eMedicine Specialties > Hematology > Stem Cells and Disorders

Lymphoma, Mediastinal: Follow-up

Author: Sonali M Smith, MD, Associate Professor, Section of Hematology/Oncology, Associate Director, Lymphoma Program, 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: Dec 8, 2009

Follow-up

Further Inpatient Care

  • The vast majority of patients can be successfully treated on an outpatient basis for front-line care.
  • Patients with relapsed or refractory disease, as demonstrated in the image below, are typically considered for stem cell transplantation. In this case, the reinduction of remission with chemotherapy as well as the high dose chemotherapy used for transplant are usually administered in the inpatient setting.
Isolated CNS relapse of primary mediastinal B-cel...

Isolated CNS relapse of primary mediastinal B-cell lymphoma.

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Isolated CNS relapse of primary mediastinal B-cel...

Isolated CNS relapse of primary mediastinal B-cell lymphoma.


Further Outpatient Care

  • Chemotherapy is usually administered on an outpatient basis every 2-3 weeks. CT and gallium (or positron emission tomography [PET]) scans are used to evaluate responses. The authors usually continue treatment for a minimum of 6 cycles of intensive chemotherapy. Patients who continue to harbor a positive mass based on PET scan findings after 6 cycles are offered autologous stem cell transplantation.
  • After completion of treatment, patients are usually seen in the outpatient clinic, at regular intervals of 2-3 months, for the first year.
  • Late adverse effects related to treatment include decreased fertility, a slightly increased incidence of secondary cancers in radiation fields (especially breast cancer among women treated during adolescence), and a slightly increased risk for secondary leukemia, especially among patients treated with combined modality therapy (ie, chemotherapy and radiation). In addition, coronary artery disease may be more common and may have an earlier onset if substantial areas of the heart are exposed to radiation. Smoking and alcohol abuse should be avoided because of their association with cancer and heart disease.

Complications

  • Neutropenic fever and infection are common complications of chemotherapy and require immediate treatment. Quinolone antibiotics such as levofloxacin or ofloxacin are often administered to prevent such infections, although their use remains somewhat controversial. If an autologous transplant is considered, prophylactic antibacterials (quinolone), antifungals (fluconazole), and antivirals (acyclovir or valacyclovir) are generally used.
  • Nausea, vomiting, and hair loss are common. Effective nausea prevention typically includes H2 antagonists.
  • Cardiac toxicity due to chemotherapy is unusual but can occur. Typically, patients undergo a multiple gated acquisition (MUGA) scan to evaluate the left ventricular ejection fraction prior to the initiation of chemotherapy. An MUGA scan is performed in most centers only if clinical concerns arise in regard to cardiomyopathy.
  • Mild peripheral neuropathy due to chemotherapy is common. Patients experience numbness in fingertips and toes. Motor neuropathy is unusual.
  • Rituximab can cause fever, chills, and, occasionally, hypotension. This is particularly common during the first cycle of treatment but is unusual with subsequent treatments. Rare anaphylactic reactions have been reported. Recent reports have shown that some patients with hematologic malignancies who were treated with rituximab have had hepatitis B virus (HBV) reactivation, with fulminant hepatitis, hepatic failure, and death. Persons at high risk of HBV infection should be screened before the initiation of Rituxan. Carriers of HBV should be closely monitored for clinical and laboratory signs of active HBV infection and for signs of hepatitis during and up to several months after rituximab (Rituxan) therapy.
  • Late adverse effects related to treatment include decreased fertility, a slightly increased incidence for secondary cancers in radiation fields (especially breast cancer among women treated during adolescence), and a slightly increased risk for secondary leukemia, especially among those treated with combined modality therapy (ie, chemotherapy and radiation). In addition, coronary artery disease may be more common and may have an earlier onset if substantial areas of the heart are exposed to radiation. Smoking and alcohol abuse should be avoided because of their association with cancer and heart disease.

Prognosis

  • Of all patients, 50-80% are cured with the initial treatment.
  • If the gallium (or PET) scan findings are negative after treatment and a complete radiological remission is obtained, the prognosis is excellent.
  • Practically all recurrences occur in the first year after diagnosis. A patient in continuous remission for 2 years after the initial diagnosis is highly likely to be cured.
  • Patients who do not respond to the initial treatment or who develop disease recurrence should undergo high-dose chemotherapy and autologous transplantation. Approximately one third of these patients achieve long-term remissions.
  • Neither the International Prognostic Index (IPI) nor the age-adjusted IPI (aaIPI) are necessarily predictive of outcome, and new prognostic models are needed.

Patient Education

For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Lymphoma.

Miscellaneous

Medicolegal Pitfalls

  • The differential diagnoses with other malignancies that involve the mediastinum are extremely important because they affect management and outcome.
  • Adequate diagnostic biopsy is needed and may require surgery. Ancillary studies, which include immunohistochemistry, immunophenotyping (flow cytometry), and gene rearrangement studies, are often necessary to establish the diagnosis.
 


More on Lymphoma, Mediastinal

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

  1. Miles RR, Mankey CC, Seiler CE 3rd, Smith LB, Teruya-Feldstein J, Hsi ED, et al. Expression of Grb2 distinguishes classical Hodgkin lymphomas from primary mediastinal B-cell lymphomas and other diffuse large B-cell lymphomas. Hum Pathol. Aug 26 2009;[Medline].

  2. Dubashi B, Cyriac S, Tenali SG. Clinicopathological analysis and outcome of primary mediastinal malignancies - A report of 91 cases from a single institute. Ann Thorac Med. Jul 2009;4(3):140-2. [Medline]. [Full Text].

  3. Wu D, Dutra B, Lindeman N, Takahashi H, Takeyama K, Harris NL, et al. No evidence for the JAK2 (V617F) or JAK2 exon 12 mutations in primary mediastinal large B-cell lymphoma. Diagn Mol Pathol. Sep 2009;18(3):144-9. [Medline].

  4. Sun W, Song K, Zervos M, Pass H, Cangiarella J, Bizekis C, et al. The diagnostic value of endobronchial ultrasound-guided needle biopsy in lung cancer and mediastinal adenopathy. Diagn Cytopathol. Nov 3 2009;[Medline].

  5. Zinzani PL, Stefoni V, Finolezzi E, Brusamolino E, Cabras MG, Chiappella A, et al. Rituximab combined with MACOP-B or VACOP-B and radiation therapy in primary mediastinal large B-cell lymphoma: a retrospective study. Clin Lymphoma Myeloma. Oct 2009;9(5):381-5. [Medline].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. 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].

  18. 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].

  19. 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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Keywords

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

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Contributor Information and Disclosures

Author

Sonali M Smith, MD, Associate Professor, Section of Hematology/Oncology, Associate Director, Lymphoma Program, 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/BiogenIdec Consulting fee Speaking and teaching; Lilly Consulting fee Speaking and teaching; Cephalon Consulting fee Consulting; Allos Consulting fee Speaking and teaching; Spectrum Pharmaceuticals Consulting fee Consulting

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 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: Nothing to disclose.

Medical Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Paul 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 Hematology, and Royal College of Physicians
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC 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, Southwest Medical Consultants, SC, Department of Medicine, 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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