Which systems are used to stage Burkitt lymphoma/Burkitt-like lymphoma (BL/BLL)?

Updated: Dec 20, 2019
  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Staging

Because most patients will present with nodal or extranodal masses, different staging systems have been proposed.

The National Cancer Institute staging system is as follows:

  • A - Single solitary extra-abdominal site
  • AR - Intra-abdominal, more than 90% of tumor resected
  • B - Multiple extra-abdominal tumors
  • C - Intra-abdominal tumor
  • D - Intra-abdominal plus 1 or more extra-abdominal sites

The Ann Arbor system and St. Jude/Murphy staging (commonly used) consist of 4 stages.

Stage I is as follows:

  • Single tumor (extranodal)
  • Single anatomic area (nodal)

Stage II is as follows:

  • Single tumor (extranodal) with regional node involvement
  • Primary gastrointestinal tumor
  • Lymphoma involving nodal areas on the same side of the diaphragm

Stage IIR consists of completely resected intra-abdominal disease.

Stage III is as follows:

  • Lymphoma involving sites on opposite sides of the diaphragm
  • All primary intrathoracic tumors
  • All paraspinal or epidural tumors
  • Extensive intra-abdominal disease

Stage IV consists of any of the above, with CNS or bone marrow involvement (< 25%) at presentation.

The risk-adapted approach is used to treat most patients in the present-day clinical setting. Patients are divided into 2 broad groups, low- and high-risk patients. Low-risk patients have nonbulky disease (< 10 cm), early stage (I or II) disease, good performance status, and a normal lactate dehydrogenase (LDH) level. High-risk patients include all other patients.


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