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Name
Follicular lymphoma, grade 2
ICD-O-2 Morphology
9691/3: Follicular lymphoma, grade 2
Effective
1992 - 2000
ICD-O-3 Morphology
9691/3: Follicular lymphoma, grade 2
Effective
2001 and later
Reportable
for cases diagnosed
1992 and later
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, spleen, bone marrow (BM), peripheral blood (PB), and Waldeyer ring, skin, duodenum, ocular adnex, breast and testis
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Follicular lymphoma, NOS (9690/3) histology is a generic disease description. DCO cases or path report only cases usually stay in this classification. The NOS histology may be the working diagnosis. Further review of the medical record should be done to look for the tests listed as definitive diagnosis.
The more specific follicular lymphomas are:
1. Follicular lymphoma, grade 1 (9695/3)
2. Follicular lymphoma, grade 2 (9691/3)
3. Follicular lymphoma, grade 3 [3A, 3B] (9698/3)
When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code.
FL is graded according to the proportion of large cells (centroblasts). Studies suggest this histologic grading predicts clinical outcome, with more large cells behaving more aggressively and having a higher likelihood of transformation to diffuse large cell lymphoma.
1) Grade 1-2 (low grade): 0-15 centroblasts per HPF (9691/3)
2) Grade 1: 0-5 centroblasts per HPF (9695/3)
3) Grade 2: 6-15 centroblasts per HPF (9691/3)
4) Grade 3: > 15 centroblasts per HPF (9698/3)
5) Grade 3A: Centrocytes present (9698/3)
6) Grade 3B: Solid sheets of centroblasts (9698/3)
Most patients present with widespread disease, including peripheral and central (abdominal and thoracic) lymphadenopathy and splenomegaly. The BM is involved in 40-70%.
Only 1/3 of patients present with stage I or II at the time of diagnosis.
When any area of diffuse large-B-cell lymphoma (DLBCL) is present in a FL the disease should be reported as diffuse large B-cell lymphoma (9680/3) (See PH rules).
For more information on lymphoma, see the NCI website: http://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq#section/_1 or http://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq#section/_129
The more specific follicular lymphomas are:
1. Follicular lymphoma, grade 1 (9695/3)
2. Follicular lymphoma, grade 2 (9691/3)
3. Follicular lymphoma, grade 3 [3A, 3B] (9698/3)
When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code.
FL is graded according to the proportion of large cells (centroblasts). Studies suggest this histologic grading predicts clinical outcome, with more large cells behaving more aggressively and having a higher likelihood of transformation to diffuse large cell lymphoma.
1) Grade 1-2 (low grade): 0-15 centroblasts per HPF (9691/3)
2) Grade 1: 0-5 centroblasts per HPF (9695/3)
3) Grade 2: 6-15 centroblasts per HPF (9691/3)
4) Grade 3: > 15 centroblasts per HPF (9698/3)
5) Grade 3A: Centrocytes present (9698/3)
6) Grade 3B: Solid sheets of centroblasts (9698/3)
Most patients present with widespread disease, including peripheral and central (abdominal and thoracic) lymphadenopathy and splenomegaly. The BM is involved in 40-70%.
Only 1/3 of patients present with stage I or II at the time of diagnosis.
When any area of diffuse large-B-cell lymphoma (DLBCL) is present in a FL the disease should be reported as diffuse large B-cell lymphoma (9680/3) (See PH rules).
For more information on lymphoma, see the NCI website: http://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq#section/_1 or http://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq#section/_129
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
See Abstractor Notes
Module Rule
None
Definition
Follicular lymphoma (FL) is a neoplasm composed of follicle centre (germinal center) B cells (typically both centrocytes and centroblasts/large transformed cells), which usually has at least a partially follicular pattern. Progression in cytological grade is common during the natural history of the disease.
There are several variants/subtypes of Follicular lymphoma
1) Diffuse variant of follicular lymphoma. See 9690/3.
2) Duodenal-type follicular lymphoma. See 9695/3.
3) Large B-cell lymphoma with IRF4 rearrangement. See 9698/3.
4) Pediatric-type follicular lymphoma. See 9690/3.
5) Testicular follicular lymphoma. See 9698/3.
There are several variants/subtypes of Follicular lymphoma
1) Diffuse variant of follicular lymphoma. See 9690/3.
2) Duodenal-type follicular lymphoma. See 9695/3.
3) Large B-cell lymphoma with IRF4 rearrangement. See 9698/3.
4) Pediatric-type follicular lymphoma. See 9690/3.
5) Testicular follicular lymphoma. See 9698/3.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Immunophenotyping
BCL2+ (expression/positive)
BCL6+ (expression/positive)
CD5- (no expression/negative)
CD10+ (expression/positive)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)
CD23+ (expression/positive)
CD43- (no expression/negative)
CD79a+ (expression/positive)
GCET1+ (expression/positive)
GCET2 (HGAL) positive
IgD- (no expression/negative)
IgM+ (expression/positive)
LMO2+ (expression/positive)
PAX5+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Radiation therapy
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
202.0 Nodular lymphoma
Corresponding ICD-10 Codes
C82.1 Non-Hodgkin lymphoma mixed small cleaved and large cell, follicular
Corresponding ICD-10-CM Codes (U.S. only)
C82.1 Follicular lymphoma grade II (effective October 01, 2015)
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (or no known reason)
Lymphadenopathy (abdominal and thoracic)
Skin rash or itchy skin
Splenomegaly
Weight loss (for no known reason)
Diagnostic Exams
Blood chemistry studies
Cytogenetics studies
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
Progression and Transformation
25-35% of patients transform to DLBCL
Epidemiology and Mortality
Age: 60 years median age (rare occurrence under 20 years of age)
Incidence: Follicular lymphomas account for 20% of all lymhpomas
Sex: slight female predominance
Survival: 8-15 years median survival, closely related to the extent of the disease at diagnosis
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: 266-281
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 266-281
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
