Follicular lymphoma, grade 1

Name
Follicular lymphoma, grade 1
ICD-O-2 Morphology
9695/3
Effective 1992 - 2000
ICD-O-3 Morphology
9695/3
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

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
Diffuse, follicular lymphoma, grade 1
Follicular lymphoma, diffuse, grade 1
Follicular lymphoma, small cleaved cell
Grade 1, follicular lymphoma, diffuse
Malignant lymphoma, lymphocytic, poorly differentiated, nodular [OBS]
Malignant lymphoma, small cleaved cell, follicular [OBS]
Definition
A neoplasm of follicle centre B cells (centrocytes/cleaved follicle centre cells (FCC) and centroblasts/noncleaved FCC) which has at least a partially follicular pattern
0-5 centroblasts/hpf
Abstractor Notes
FL grade 1 may subsequently be diagnosed with a grade 2 or grade 3 FL. This is not a new primary, it is noting that there are more centroblasts in the BM and the disease is more aggressive.

Code follicular lymphoma, grade 2, when there is a grade such as 1-2. Assign the histology code of the higher grade (9691/3), even though the grade 1 histology code (9695/3) is numerically higher.

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.

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).

There are three variants of FL:
1. Pediatric follicular lymphoma (mostly grade 3) involves cervical lymph nodes, other peripheral lymph nodes, or Waldeyer ring. Other extranodal involvement may occur. Children with FL typically have early stage disease. Pediatric FL lymphoma typically lacks BCL2 protein expression and t(14;18).

2. Primary intestinal follicular lymphoma in the GI tract occurs most commonly in the small intestine, and frequently involves the duodenum.

3. Duodenal follicular lymphoma is predominantly found in the second portion of the duodenum, presenting as multiple polyps. The diagnosis is most often an incidental finding. Most patients have localized disease and prognosis is excellent even without treatment.

Other extranodal follicular lymphomas occur in almost any extranodal site. Patients usually have localized extranodal disease and systemic relapses are rare. Testicular follicular FL are reported with increased frequency in children, but also are reported in adults.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
BCL2 gene rearrangements
Chromosomal translocations T(14;18)
IGH(VD-J) gene rearrangement
Immunoglobin heavy and light chain genes rearranged
Translocation t(14;18)(q32;q21)
Immunophenotyping
BCL2+
BCL6+
CD5-
CD10+
CD19
CD20
CD22
CD43-
CD79a
Heavy and light chains rearranged; t(14;18)(q32:q21)-85% with rearrangement of Bcl-2 gene
Tumor cells Slg+(IgM+/-, IgD, IgG)
Treatments
Chemotherapy
Immunotherapy
Radiation
Stem cell transplant
Transformations from
None
Corresponding ICD-9 Codes
202.0 Nodular lymphoma
Corresponding ICD-10 Codes
C82.0 Follicular non-Hodgkin's lymphoma, Small cleaved cell
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C82.0 Follicular lymphoma, grade I
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Lymphadenopathy (abdominal and thoracic)
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
Bone marrow aspiration and biopsy
Complete blood count (CBC)
Cytogenetics analysis
CT (CAT) scan
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
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