UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Staging and prognosis of Hodgkin lymphoma

Authors
Peter M Mauch, MD
George P Canellos, MD
Section Editor
Arnold S Freedman, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Once a diagnosis of Hodgkin lymphoma (HL, formerly called Hodgkin's disease) is made, the clinical stage of disease is determined to guide treatment and act as a measure of prognosis. The main goals of staging are to measure the extent of disease and associated prognostic factors, and to define disease manifestations that can be reevaluated during and after treatment to determine the effectiveness of therapy. Staging also serves an important role for the comparison of treatments among studies.

The TNM (tumor node metastasis) staging system that is generally used for solid tumors is not applicable to lymphoma, since it is based upon the concept of a primary tumor and metastasis. Instead, the staging system used for HL is an anatomical classification, which is based upon the concept that HL spreads in a predictable pattern of contiguous disease. HL starts at a single site within the lymphatic system, usually a lymph node, and then progresses to adjacent lymph nodes via lymphatic channels before disseminating to distant nonadjacent sites and organs.

Patients with HL are staged according to the Ann Arbor staging system with Cotswolds modifications [1,2]. This is a four-stage system (stages I to IV). The original Ann Arbor staging system was developed in 1974 and was principally based upon the use of staging laparotomy and lymphangiogram [1]. In 1988, the Cotswold modifications added valuable information regarding the presence of bulky disease and the efficacy of imaging studies for staging (table 1) [2]. In 2014, the Lugano classification proposed further revisions to clarify the role of positron emission tomography (PET) and better define extranodal involvement [3,4].

For treatment purposes, cases of HL are commonly classified as early stage disease (stage I to II) or advanced stage disease (stage III to IV). Patients with early stage HL are then further stratified into favorable and unfavorable subsets.

This topic review will discuss the staging of patients with HL. Other topic reviews discuss the clinical presentation, diagnosis, treatment, and other prognostic measures of these patients. (See "Epidemiology, pathologic features, and diagnosis of classical Hodgkin lymphoma" and "Initial evaluation and diagnosis of classical Hodgkin lymphoma in adults" and "Overview of the treatment of classical Hodgkin lymphoma in adults".)

                   

