ALK-positive large B-cell lymphoma

Name
ALK-positive large B-cell lymphoma
ICD-O-3 Morphology
9737/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 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
ALK-positive LBCL
ALK-positive plasmablastic B-cell lymphoma
Large B-cell lymphoma expressing the ALK kinase and lacking the t(2;5) translocation
Definition
This is a neoplasm of ALK-positive monomorphic large immunoblast-like B cells, sometimes with plasmablastic differentiation. This lymphoma is very rare (<1% of DLBCL) with less than 40 cases reported so far.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9684/3.) This is a very rare neoplasm. The tumor involves mainly lymph nodes or presents as a mediastinal mass. Involvement of extranodal sites have also been reported (nasopharynx, tongue, stomach, bone and soft tissues). Most patients present with Stage III/IV. Because these tumors are negative for CD20 antigen, they are insensitive to rituximab.
Definitive Diagnostic Methods
Genetics testing
Histologic confirmation
Immunophenotype
Karyotyping
Genetics Data
CLTC-ALK fusion gene
Immunoglobulin genes are clonally rearranged
t(2;17)(p23;q23)
Immunophenotyping
ALK-positive
CD3-
CD20-
CD30-
CD45-
CD70a-
CD138+
EMA
VS38+
Treatments
Chemotherapy
Transformations to
No Transformations
Transformations from
No Transformations
Corresponding ICD-9 Codes
200.6 Anaplastic 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)
C84.6 Anaplastic large cell lymphoma, ALK positive
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None