Follicular lymphoma, grade 2

Name
Follicular lymphoma, grade 2
ICD-O-2 Morphology
9691/3
Effective 1992 - 2000
ICD-O-3 Morphology
9691/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
Diffuse, follicular lymphoma, grade 2
Follicular lymphoma, diffuse, grade 2
Grade 2, follicular lymphoma, diffuse
Malignant lymphoma, mixed cell type, follicular [OBS]
Malignant lymphoma, mixed cell type, nodular [OBS]
Malignant lymphoma, mixed lymphocytic-histiocytic, nodular [OBS]
Malignant lymphoma, mixed small cleaved/large 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
6-15 centroblasts/hpf
Abstractor Notes
Follicular lymphoma (FL) predominantly involves lymph nodes, but also spleen, bone marrow (BM), peripheral blood (PB) and Waldeyer ring. Less commonly, FL may involve non-hematopoietic extranodal sites such as gastrointestinal tract or soft issue; this usually occurs in a setting of widespread nodal disease. FL may occasionally be primary in extranodal sites including skin, GI tract, particularly the duodenum, occular adnexa, breast, and testis. 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. FL has been 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 progression 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 (See PH rules).

Patients with FL grade 2 may subsequently be diagnosed with FL grade 3. This is not a new primary, it indicates that there are more centroblasts in the bone marrow and the disease is more aggressive.

There are three variants of FL: pediatric FL, primary intestinal FL, and other extranodal follicular lymphomas.

Pediatric FL 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 typically lacks BCL2 protein expression and t(14;15). Most are grade 3.

Primary intestinal FL in the GI tract occurs most commonly in the small intestine, and frequently involves the duodenum. Duodenal FL 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 FL occur in almost any extranodal site. Patients usually have localized extranodal disease and systemic relapses are rare. Testicular FL are reported with increased frequency in children, but also are reported in adults.

Code follicular lymphoma, grade 2, when there is a grade such as 1-2. Take the higher grade, even though the grade 1 histology code is higher.
Definitive Diagnostic Methods
FISH
Histologic confirmation (with or without)
Immunophenotype
Genetics Data
BCL2 gene rearrangements
Chromosomal translocations T(11;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 Sig+(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
C82.1 Mixed small cleaved and large cell follicular
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C82.1 Follicular lymphoma grade II
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None