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

There are two reportable primaries for this case -- follicular lymphoma in 2010 and DLBCL in 2012.

First determine the histologies needed to to determine the number of primaries to report. We determined the histologies are follicular lymphoma, grade 1 for 2010 and DLBCL for 2012 as follows:

• Rule M4, a mix of DBLCL and follicular lymphoma, grade 3 is 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). Additionally, the instruction per Rule PH11 indicates one is to code the histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma (i.e., follicular lymphoma, grade 3) are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

Per the Hematopoietic database, follicular lymphoma (all types are chronic) transforms to DLBCL (acute). Per Rule M 10 instructions, "Abstract as multiple primaries when a neoplasm is as a neoplasm there is a of an neoplasm after the chronic diagnosis." Therefore, abstract the DLBCL as a second primary.

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.

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

Refer to the Hematopoietic Database and Manual to determine the primary site.

Leukemias are coded to C421 [bone marrow]. The ONLY neoplasm that is coded to C420 [blood] is Waldenstrom's macroglobulinemia [9761/3].

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.

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

This case should be accessioned as a multiple primary: chronic myelogenous leukemia t(9;22) (q34;q11) [9863/3] diagnosed in 1993 and acute myeloid leukemia [9861/3] diagnosed in 2011 per Rule M15.

Use the diagnosis date to determine the appropriate manual and rules to follow to determine the histologies for this case. To determine the histology of the 1993 diagnosis, use the ICD-O-2. The Heme Manual & DB will be used to determine the number of primaries and the histology of the 2011 diagnosis of AML.

Rules M8-M13 in the Heme Manual cannot be applied to this case because no transformation occurred. CML does not transform to another neoplasm.

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.

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

This case was originally diagnosed in 2009, prior to the development of Hematopoietic Database. Therefore it is necessary to use the ICD-O-3 to code histology to 9684/3 [diffuse large B-cell lymphoma, immunoblastic]. Use the original histology diagnosed for the merged record because DLBCL, immunoblastic, and plasmablastic lymphoma are the same primary. Do not change the histology to code 9735/3 [plasmablastic lymphoma].

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.

Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

This case is not reportable. Rosai-Dorfman disease is not listed in the ICD-O-3. To be reportable, a neoplasm must be listed in the ICD-O-3 and originate in a reportable brain/CNS site.

Assign code 8502/3 [secretory carcinoma of breast].

Acinar carcinoma [8550/3] describes a very typical type of salivary gland tumor only. This histology code does not adequately capture the histology in this case which describes a secretory carcinoma that is similar to mammary cancer. Both of these elements are reflected in the histology code 8502/3 [secretory carcinoma of breast].

Accession two primaries per Rule M17, neuroendocrine carcinoma [8246/3] of the rectosigmoid junction [C199], and adenocarcinoma in a villous adenoma [8261/3] of the rectosigmoid junction [C199]. There are two tumors with ICD-O-3 histology codes that differ at the third number.

Rule M13 does not apply to neuroendocrine carcinoma. Rule M16 does not apply to this case because there are two specific histologies.

When the latest WHO classification for heme neoplasms was written in 2008, there was a lot of controversy about whether or not the FL grading system was useful or not. A number of papers have been written stating that grades 1 and 2 do not have a statistically different survival or transformation rate. Given that the controversy had not been settled by those in the clinical world, the WHO recommended analyzing grades 1 and 2 together. They did not, however, remove either grade 1 or 2 from their classification. When the WHO intend to change their classification (have both grades classified under one histology number), they omit one code from their book (make it obsolete) and change the definition for the other code. The 2008 WHO book did not make either ICD-O-3 code obsolete. Therefore, we continue to collect the cases as designated by the pathologist. If the controversy is settled before the next WHO classification, you may see changes in the codes.

Additionally, since the 2008 WHO book was written, there have been some clinical papers challenging the designation of grade 3. They contend that grade 3 can be mistaken for low-grade.

The grades for follicular lymphoma are based on the number of centroblasts per high powered field (HPF). The number of centroblasts for grade 1 is 0-5; for grade 2 is 6-15, for grade 3a and 3b is >15 centroblasts. 3a has centrocytes and 3b has no centrocytes.

Per Rule M4, this is a single primary. According to Rule M4, one is to abstract 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, code the histology to 9680/3 [diffuse large B-cell lymphoma] and the primary site to C422 [spleen]. According to PH11, 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.