Search Database ICD-O-3 Code Lists

Name

Follicular lymphoma, grade 3

ICD-O-1 Morphology

9615/3: Malignant lymphoma, follicular center cell, NOS
9633/3: Malignant lymphoma, follicular center cell, non-cleaved NOS
9693/3: Malignant lymphoma, lymphocytic, well differentiated, nodular
9697/3: Malignant lymphoma, centroblastic type, follicular
9698/3: Follicular lymphoma, grade 3
Effective 1978 - 1991

ICD-O-2 Morphology

9693/3: Malignant lymphoma, lymphocytic, well differentiated, nodular
9697/3: Malignant lymphoma, centroblastic type, follicular
9698/3: Follicular lymphoma, grade 3
Effective 1992 - 2000

ICD-O-3 Morphology

9698/3: Follicular lymphoma, grade 3
Effective 2001 and later

Reportable

for cases diagnosed 1978 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).

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

Alternate Names

Diffuse, follicular lymphoma, grade 3
Follicular lymphoma, diffuse, grade 3
Follicular lymphoma, grade 3A
Follicular lymphoma, grade 3B
Follicular lymphoma, high grade
Large B-cell lymphoma with IRF4 rearrangement
Malignant lymphoma, centroblastic, follicular
Malignant lymphoma, histiocytic, nodular [OBS]
Malignant lymphoma, large cell, follicular, NOS
Malignant lymphoma, large cell, noncleaved, follicular [OBS]
Malignant lymphoma, large cleaved cell, follicular [OBS]
Malignant lymphoma, lymphocytic, well differentiated, nodular [OBS]
Malignant lymphoma, noncleaved cell, follicular, NOS [OBS]

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 below.
4) Pediatric-type follicular lymphoma. See 9695/3.
5) Testicular follicular lymphoma. See below.

Large B-cell lymphoma (LBCL) with IRF4 rearrangement is an uncommon subtype of LBCL that can be entirely diffuse, follicular and diffuse, or entirely follicular. It is characterized by strong expression of IRF4/MUM1, usually with IFR4 rearrangement. It occurs primarily in children and young adults, with predominantly Waldeyer ring or head and neck lymph node involvement.

Testicular follicular lymphoma is a distinctive variant of FL. They are reported with higher frequency in children, but area also seen rarely in adults. They differ biologically from fl in that they lack evidence of the BCL2 translocation. Cytologically they are of high cytological grade, usually grade 3A, but have a good prognosis, even without additional therapy beyond surgical resection.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

CREBBP mutation
EPHA7 mutation
KMT2D (MLL2) mutation
MEF2B mutation
MYC gain
TNFAIP3 (also called A20) deletion, mutation
TNFRSF14 deletion, mutation

Immunophenotyping

BCL2+ (expression/positive)
BCL6+ (expression/positive)
CD5- (no expression/negative)
CD10+ (expression/positive)
CD19+ (expression/positive)
CD22+ (expression/positive)
CD23+ (expression/positive)
CD43- (no expression/negative)
CD79a+ (expression/positive)
GCET1+ (expression/positive)
GCET2 (HGAL)+ (expression/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

C82.2 Non-Hodgkin lymphoma large cell, follicular

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

C82.2 Follicular lymphoma grade III, unspecified (effective October 01, 2015)
C82.3 Follicular lymphoma grade IIIa (effective October 01, 2015)
C82.4 Follicular lymphoma grade IIIb (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Lymphadenopathy (abdominal and thoracic)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes in the neck, underarm, groin or stomach
Skin rash or itchy skin
Splenomegaly
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
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

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

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary