smith, MD, Assistant Professor, Associate Director, Lymphoma Program, Department of Medicine, Section of Hematology/Oncology, The University of Chicago Medical Center
Coauthor(s): Koen W Van Besien, MD, Director of Stem Cell Transplantation and Lymphoma, Associate Professor, Department of Internal Medicine, Section of Hematology/Oncology, University of Chicago; Andrew S Artz, MD, Assistant Professor, Department of Medicine, Section of Hematology/Oncology, The University of Chicago Pritzker School of Medicine
Contributor Information and Disclosures

Updated: Oct 12, 2007

Introduction

Background

Primary mediastinal B-cell lymphoma (PMBL) is a diffuse large B-cell lymphoma that arises in the thymus and mainly affects young adults. PMBL was recognized as a specific entity in the Revised European-American Classification of Lymphoid Neoplasms (REAL) and accounts for approximately 5% of all patients with aggressive lymphomas, a frequency roughly similar to that of Burkitt lymphoma and lymphoblastic lymphoma. New evidence with gene expression profiling suggests that this disease may resemble Hodgkin lymphoma more so than other types of diffuse large B-cell lymphoma. Because of its skewed age distribution, PMBL accounts for a much higher proportion of both younger patients and those undergoing autologous transplantation. Fifty to eighty percent of patients are cured with a modern intensive combination chemotherapy that is often followed by involved field radiation or autologous transplantation.

Pathophysiology

A rapidly proliferating tumor that arises in the anterior mediastinum (see Image 1), PMBL often causes symptoms via the compression of intrathoracic structures (ie, superior vena cava syndrome) or via the invasion of lungs, pleura, and pericardium. The disease's spread to parenchymal organs, such as the liver, the kidneys, or the central nervous system, is common at the time of recurrence.

Frequency

United States

PMBL accounts for approximately 5% of the lymphomas in the Western world. It accounts for a higher percentage of lymphomas in younger people.

International

The incidence of PMBL outside of the Western world is not clearly defined. In a Japanese series of over 800 patients seen in a single university setting, roughly 7% had non-Hodgkin lymphoma of the mediastinum, but the specific histology is not reported.

Mortality/Morbidity

  • The disease has an aggressive course and, if untreated, causes death in those affected.
  • With appropriate management, 50-80% of patients can be cured.

Sex

PMBL is slightly more common in females than in males. This sex distribution is the opposite of that for other types of lymphoma.

Age

  • The median age at diagnosis is 30 years.
  • Very few patients diagnosed with PMBL are older than 50 years.

Clinical

History

  • Primary mediastinal B-cell lymphoma (PMBL) is a mediastinal tumor that grows rapidly and is sometimes palpable in the supraclavicular area.
  • Superior vena cava syndrome is common.
  • Phrenic nerve palsy, dysphagia, hoarseness, and breast swelling (in women) can occur.
  • Shortness of breath can be due to pleural effusion or massive mediastinal mass.
  • Systemic symptoms (fever, weight loss, night sweats) occur in 30% of patients.
  • If recurrence develops, a hematogenous pattern of spread to parenchymal organs, such as the liver, kidneys, or brain, is common.

Physical

  • Superior vena cava syndrome with congestion of face and upper extremities
  • Palpable mass in the supraclavicular area
  • Dullness at the lung bases
  • Respiratory distress
  • Peripheral adenopathy (unusual except in the supraclavicular area)

Causes

  • The cause is unknown.
  • Although a clear familial or genetic predisposition has not been identified, a Scandinavian registry study showed an increased risk of non-Hodgkin lymphoma for family members of patients with a prior diagnosis of lymphoma. Whether or not this is true for other populations is unknown.
  • No relationship to toxic or noxious agents exists.
  • A constitutive activation of NF-k B pathways, which induces cell proliferation (based on recent evidence). Amplification of the REL and JAK-2 genes may also contribute to pathogenesis.

More on Lymphoma, Mediastinal

Overview: Lymphoma, Mediastinal
Differential Diagnoses & Workup: Lymphoma, Mediastinal
Treatment & Medication: Lymphoma, Mediastinal
Follow-up: Lymphoma, Mediastinal
Multimedia: Lymphoma, Mediastinal
References
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References

  1. Altieri A, Bermejo JL, Hemminki K. Familial risk for non-Hodgkin lymphoma and other lymphoproliferative malignancies by histopathologic subtype: the Swedish Family-Cancer Database. Blood. Jul 15 2005;106(2):668-72. [Medline].

