Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
ICD-O-3 Morphology
Effective 2001 and later
for cases diagnosed 2001 and later
Primary Site(s)
See Abstractor Notes and Module 7

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6 - B-cell
Module Rule
Alternate Names
BALT lymphoma
Bronchial-associated lymphoid tissue lymphoma
MALT lymphoma
Marginal zone B-cell lymphoma, NOS
Marginal zone lymphoma, NOS
Monocytoid B-cell lymphoma
Mucosal-associated lymphoid tissue lymphoma
Nodal marginal zone lymphoma
Nodal MZL
Parafollicular B-cell lymphoma
Pediatric nodal marginal zone lymphoma
SALT lymphoma
Skin-associated lymphoid tissue lymphoma
Extranodal involving MALT tissue, nodal without evidence of MALT or splenic disease. Lymphoma derived from B cells present in marginal zone, outer part of mantle zone of B-cell follicles. Mantle zone of follicles more developed in lymphoid organs facing high influx of antigens, representing mucosal immune system.
Abstractor Notes
Do not code the primary site to spleen for this histology. If the primary site is spleen, code the histology to 9689/3 (splenic marginal zone lymphoma)

50% of all cases of MALT lymphoma occur in the gastrointestinal tract. Within the GI tract, the stomach is the most common primary site. Other sites of involvement include salivary gland, lung, head and neck, ocular adnexa, skin, thyroid, and breast. The majority of patients present with stage I or II disease. A minority have bone marrow (BM) involvement. The frequency of BM involvement is lower in gastric cases and higher in MALT lymphomas arising in the lung and occular adnexa. Multiple extranodal sites may be involved in up to 25% of extragastric cases at the time of presentation. MALT lymphomas may have involvement of multiple extranodal sites, particularly of paired organs (e.g. salivary glands) or organ systems (e.g. gastrointestinal tract, skin). The differential diagnosis includes the history of reactive inflammatory process that typically precedes the lymphoma including: Heliobacter pylori, gastritis, lymphoepithelial sialadenitis, Hashimoto thyroiditis, and other small B-cell lymphomas (follicular lymphoma, mantle cell lymphoma, small lymphocytic lymphoma). The demonstration of immunoglobulin light chain restriction is important in the differential diagnosis.

There are three variants of MALT: IPSID, nodal marginal zone lymphoma, and pediatric nodal marginal zone lymphomas. All are coded to 9699/3.

IPSID is the abbreviation for immunoproliferative small intestinal disease. This variant was recently added to the growing list of infectious pathogen-associated human lymphomas. IPSID involves mainly the proximal small intestine. Geographically, IPSID is most prevalent in the Middle East and Africa.

Nodal marginal zone lymphoma (NMZL) usually presents with involvement of peripheral lymph nodes. Occasionally the bone marrow and peripheral blood are involved. NMZL is a primary B-cell neoplasm that morphologically resembles lymph nodes involved by marginal zone lymphoma of extranodal or splenic types, but without evidence of extranodal or splenic disease. The prognosis for pediatric NMZL is excellent with very low relapse rates and long survival after conservative treatment.

Pediatric nodal marginal zone lymphoma in the pediatric age group has distinct clinical and morphological characteristics. It presents predominantly in males and with localized (90% are Stage I) disease, mainly in the head and neck lymph nodes. Studies for clonal rearrangements of IGH@chain are necessary to help distinguish pediatric NMZL from reactive conditions.
Definitive Diagnostic Methods
Histological confirmation
Genetics Data
Chromosomal translocations t(11;18)
Immunoglobulin heavy and light chain genes rearranged
Mutated VH3
Mutated VH4
Production of a chimeric protein (AP12-MALTq,FOXP1) respectively
Translocations (q21, q21)
Translocations t(1;14)(p22;q32)
Translocations t(3;14)(p14.1;q32)
Translocations t(14;18)(q32;q21)
Trisomy 3, 18
CD43 coexpression
Cyclin D1-
IgM expression
Tumor cells may express IgA
Tumor cells may express IgG
Other RX
Transformations from
Corresponding ICD-9 Codes
200.3 Marginal zone lymphoma
Corresponding ICD-10 Codes
C83.0 Diffuse non-Hodgkin's lymphoma, small cell (diffuse)
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C88.4 Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT lymphoma]
C83.0 Small cell B-cell lymphoma
Signs and Symptoms
Diagnostic Exams
Recurrence and Metastases
Epidemiology and Mortality