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Hodgkin Diagnosis

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Adult Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version

Stage Information for Adult HL

  • Ann Arbor Classification System
  • Prognostic Groups

Clinical staging for patients with adult Hodgkin lymphoma (HL) includes the following:

  • Physical examination and history.
  • Laboratory studies (including sedimentation rate).
  • Thoracic and abdominal/pelvic computerized tomographic (CT) scans with or without positron emission tomography (PET).[1] PET scans combined with CT scans have become the standard imaging for clinical staging.[2]

Staging laparotomy is no longer recommended and should be considered only when the results will allow substantially less treatment. Staging laparotomy should not be done in patients who require chemotherapy. If the laparotomy is required for treatment decisions, the risks of potential morbidity should be considered.[3-6]

Bone marrow involvement occurs in 5% of patients; marrow involvement is more prevalent in the context of constitutional B symptoms and anemia, leukopenia, or thrombocytopenia. In a retrospective review and meta-analysis of 955 patients in nine studies, fewer than 2% of patients with positive bone marrow biopsy results had only stage I or stage II disease on PET-CT scans.[7] Omission of the bone marrow biopsy for PET-CT–designated early-stage patients did not change treatment selection.[7] In addition, focal skeletal bone lesions on PET-CT predicted bone marrow involvement with a 96.9% (93.0%–99.08%) sensitivity and 99.7% (98.9%–100%) specificity.[7] For these reasons, PET-CT has replaced bone marrow biopsy in the clinical staging of newly diagnosed HL.

Massive mediastinal disease has been defined by the Cotswolds meeting as a thoracic ratio of maximum transverse mass diameter of 33% or more of the internal transverse thoracic diameter measured at the T5/6 intervertebral disc level on chest radiography.[1] Some investigators have designated a lymph node mass measuring 10 cm or more in greatest dimension as massive disease.[8] Other investigators use a measurement of the maximum width of the mediastinal mass divided by the maximum intrathoracic diameter.[9]

The E designation is used when well-localized extranodal lymphoid malignancies arise in or extend to tissues beyond, but near, the major lymphatic aggregates. Stage IV refers to disease that is diffusely spread throughout an extranodal site, such as the liver. If pathologic proof of involvement of one or more extralymphatic sites has been documented, the symbol for the site of involvement, followed by a plus sign (+), is listed.

 

Table 1. Notations for Identifying Sites

N = nodesH = liverL = lungM = bone marrow
S = spleenP = pleuraO = boneD = skin

Ann Arbor Classification System

The staging classification that is currently used for HL was adopted in 1971 at the Ann Arbor Conference,[10] with some modifications 18 years later from the Cotswolds meeting.[1] The American Joint Committee on Cancer (AJCC) has adopted the Ann Arbor classification system to classify the anatomic extent of disease in adult HL.[11]

 

Table 2. Anatomic Stage/Prognostic Groups

StageDescription
Reprinted with permission from AJCC: Hodgkin and non-Hodgkin lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 607-11.
b) Involvement of extralymphatic sites is designated by the letter E.
c) The number of regions involved may be indicated by an arabic numeral, as in, for example, II3.
d) Splenic involvement is designated by the letter S.
IInvolvement of a single lymphatic site (i.e., nodal region, Waldeyer ring, thymus or spleen) (I).
Localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE)b (rare in Hodgkin lymphoma).
IIInvolvement of ≥2 lymph node regions on the same side of the diaphragm (II).
Localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).c
IIIInvolvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE, S). d
IVDiffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement.
Isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s).
Includes any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.

Prognostic Groups

Many investigators and many new clinical trials employ a clinical staging system that divides patients into three major groups that are also useful for the clinician:[12]

  • Early favorable.
  • Early unfavorable.
  • Advanced.

The group assignment depends on:

  • Whether the patient has early or advanced disease.
  • The type and number of adverse prognostic factors present.

Early-stage adverse prognostic factors:

  • Large mediastinal mass (>33% of the thoracic width on chest x-ray, ≥10 cm on CT scan).
  • Extranodal involvement.
  • Elevated erythrocyte sedimentation rate (>30 mm/h for B stage [symptoms], >50 mm/h for A stage [symptoms]).
  • Involvement of three or more lymph node areas.
  • Presence of B symptoms.

Early favorable group: Clinical stage I or II without any of the adverse prognostic factors listed above.

Early unfavorable group: Clinical stage I or II with one or more of the adverse prognostic factors listed above.

Advanced-stage adverse prognostic factors:

For patients with advanced-stage HL, the International Prognostic Factors Project on Advanced Hodgkin’s Disease developed the International Prognostic Index with a score that is based on the following seven adverse prognostic factors:[13]

  • Albumin level lower than 4.0 g/dL.
  • Hemoglobin level lower than 10.5 g/dL.
  • Male sex.
  • Age 45 years or older.
  • Stage IV disease.
  • White blood cell (WBC) count of 15,000/mm3 or higher.
  • Absolute lymphocytic count lower than 600/mm3 or lymphocyte count higher than 8% of the total WBC count.

Advanced group: Clinical stage III or IV with up to three of the adverse risk factors listed above. Patients with advanced disease have a 60% to 80% freedom from progression of disease at 5 years from treatment with first-line chemotherapy.[13][Level of evidence: 3iiiDiii]

 

References

  1. 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 7 (11): 1630-6, 1989. [PUBMED Abstract]
  2. 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 127 (12): 1531-8, 2016. [PUBMED Abstract]
  3. Urba WJ, Longo DL: Hodgkin’s disease. N Engl J Med 326 (10): 678-87, 1992. [PUBMED Abstract]
  4. Sombeck MD, Mendenhall NP, Kaude JV, et al.: Correlation of lymphangiography, computed tomography, and laparotomy in the staging of Hodgkin’s disease. Int J Radiat Oncol Biol Phys 25 (3): 425-9, 1993. [PUBMED Abstract]
  5. Mauch P, Larson D, Osteen R, et al.: Prognostic factors for positive surgical staging in patients with Hodgkin’s disease. J Clin Oncol 8 (2): 257-65, 1990. [PUBMED Abstract]
  6. Dietrich PY, Henry-Amar M, Cosset JM, et al.: Second primary cancers in patients continuously disease-free from Hodgkin’s disease: a protective role for the spleen? Blood 84 (4): 1209-15, 1994. [PUBMED Abstract]
  7. 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 25 (5): 921-7, 2014. [PUBMED Abstract]
  8. Bradley AJ, Carrington BM, Lawrance JA, et al.: Assessment and significance of mediastinal bulk in Hodgkin’s disease: comparison between computed tomography and chest radiography. J Clin Oncol 17 (8): 2493-8, 1999. [PUBMED Abstract]
  9. Mauch P, Goodman R, Hellman S: The significance of mediastinal involvement in early stage Hodgkin’s disease. Cancer 42 (3): 1039-45, 1978. [PUBMED Abstract]
  10. Carbone PP, Kaplan HS, Musshoff K, et al.: Report of the Committee on Hodgkin’s Disease Staging Classification. Cancer Res 31 (11): 1860-1, 1971. [PUBMED Abstract]
  11. Hodgkin and non-Hodgkin lymphomas. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 607-11.
  12. Jost LM, Stahel RA; ESMO Guidelines Task Force: ESMO Minimum Clinical Recommendations for diagnosis, treatment and follow-up of Hodgkin’s disease. Ann Oncol 16 (Suppl 1): i54-5, 2005. [PUBMED Abstract]
  13. 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 339 (21): 1506-14, 1998. [PUBMED Abstract]

 

Source:

https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq7835…