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ICD-O-1 Morphology
Effective
1992 - 2000
Reportable
for cases diagnosed
1992 and later
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, gastrointestinal tract, and Waldeyer ring
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Peripheral blood involvement common, present in almost all patients by flow cytometry. B symptoms in about half of patients.
Most patients present in Stage III or IV. Spleen, bone marrow and liver are common metastatic sites.
Patients presenting with peripheral blood, bone marrow and possibly splenic involvement but without adenopathy are reported to have better prognosis.
Most patients present in Stage III or IV. Spleen, bone marrow and liver are common metastatic sites.
Patients presenting with peripheral blood, bone marrow and possibly splenic involvement but without adenopathy are reported to have better prognosis.
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
Mantle zone lymphoma [OBS]
MCL
Definition
Mantle cell lymphoma is a mature B-cell neoplasm usually composed of monomorphic small to medium-sized lymphoid cells with irregular nuclear contours.
Leukemic non-nodal mantle cell lymphoma is defined as mantle cell lymphoma in which the patient presents with peripheral blood, bone marrow, and sometimes splenic involvement but without significant adenopathy.
There are several variants of Mantle cell lymphoma
1) Blastoid: An aggressive variant where cells resemble lymphoblasts with dispersed chromatin and a high mitotic rate.
2) Pleomorphic: An aggressive variant where cells are pleomorphic, but many are large with oval to irregular nuclear contours, generally pale cytoplasm, and other prominent nucleoli in at least some of the cells.
3) Small-cell: Cells are small round lymphocytes with more clumped chromatin, either admixed or predominant, mimicking a small lymphocytic lymphoma
4) Marginal zone-like: There are prominent foci of cells with abundant pale cytoplasm resembling marginal zone or monocytoid B cells, mimicking a marginal zone lymphoma, sometimes these paler foci also resemble proliferation centers of chronic lymphocytic leukemia/small lymphocytic lymphoma
Leukemic non-nodal mantle cell lymphoma is defined as mantle cell lymphoma in which the patient presents with peripheral blood, bone marrow, and sometimes splenic involvement but without significant adenopathy.
There are several variants of Mantle cell lymphoma
1) Blastoid: An aggressive variant where cells resemble lymphoblasts with dispersed chromatin and a high mitotic rate.
2) Pleomorphic: An aggressive variant where cells are pleomorphic, but many are large with oval to irregular nuclear contours, generally pale cytoplasm, and other prominent nucleoli in at least some of the cells.
3) Small-cell: Cells are small round lymphocytes with more clumped chromatin, either admixed or predominant, mimicking a small lymphocytic lymphoma
4) Marginal zone-like: There are prominent foci of cells with abundant pale cytoplasm resembling marginal zone or monocytoid B cells, mimicking a marginal zone lymphoma, sometimes these paler foci also resemble proliferation centers of chronic lymphocytic leukemia/small lymphocytic lymphoma
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Heavy and light chain genes rearranged, t(11;14)(q13;q32) in virtually all cases, overexpression CYCLIN D mRNA, mutation or deletions of ATM gene
Immunophenotyping
BCL2+ (expression/positive)
BCL6- (no expression/negative)
CD5+ (expression/positive)
CD10- (no expression/negative)
CD23- (no expression/negative) or weakly positive
CD43+ (expression/positive)
Cyclin D1+ (expression/positive)
FMC7+ (expression/positive)
IgM/IgD+ (expression/positive)
IRF4+ (expression/positive)
MUM1+ (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-9 Codes
200.4 Mantle cell lymphoma
Corresponding ICD-10 Codes
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)
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
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 285-290
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 285-290
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
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
National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