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Wed May 04 00:00:00 GMT+00:00 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Carbone PP, Kaplan HS, Musshoff K, et al. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res 1971; 31:1860.
  2. Lister TA, Crowther D, Sutcliffe SB, et al. Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin's disease: Cotswolds meeting. J Clin Oncol 1989; 7:1630.
  3. Cheson BD, Fisher RI, Barrington SF, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol 2014; 32:3059.
  4. Barrington SF, Mikhaeel NG, Kostakoglu L, et al. Role of imaging in the staging and response assessment of lymphoma: consensus of the International Conference on Malignant Lymphomas Imaging Working Group. J Clin Oncol 2014; 32:3048.
  5. Barrington SF, Mikhaeel NG. When should FDG-PET be used in the modern management of lymphoma? Br J Haematol 2014; 164:315.
  6. Hutchings M, Loft A, Hansen M, et al. Position emission tomography with or without computed tomography in the primary staging of Hodgkin's lymphoma. Haematologica 2006; 91:482.
  7. Naumann R, Beuthien-Baumann B, Reiss A, et al. Substantial impact of FDG PET imaging on the therapy decision in patients with early-stage Hodgkin's lymphoma. Br J Cancer 2004; 90:620.
  8. Barrington SF, Kirkwood AA, Franceschetto A, et al. PET-CT for staging and early response: results from the Response-Adapted Therapy in Advanced Hodgkin Lymphoma study. Blood 2016; 127:1531.
  9. Advani RH, Horning SJ. Treatment of early-stage Hodgkin's disease. Semin Hematol 1999; 36:270.
  10. Ng AK, Weeks JC, Mauch PM, Kuntz KM. Decision analysis on alternative treatment strategies for favorable-prognosis, early-stage Hodgkin's disease. J Clin Oncol 1999; 17:3577.
  11. Macintyre EA, Vaughan Hudson B, Linch DC, et al. The value of staging bone marrow trephine biopsy in Hodgkin's disease. Eur J Haematol 1987; 39:66.
  12. Howell SJ, Grey M, Chang J, et al. The value of bone marrow examination in the staging of Hodgkin's lymphoma: a review of 955 cases seen in a regional cancer centre. Br J Haematol 2002; 119:408.
  13. Vassilakopoulos TP, Angelopoulou MK, Constantinou N, et al. Development and validation of a clinical prediction rule for bone marrow involvement in patients with Hodgkin lymphoma. Blood 2005; 105:1875.
  14. El-Galaly TC, d'Amore F, Mylam KJ, et al. Routine bone marrow biopsy has little or no therapeutic consequence for positron emission tomography/computed tomography-staged treatment-naive patients with Hodgkin lymphoma. J Clin Oncol 2012; 30:4508.
  15. Adams HJ, Kwee TC, de Keizer B, et al. Systematic review and meta-analysis on the diagnostic performance of FDG-PET/CT in detecting bone marrow involvement in newly diagnosed Hodgkin lymphoma: is bone marrow biopsy still necessary? Ann Oncol 2014; 25:921.
  16. Munker R, Hasenclever D, Brosteanu O, et al. Bone marrow involvement in Hodgkin's disease: an analysis of 135 consecutive cases. German Hodgkin's Lymphoma Study Group. J Clin Oncol 1995; 13:403.
  17. Specht L. Prognostic Factors in Hodgkin's Disease. Semin Radiat Oncol 1996; 6:146.
  18. Crnkovich MJ, Hoppe RT, Rosenberg SA. Stage IIB Hodgkin's disease: the Stanford experience. J Clin Oncol 1986; 4:472.
  19. Crnkovich MJ, Leopold K, Hoppe RT, Mauch PM. Stage I to IIB Hodgkin's disease: the combined experience at Stanford University and the Joint Center for Radiation Therapy. J Clin Oncol 1987; 5:1041.
  20. Gobbi PG, Cavalli C, Gendarini A, et al. Reevaluation of prognostic significance of symptoms in Hodgkin's disease. Cancer 1985; 56:2874.
  21. Thar TL, Million RR, Hausner RJ, McKetty MH. Hodgkin's disease, stages I and II: relationship of recurrence to size of disease, radiation dose, and number of sites involved. Cancer 1979; 43:1101.
  22. Fabian CJ, Mansfield CM, Dahlberg S, et al. Low-dose involved field radiation after chemotherapy in advanced Hodgkin disease. A Southwest Oncology Group randomized study. Ann Intern Med 1994; 120:903.
  23. Bartlett NL, Rosenberg SA, Hoppe RT, et al. Brief chemotherapy, Stanford V, and adjuvant radiotherapy for bulky or advanced-stage Hodgkin's disease: a preliminary report. J Clin Oncol 1995; 13:1080.
  24. Musshoff K, Boutis L. Therapy results in Hodgkin's disease Freiburg i.Br., 1948-1966. Cancer 1968; 21:1100.
  25. Hasenclever D, Diehl V. A prognostic score for advanced Hodgkin's disease. International Prognostic Factors Project on Advanced Hodgkin's Disease. N Engl J Med 1998; 339:1506.
