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Question: 20120066 Status
Final

References
Source 1:   Heme & Lymph Manual & DB
pgs:  
Notes:  
Source 2:  
pgs:  
Notes:  


Question
Histology/Primary site--Heme & Lymphoid Neoplasms: How are the histology and primary site coded if the patient has monomorphic B-cell post-transplant lymphoproliferative disorder with features of diffuse large B-cell lymphoma involving the intramuscular chest wall and right frontal lobe of the brain? See Discussion.



Discussion
The patient is a 12 year old with a history of Fanconi anemia, status post stem cell transplant. In May, 2012 the patient was diagnosed with monomorphic B-cell PTLD with features of diffuse large B-cell lymphoma.



Answer
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per Rule M14, accession this is a single primary. Per PH27, code the primary site to C809 [unknown} and per PH1, code the histology to 9680/3 [diffuse large B-cell lymphoma].

Per Rule M14, abstract as a single primary when post-transplant lymphoproliferative disorder is diagnosed simultaneously with any B-cell lymphoma, T-cell lymphoma, Hodgkin lymphoma or plasmacytoma/myeloma.

Per PH1, code the histology of the accompanying lymphoma or plasmacytoma/myeloma when the diagnoses of post-transplant lymphoproliferative disorder and any B-cell lymphoma, T-cell lymphoma, Hodgkin lymphoma, or plasmacytoma/myeloma occur simultaneously.

Per PH27, code the primary site to C809 [unknown primary site] because there is no lymph node involvement, but there is involvement of two extranodal sites.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

History
This SINQ question has been updated to the Hematopoietic & Lymphoid Neoplasm Manual & Database published January 2014. The original answer below was written based on the rules in 2012 For cases diagnosed 2012 and later, access the Hematopoietic Database at

http://seer.cancer.gov/tools/heme/.

Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. The 2012 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.

Code the primary site to C809 [unknown primary site] and the histology as diffuse large B-cell lymphoma [9680/3]. The steps used to arrive at this decision are:

Step 1: Enter PTLD in the Heme DB to find the histology. Click on the SEARCH button. Ensure that the term "post-transplant lymphoproliferative disorder" [9971/3] is highlighted on the screen.

Step 2: Scroll down to the ALTERNATIVE NAMES section. Note that “monomorphic B-cell post-transplant lymphoproliferative disorder” is not a synonym for PTLD. Continue to scroll down to the ABSTRACTOR NOTE section which indicates there are two types of PTLD, monomorphic and polymorphic. Monomorphic PTLD is a PTLD that has an accompanying B-cell lymphoma, T-cell lymphoma, Classical Hodgkin lymphoma, or a Plasmacytoma. When there is both a PTLD and a lymphoma or plasmacytoma, the histology is coded to the lymphoma or plasmacytoma – in this case a diffuse large B-cell lymphoma.

Step 3: Enter diffuse large B-cell lymphoma in the Heme DB to find the correct histology. Click on the SEARCH button. Ensure that the term “diffuse large B-cell lymphoma (DLBCL)” [9680/3] is highlighted on the screen.

Step 4: Determine the primary site. No primary site is given per the Heme DB, but the PRIMARY SITE(S) information states Module 7 applies to DLBCL. Click on the 2012 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual scroll to Module 7 to check the Primary Site and Histology Rules. The rules within the Module are intended to be reviewed in consecutive order. Start at Rule PH25 and stop at the first rule that applies to the case you are processing. Stop at Rule PH37. Code the primary site to C809 [unknown primary site] as there is no lymph node involvement, but there is involvement of two extranodal sites.



Last Updated
06/30/2014

Date Finalized
08/29/2012