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This is the same primary, the mantle cell lymphoma.

Differential diagnoses cannot be used to assign histology. For the 2014 diagnosis, the only histology that can be assigned is 9591/3 for non-Hodgkin lymphoma, NOS. (CLL, mantle cell lymphoma and lymphoplasmacytic lymphoma are all NHL's.)

Compare the 1997 diganosis of mantle cell lymphoma with the 2014 diagnosis of non-Hodgkin lymphoma. Start with Rule M1. The first rule that applies is Rule M15, which instructs you to use the multiple primaries calculator. Enter 9673/3 and then 9591/3 and then calculate. The result is same primary.

If at a later time one of the differential diagnoses is confirmed, apply the rules again.

Assign code 5 [T-cell] for anaplastic large cell lymphoma [9714/3] unless the pathologist specifies that the histology is a B-cell disease process. See Grade rule G2, Note 2.

In the Heme DB, there is a default value in the Grade field for histologies that do not have a grade specified. However, this particular histology does not default to code 9. There was an error in the Grade section of the 2010 and 2012 versions of Heme DB that has now been corrected in the latest release.

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

Code the primary site to C421 [bone marrow] and the histology to 9680/3 [diffuse large B-cell lymphoma] per Rule PH26. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is listed under Alternative Names section of the Heme BD for DLBCL [9680/3]. This patient has bone marrow involvement only. The Note for Rule PH26 instructs one to code the primary site to the bone marrow when all physical exams or work-up were negative for lymph node, tissue, or organ involvement OR no other work-up was done.

The histology is not coded 9684/3 [malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS]. This histology code became obsolete in 1/1/2010. Diffuse large B-cell lymphoma, immunoblastic variant is also listed under Alternative Names section of the Heme BD for DLBCL.

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.

Assign C269 for Gastrointestinal tract, NOS. Apply Rule PH24, code to the organ when only an organ is involved. This rule can be used for NOS sites such as GI tract, NOS.

Based on the information provided, this lymphoma is confined to the GI tract -- stomach and small bowel.

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.

Code the histology to 8520/3 [lobular carcinoma].

The 4th Edition of the WHO Classification of Tumors of the Breast now describes five variants of invasive lobular carcinoma. These variants are solid type, alveolar, pleomorphic, tubulolobular, and mixed-type. WHO has not yet proposed new ICD-O codes be assigned to these variants. The upcoming solid tumor (MP/H) revisions will include instructions on coding these variants.

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

Code the histology to 9689/3 [splenic marginal zone lymphoma] per Rule PH29 and Case Reportability Instruction #6 in the Heme Manual. Case Reportability Instruction #6 indicates, "Report the case when there is a (physician's statement) of reportable hematopoietic or lymphoid neoplasm."

The pathology gave an NOS diagnosis, low grade B-cell lymphoma [9591/3]. The physician clinically stated this was a splenic marginal zone lymphoma [9689/3]. Rule PH 29 states to code the specific histology when the diagnosis is one non-specific histology AND one specific histology AND the Heme DB MP Calculator indicates they are the same primary. Per the Multiple Primaries Calculator, these two histologies indicate the same 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.

Code the histology to diffuse large B-cell lymphoma [9680/3] per Rule PH11.

Code the histology to 9680/3 [diffuse large B-cell lymphoma] when DLBCL and any other non-Hodgkin lymphoma (follicular lymphoma, grade 3 in this case) are present in the same anatomic location. DLBCL is coded because it is the more aggressive histology.

NOTE: This answer assumes these two histologies are present simultaneously in the same anatomic location. Per Rule M4, this is a single 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.

Polycythemia Vera (PV) [9950/3] is reportable. The Abstractor Notes section in the Hematopoietic Database for PV indicates there are three phases of PV. The third phase is referred to as the "spent" or "post-polycythemic myelofibrosis phase". This patient appears to be in the third phase of PV. This would not be reported as a new primary if PV has already been reported.

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 single primary, acute myeloid leukemia [9861/3].

MDS diagnosed prior to 1/1/2001 is not a reportable disease process. However, because MDS is currently a reportable disease process, it must be considered when trying to determine whether the AML represents a separate primary.

• If the Heme DB indicates MDS and AML represent different (separate) disease processes, only one primary is reported (i.e., AML) because the 1999 diagnosed MDS is not reportable.

• If the Heme DB indicates MDS and AML represent the same disease process, then no primaries are reported because MDS was not reportable in 1999.

Rule M2 does not apply to this case because more than one histology is mentioned in the scenario. According to the Heme DB, MDS can transform to AML. Rules M8-M13 apply to cases involving transformation. In this case, Rule M10 applies because the patient was diagnosed with a chronic neoplasm (myelodysplastic syndrome) followed greater than 21 days later by an acute neoplasm (AML).

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.