  26. Moccia AA, Donaldson J, Chhanabhai M, et al. International Prognostic Score in advanced-stage Hodgkin's lymphoma: altered utility in the modern era. J Clin Oncol 2012; 30:3383.
  27. Bierman PJ, Lynch JC, Bociek RG, et al. The International Prognostic Factors Project score for advanced Hodgkin's disease is useful for predicting outcome of autologous hematopoietic stem cell transplantation. Ann Oncol 2002; 13:1370.
  28. Klimm B, Goergen H, Fuchs M, et al. Impact of risk factors on outcomes in early-stage Hodgkin's lymphoma: an analysis of international staging definitions. Ann Oncol 2013; 24:3070.
  29. Hoppe RT, Coleman CN, Cox RS, et al. The management of stage I--II Hodgkin's disease with irradiation alone or combined modality therapy: the Stanford experience. Blood 1982; 59:455.
  30. Mauch P, Goodman R, Hellman S. The significance of mediastinal involvement in early stage Hodgkin's disease. Cancer 1978; 42:1039.
  31. Leopold KA, Canellos GP, Rosenthal D, et al. Stage IA-IIB Hodgkin's disease: staging and treatment of patients with large mediastinal adenopathy. J Clin Oncol 1989; 7:1059.
  32. Schomberg PJ, Evans RG, O'Connell MJ, et al. Prognostic significance of mediastinal mass in adult Hodgkin's disease. Cancer 1984; 53:324.
  33. Hughes-Davies L, Tarbell NJ, Coleman CN, et al. Stage IA-IIB Hodgkin's disease: management and outcome of extensive thoracic involvement. Int J Radiat Oncol Biol Phys 1997; 39:361.
  34. Mendenhall NP, Cantor AB, Barré DM, et al. The role of prognostic factors in treatment selection for early-stage Hodgkin's disease. Am J Clin Oncol 1994; 17:189.
  35. Bonadonna G, Valagussa P, Santoro A. Prognosis of bulky Hodgkin's disease treated with chemotherapy alone or combined with radiotherapy. Cancer Surv 1985; 4:439.
  36. Specht L, Nordentoft AM, Cold S, et al. Tumor burden as the most important prognostic factor in early stage Hodgkin's disease. Relations to other prognostic factors and implications for choice of treatment. Cancer 1988; 61:1719.
  37. Mauch P, Tarbell N, Weinstein H, et al. Stage IA and IIA supradiaphragmatic Hodgkin's disease: prognostic factors in surgically staged patients treated with mantle and paraaortic irradiation. J Clin Oncol 1988; 6:1576.
  38. Cosset JM, Henry-Amar M, Meerwaldt JH, et al. The EORTC trials for limited stage Hodgkin's disease. The EORTC Lymphoma Cooperative Group. Eur J Cancer 1992; 28A:1847.
  39. Roach M 3rd, Brophy N, Cox R, et al. Prognostic factors for patients relapsing after radiotherapy for early-stage Hodgkin's disease. J Clin Oncol 1990; 8:623.
  40. Healey EA, Tarbell NJ, Kalish LA, et al. Prognostic factors for patients with Hodgkin disease in first relapse. Cancer 1993; 71:2613.
  41. Mauch PM, Kalish LA, Marcus KC, et al. Long-term survival in Hodgkin's disease relative impact of mortality, second tumors, infection, and cardiovascular disease. Cancer J Sci Am 1995; 1:33.
  42. Henry-Amar M. Second cancers after radiotherapy and chemotherapy for early stages of Hodgkin's disease. J Natl Cancer Inst 1983; 71:911.
  43. Pedersen-Bjergaard J, Larsen SO. Incidence of acute nonlymphocytic leukemia, preleukemia, and acute myeloproliferative syndrome up to 10 years after treatment of Hodgkin's disease. N Engl J Med 1982; 307:965.
  44. Anderson H, Deakin DP, Wagstaff J, et al. A randomised study of adjuvant chemotherapy after mantle radiotherapy in supradiaphragmatic Hodgkin's disease PS IA-IIB: a report from the Manchester lymphoma group. Br J Cancer 1984; 49:695.
  45. Tubiana M, Henry-Amar M, Hayat M, et al. The EORTC treatment of early stages of Hodgkin's disease: the role of radiotherapy. Int J Radiat Oncol Biol Phys 1984; 10:197.
  46. Gospodarowicz MK, Sutcliffe SB, Clark RM, et al. Analysis of supradiaphragmatic clinical stage I and II Hodgkin's disease treated with radiation alone. Int J Radiat Oncol Biol Phys 1992; 22:859.
  47. Henry-Amar M, Friedman S, Hayat M, et al. Erythrocyte sedimentation rate predicts early relapse and survival in early-stage Hodgkin disease. The EORTC Lymphoma Cooperative Group. Ann Intern Med 1991; 114:361.
  48. Liao Z, Ha CS, Vlachaki MT, et al. Mantle irradiation alone for pathologic stage I and II Hodgkin's disease: long-term follow-up and patterns of failure. Int J Radiat Oncol Biol Phys 2001; 50:971.