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Lymphoma, Mediastinal: Differential Diagnoses & Workup

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

Differential Diagnoses

Acute Lymphoblastic Leukemia
Germ Cell Tumors
Hodgkin Disease
Lymphoma, Lymphoblastic
Lymphoma, Malignant Anaplastic (Ki 1+)
Thymoma

Other Problems to Be Considered

Carcinoma
Grey zone lymphoma (a very rare subtype of lymphoma that has pathobiologic features of both Hodgkin and non-Hodgkin lymphoma)

Workup

Laboratory Studies

  • Perform a CBC count with differential and platelets.
  • Perform electrolyte panel and liver function tests.
  • An elevated serum lactic dehydrogenase (LDH) value is an adverse prognostic feature.
  • An elevated b -2 microglobulin level is also an adverse prognostic feature.
  • The markers alpha-fetoprotein and b human chorionic gonadotropin (b HCG) are often highly elevated in patients with mediastinal germ cell tumors, constituting an important differential diagnosis in males.

Imaging Studies

  • Obtain a chest radiograph (posteroanterior, lateral). A mass larger than one third of the diameter of the thorax is considered large and indicates a poor prognosis.
  • Obtain CT scans (chest, abdomen, pelvis). Extension to the pleura, pericardium, and even the chest wall is common. Invasion of the liver, kidneys, and peripheral lymph nodes is more common at the time of recurrence.
  • Findings from a gallium scan are almost always strongly positive. Findings are negative following successful treatment. Recently, positron emission tomography (PET) scans are more widely used; PET represents a convenient, and probably more sensitive, alternative to a gallium scan. Several investigators have demonstrated that an early metabolic response (ie, complete resolution of all PET-avid disease) following 1-4 cycles of chemotherapy is predictive of an excellent outcome. Similarly, negative PET scan findings at the end of treatment carry a good prognosis. However, false-positive results of PET activity due to inflammation, thymic rebound, infection, or granulomatous disease can occur. The specific positive and negative predictive values of PET for patients with PMBL are not known.
  • Consider other imaging studies if they are clinically indicated (eg, head CT scan or MRI, if the patient has neurologic problems).

Other Tests

Consider performing a multiple gated acquisition (MUGA) scan to assess cardiac function before anthracycline-based chemotherapy.

Procedures

  • Bone marrow aspirate and biopsy are necessary for staging. A unilateral sample is sufficient if the biopsy specimen is larger than 2 cm.
  • Biopsy of the lymph node or of the mediastinal mass with the use of mediastinoscopy or parasternotomy is necessary. Fine-needle aspiration is usually not diagnostic in this disorder due to lack of morphologic architecture and the difficulty in distinguishing PMBL from other lymphomas.
  • Other tests should be performed if clinically indicated (eg, thoracentesis for pleural effusion, lumbar puncture for neurologic symptoms).

Histologic Findings

Diffuse infiltrate consists of large cells. Clear cells are common. Fibrosis occurs variably in some areas of the tumor. Immunophenotype is as follows: CD19+ and CD20+, but CD21- and HLA-DR- (in contrast to other diffuse large B-cell lymphomas). The B-cell receptor gene is rearranged.

Staging

  • Perform a physical examination with attention to lymphadenopathy, splenomegaly, and hepatomegaly. Performance status should be noted because this is an important prognostic indicator. Routine laboratory studies include serum lactic acid dehydrogenase (LDH) and serum b -2 microglobulin.
  • Obtain a chest radiograph (posteroanterior and lateral). A mass larger than one third of the diameter of the thorax is considered large and indicates a poor prognosis.
  • Obtain CT scans of the chest, abdomen, and pelvis. In addition, obtain a positron emission tomography (PET) or gallium scan.
    • Extension to the pleura, the pericardium, and even the chest wall is common.
    • Invasion of the liver, the kidneys, and the peripheral lymph nodes is more common at the time of recurrence.
  • Gallium scan findings are almost always positive. Negative findings after treatment indicate a low risk of recurrence.
  • Unilateral bone marrow aspirate and biopsy: Bone marrow involvement is very unusual in this type of lymphoma.
  • Other tests, such as thoracentesis and lumbar puncture, should be considered when clinically indicated.

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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