Search Database ICD-O-3 Code Lists

Name

Mantle cell lymphoma (MCL)

ICD-O-3 Morphology

9673/3: Mantle cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, gastrointestinal tract, Waldeyer ring.
Common metastatic sites CNS, lung, and bone marrow/peripheral blood.
Spleen involvement is common due to dissemination of disease

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Mantle cell lymphoma (MCL) is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

MCL usually involves lymph nodes, but extranodal involvement is also common, particularly of the Waldeyer ring, gastrointestinal tract, which involvement can manifest with numerous polypoid lesions. Other organs that may be involved include the skin, endocrine glands, lungs, and CNS, most often occurring as relapsing disease.

Leukemic non-nodal mantle cell lymphoma (nnMCL) is a mantle cell that occurs in the blood, bone marrow and spleen (no involvement of organs or lymph nodes). Primary site for these cases is C421 (bone marrow).

Note: If the bone marrow biopsy is positive for mantle cell, don’t automatically assign primary site C421. Look for other involvement first, and if none, assign C421.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Cyclin D1-negative mantle cell lymphoma (Cyclin D1-neg MCL)
Cyclin D1-positive mantle cell lymphoma (Cyclin D1-pos MCL)
Leukemic non-nodal mantle cell lymphoma (nnMCL)
Malignant lymphoma, centrocytic [OBS]
Malignant lymphomatous polyposis
Mantle cell lymphoma, blastic/blastoid
Mantle cell lymphoma, marginal zone-like
Mantle cell lymphoma, small cell
Mantle zone lymphoma [OBS]

Definition

Mantle cell lymphoma (MCL) is a mature B-cell neoplasm derived from the mantle zone of lymphoid follicles and typically composed of small to medium-sized monomorphic cells expressing CD5, SOX11, and cyclin D1. It is associated with CCND-family rearrangements, most commonly CCND1. (WHO 5th edition)

Leukemic non-nodal mantle cell lymphoma (nnMCL) is characterized by the involvement of blood, bone marrow, and spleen by neoplastic cells with morphological and immunophenotypic similarities to nodal mantle cell lymphoma (MCL), with absent or minimal evidence of lymphadenopathy, and usually with an asymptomatic presentation. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

CCND1 rearrangement
CCND2 rearrangement

Immunophenotyping

CD5+ (expression/positive)
CD43+ (expression/positive)
CD79a+ (expression/positive)
Cyclin D1+ (expression/positive) (Cyclin D1-positive MCL)
Cyclin D1- (no expression/negative) (Cyclin D1-negative MCL)
FMC7+ (expression/positive)
SOX11+ (expression/positive)
CD10+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)

Treatments

Chemotherapy
Immunotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C83.1 Non-Hodgkin lymphoma small cleaved cell (diffuse)

Corresponding ICD-10-CM Codes (U.S. only)

C83.1_ Mantle cell lymphoma (effective October 01, 2015)
C83.1A Mantle cell lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Hepatosplenomegaly
Lymphocytosis
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: 60 years median age
Incidence: 3-10% of non-Hodgkin lymphomas
Sex: male predominance
Survival: 3-5 years median survival

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mantle Cell Lymphoma
Pages: Part B: 446-454

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Indolent B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/18/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/indolent-b-cell-lymphoma-treatment-pdq. Accessed <03/04/2025>. [PMID: 37437080]
Section: Indolent B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary