Diffuse large B-cell lymphoma (DLBCL), NOS

Name
Diffuse large B-cell lymphoma (DLBCL), NOS
ICD-O-1 Morphology
9640/3
Effective 1978 - 1991
ICD-O-2 Morphology
9680/3
Effective 1992 - 2000
ICD-O-3 Morphology
9680/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
Module 6: PH11, PH13
Alternate Names
Angioendotheliomatosis [OBS] see 9712/3
Angiotropic lymphoma [OBS] see 9712/3
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
Burkitt-like lymphoma
Diffuse large B-cell lymphoma, NOS centroblastic variant
Diffuse large B-cell lymphoma, NOS immunoblastic variant
Diffuse large B-cell lymphoma/Intravascular large B-cell lymphoma
DLBCL
DLBCL/BL (Gray zone lymphoma)
DLBCL associated with chronic inflammation
Double hit lymphoma
EBV Positive DLBCL of the elderly
EBV-associated B-cell lymphoproliferative disorder of the elderly
Germinal centre B-cell-like GCB
Histiocyte-rich large B-cell lymphoma [OBS] see 9688/3
Hodgkin-like anaplastic large cell lymphoma
Intravascular B-cell lymphoma [OBS] see 9712/3
Intravascular large B-cell lymphoma [OBS] see 9712/3
Lymphomatoid granulomatosis grade 3
Malignant lymphoma, centroblastic, diffuse
Malignant lymphoma, centroblastic, NOS
Malignant lymphoma, histiocytic, diffuse
Malignant lymphoma, histiocytic, NOS [OBS]
Malignant lymphoma, large B-cell, diffuse, centroblastic, NOS
Malignant lymphoma, large cell, cleaved, NOS [OBS]
Malignant lymphoma, large cell, cleaved and non cleaved [OBS]
Malignant lymphoma, large cell, diffuse, NOS [OBS]
Malignant lymphoma, large cell, non cleaved, NOS
Malignant lymphoma, large cell, noncleaved, diffuse
Malignant lymphoma, large cell, NOS
Malignant lymphoma, large cleaved cell, NOS [OBS]
Malignant lymphoma, noncleaved, diffuse, NOS [OBS]
Malignant lymphoma, noncleaved, NOS
Non-germinal centre B-cell-like; non-GCB
PCLBCL leg type
PCNSL
PIOL
Primary CNS lymphoma
Primary cutaneous DLBCL, leg type
Primary DLBCL of the CNS
Richter syndrome
Splenic EBV-associated B-cell lymphoproliferative disorder
T-cell rich B-cell lymphoma [OBS] see 9688/3
T-cell rich/histiocyte rich large B-cell lymphoma [OBS] see 9688/3
T-cell rich large B-cell lymphoma [OBS] see 9688/3
Triple hit lymphoma
Definition
Lymphoma with diffuse proliferation of large neoplastic B lymphoid cells with nuclear size exceeding macrophage nuclei, more than twice size of normal lymphocytes. Normal architecture of node or extranodal tissue replaced in diffuse pattern. Morphologic variants: centroblastic, immunoblastic, and anaplastic.
Abstractor Notes
Diffuse large B-cell lymphoma has several variants, all of which are coded to 9680/3.

1. DLBCL of the CNS: Approximately 60% are supratentorial. 20-40% have multiple lesions. Approximately 20% have intraocular lesions. Many of the intraocular lesions are contralateral, and the patient may develop parenchymal lesions. Sporadic systemic recurrences may affect any organ but relatively frequently the testis and breast.

2. DLBCL associated with chronic inflammation: common sites are pleural cavity, bone (especially femur) joint, and periarticular soft tissue. Tumor mass >10 cm in most case of pleural cavity disease (pyothorax-associated lymphoma or PAL).

3. EBV positive DLBCL of the elderly: Occurs in patients who are greater than 50 years old and have no known immunodeficiency or history of lymphoma. 70% have extranodal involvement, most commonly skin, lung, tonsil, and stomach with or without LN involvement. The remaining 30% present with LN involvement only.

4. Primary cutaneous DLBCL, leg type: these lymphomas usually affect the lower leg but 10-15% arise in other sites. Patient presents with red or bluish-red tumors on one or both of the lower legs. Frequently disseminates to other sites.

5. B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt lymphoma: more than half present with widespread extranodal disease. Bone marrow and peripheral blood may be involved. Patients present with lymphadenopathy or mass lesions in extranodal sites.

Code the primary site to skin when there is skin infiltration with large B-cell lymphoma; B-cell lymphoma, large cell type or large cell lymphoma and the lymphoma is limited to skin and the regional lymph nodes. If there is involvement of lymph node(s) that are not regional for the skin site involved, or involvement of bone marrow or organ(s), do not code skin as the primary site. In this situation, skin is probably a metastatic site.

Richter syndrome is a term that is used to describe the transformation from either CLL/SLL or hairy cell leukemia to DLBCL.
Definitive Diagnostic Methods
Cytology (for primary CNS lymphoma only)
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
8q24/MYC translocations
Immunoglobulin genes clonally rearranged and hypermutated
Mutation RhoH/TTFn
Translocations involving c-MYC, BCL6, and IgH genes
Immunophenotyping
BCL2+
BCL6+/-
CD10+
CD15+
CD20+
CD22+
CD30+
CD79a+
EBNA-2+
Fox-P1+
IRF4/MUM1+
LMP-1+
Surface 4 or cytoplasmic immunoglobulin (Ig) is absent
Treatments
Chemotherapy
Hormone
Radiation
Stem cell transplant
Transformations to
None
Corresponding ICD-9 Codes
200.7 Large cell lymphoma
Corresponding ICD-10 Codes
C83.3 Diffuse non-Hodgkins lymphoma, large cell (diffuse)
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C83.3 Diffuse large B-cell lymphoma
Signs and Symptoms
Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Rapidly enlarging mass at single or multiple nodal or extranodal sites
Skin rash or itchy skin
Weight loss (for no known reason)
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: 70 years median age (occur in children and adults)
Incidence: 25-30% of adult NHL's (western countries)
Sex: slight male predominance
Survival: 5 year survival ~62% without bone marrow involvement; 5 year survival 10% with bone marrow involvement