Staging of Marginal Zone Lymphoma (MZL)

Staging in nodal MZL is performed using the Lugano modification of the Ann Arbor staging system.1

FL Stages 1-41

Nodal MZL Stage Description1
Stage I MZL involves:

  • One lymph node or a group of adjacent nodes

Extranodal status: single extranodal lesions without nodal involvement

Stage II MZL involves:

  • Two or more nodal groups on the same side of the diaphragm

Extranodal status: stage I or II by nodal extent with limited contiguous extranodal involvement

Stage II bulky MZL involves:

  • Stage II as above with “bulky” disease
Stage III MZL involves:

  • Nodes on both sides of the diaphragm
  • Nodes above the diaphragm with spleen involvement
Stage IV MZL is in its most advanced stage, with:

  • Additional non-contiguous extranodal involvement

Staging of MALT lymphoma is not standardized. Gastric MALT lymphoma is typically staged using the Lugano staging system for GI lymphomas, which is a modification of the Ann Arbor staging system.2

Gastric MALT TNM Staging Description3
Stage I Disease confined to the GI tract (single primary or multiple non-contiguous lesions

  • Stage I1 - infiltration is limited to mucosa or submucosa
  • Stage I2 - infiltration is present in the muscularis propria, serosa, or both
Stage II Disease extending into the abdomen

  • Stage II1 - localized nodal involvement (perigastric lymph nodes)
  • Stage II2 - distant nodal involvement
Stage IIE Penetration of serosa to involve adjacent organs or tissues

  • Subscripts (1 or 2) may be added to the designation if both the lymph nodes and adjacent organs are involved
Stage IV Disseminated extranodal involvement or concomitant supradiaphragmatic nodal involvement

References: 1. Cheson BD et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059-3067. 2. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.4.2019. © National Comprehensive Cancer Network, Inc. 2019. All rights reserved. Accessed July 10, 2019. To view the most recent and complete version of the guideline, go online to NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way. 3. Zucca E, Bertoni F, Yahalom J, Isaacson P. Extranodal Marginal Zone B-cell Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT lymphoma) in Armitage et al eds. Non-Hodgkin's Lymphomas. Philadelphia: Lippincott, 2010:242.