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Produced: 11/26/2024 5:28 AM
Question 20110039
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Discussion:
Follicular lymphoma was diagnosed 7/2004, grade 2 per biopsy of the bilateral breasts. Bone marrow biopsy was positive for lymphoma involving 10% of bone marrow. Imaging showed extensive lymphadenopathy mainly in abdomen/pelvis with an 8 cm mass in the right pelvis. Smaller lymph nodes were noted in the left periaortic region and also some small precarinal lymph nodes. This was a stage IVA lymphoma. The patient had six cycles of CHOP/R with an excellent response. Per the clinician's notes on 12/2005, there was no evidence of recurrence or no sign of active disease. The plan was to follow up with the patient in 6 months.
08/22/06 imaging showed new disease in the bilateral chest wall. 8/2006 bilateral breast nodule biopsies, are positive for grade 1-2 follicular lymphoma. The patient was treated with Rituxan. Per clinician's 3/2007 note, no active disease is noted. Patient was regularly followed with no evidence of disease until 10/2010. At that time, the patient had a left arm nodule biopsy which was positive for diffuse large B cell lymphoma (40%) CD positive and grade 3a follicular lymphoma (60%). RICE was recommended due to "transformation" per oncologist.
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M10, this case should be accessioned as two primaries when a neoplasm is originally diagnosed as a chronic neoplasm (is follicular lymphoma (FL), grade 2) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.
Code the histology for the first primary to 9691/3 [follicular lymphoma (FL), grade 2] and the primary site to bilateral C509 [breast, NOS]. FL can start as an extranodal disease; breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes.
Per Rule M4, abstract the 2010 disease process as a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Per Rule PH11 the primary site is coded to C779 [lymph nodes, NOS] and the histology is coded to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Rule PH11 states one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.
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 2010 (or 2012)
For cases diagnosed 2010-2011, access the Hematopoietic Database at http://seer.cancer.gov/tools/heme/. Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. For 2010-2011 diagnoses, click on the "use the 2010 database" label in the upper right corner of the screen. The 2010 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.
This case should be accessioned as two primaries. The first primary is follicular lymphoma (FL), grade 2 [9691/3] with a primary site of breast (bilateral) [C509]. FL can start as an extranodal disease and breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and a primary site of lymph nodes, NOS [C779].
The steps used to arrive at this decision are:
Enter in to the Heme DB to search for the histology. Click on the SEARCH button. Scroll down and highlight the term "follicular lymphoma, grade 2" in the RESULTS FOR ALL TERMS section of the screen to identify the histology 9691/3.
Scroll down to the TRANSFORMATIONS section information. Follicular lymphoma, grade 2 (a chronic disease process) transforms to DLBCL (an acute disease process). When a cell type is listed in the transformation section for a given malignancy, the disease process in the transformation section is considered the acute process and the disease listed at the top of the screen display is considered the chronic process. In this case, the chronic and acute disease processes were diagnosed at different times.
Determine the number of primaries. Click on the 2010 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, locate one of the three formats (i.e., flowchart, matrix or text) to check the Multiple Primary Rules. The rules are intended to be reviewed in consecutive order from Rule M1 to Rule M13. Stop at the first rule that applies to the case you are processing.
. Abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.
Determine the histology for . Enter DLBCL in to the Heme DB to search for the histology. Click on the SEARCH button. "Diffuse large B-cell lymphoma (DLBCL)" [9680/3] will be highlighted on the screen under the RESULTS FOR ALL TERMS area.
Determine the provisional histology for the 2010 diagnosis reported as DLBCL that presents as DLBCL (40%) and FL, grade 3a (60%). Click on 2010 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, locate one of the three formats (i.e., flowchart, matrix or text) to check the Primary Site Histology Rules. The rules are intended to be reviewed in consecutive order within the module.
Go to MODULE 6: Coding Primary Site and Histology for Specified Lymphoma PH16-PH24. Stop at the first rule that applies to the case you are processing. . According to that rule, when there is DLBCL and FL, code histology to DLBCL [9680/3].
Determine the primary site of the new 2010 primary. Go to MODULE 7. This module is specifically written to help code primary site for lymphomas. Start at Rule PH25. . Code the primary site to C779 [lymph nodes, NOS] when lymph node(s) are involved but no primary site/particular lymph node region is identified. It is not clear that the primary site is the arm.
Determine the primary site of the 2004 primary. Go to MODULE 7: Primary Site Rules for Lymphomas Only 9590/3-9729/3 PH25-PH37. Stop at the first rule that applies to the case you are processing. . According to this rule, code the primary site to the organ when lymphoma is present in an organ and that organ's regional lymph nodes. The primary site for the first tumor is C509 [breasts], bilateral.