  2. Coiffier B, Lepage E, Briere J, Herbrecht R, Tilly H, Bouabdallah R, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med. Jan 24 2002;346:235-42. [Medline]. [Full Text].

  3. Feuerhake F, Kutok JL, Monti S, Chen W, LaCasce AS, Cattoretti G, et al. NF{kappa}B activity, function and target gene signatures in primary mediastinal large B-cell lymphoma and diffuse large B-cell lymphoma subtypes. Blood. 2005;May 3, e-pub ahead of print:1392-9. [Medline]. [Full Text].

  4. Hamlin PA, Portlock CS, Straus DJ, Noy A, Singer A, Horwitz SM, et al. Primary mediastinal large B-cell lymphoma: optimal therapy and prognostic factor analysis in 141 consecutive patients treated at Memorial Sloan Kettering from 1980 to 1999. Br J Haematol. Sep 2005;130(5):691-9. [Medline].

  5. Lazzarino M, Orlandi E, Paulli M, Strater J, Klersy C, Gianelli U, et al. Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients. J Clin Oncol. Apr 1997;15(4):1646-53. [Medline].

  6. Pfreundschuh M, Trumper L, Kloess M, Schmits R, Feller AC, Rube C, et al. Two-weekly or 3-weekly CHOP chemotherapy with or without etoposide for the treatment of elderly patients with aggressive lymphomas: results of the NHL-B2 trial of the DSHNHL. Blood. 2004;104:634-641. [Medline]. [Full Text].

  7. Popat U, Przepiork D, Champlin R, Pugh W, Amin K, Mehra R, et al. High-dose chemotherapy for relapsed and refractory diffuse large B-cell lymphoma: mediastinal localization predicts for a favorable outcome. J Clin Oncol. Jan 1998;16(1):63-9. [Medline].

  8. 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. Sep 15 2003;198:851-62. [Medline]. [Full Text].

  9. Savage KJ, Al-Rajhi N, Voss N, Paltiel C, Klasa R, Gascoyne RD, et al. Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Ann Oncol. Jan 2006;17(1):123-30. [Medline].

  10. 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. Dec 1 2003;102(12):3871-9. [Medline].

  11. 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. Journal Surgical Oncology. May 2003;83(1):24-30. [Medline].

  12. 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. Nov 2005;29(11):1411-21. [Medline].

  13. van Besien K, Kelta M, Bahaguna P. Primary mediastinal B-cell lymphoma: a review of pathology and management. Journal of Clinical Oncology. Mar 15 2001;19(6):1855-1864. [Medline]. [Full Text].

  14. Zinzani PL, Martelli M, Magagnoli M, Pescarmona E, Scaramucci L, Palombi F, et al. Treatment and clinical management of primary mediastinal large B-cell lymphoma with sclerosis: MACOP-B regimen and mediastinal radiotherapy monitored by (67)Gallium scan in 50 patients. Blood. Nov 15 1999;94(10):3289-93. [Medline]. [Full Text].

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Further Reading

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Keywords

primary mediastinal B-cell lymphoma, PMBL, B-cell neoplasm

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Contributor Information and Disclosures

Author

Sonali M Smith, MD, Assistant Professor, Associate Director, Lymphoma Program, Department of Medicine, Section of Hematology/Oncology, The University of Chicago Medical Center
Sonali M Smith, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, and American Society of Hematology
Disclosure: Genentech None Speaking and teaching

Coauthor(s)

Koen W Van Besien, MD, Director of Stem Cell Transplantation and Lymphoma, Associate Professor, Department of Internal Medicine, Section of Hematology/Oncology, University of Chicago
Koen W Van Besien, MD is a member of the following medical societies: American Society of Clinical Oncology and American Society of Hematology
Disclosure: Nothing to disclose.

Andrew S Artz, MD, Assistant Professor, Department of Medicine, Section of Hematology/Oncology, The University of Chicago Pritzker School of Medicine
Andrew S Artz, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, and American Society of Hematology
Disclosure: Amgen Grant/research funds Other; Amgen Consulting fee Consulting

Medical Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University
Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Clinical Oncology, American Society of Hematology, and Royal College of Physicians
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ronald A Sacher, MD, Director of the Hoxworth Blood Center, Professor, Departments of Internal Medicine and Pathology, University of Cincinnati Medical Center
Ronald A Sacher, MD is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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