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As of 2016: Code histology to 9085/3 [mixed germ cell tumor]. Combine 9065 and 9085 for analysis purposes.                            

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

This case should be accessioned as two primaries: follicular lymphoma [9690/3] and diffuse large B-cell lymphoma [9680/3] per Rule M10. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (follicular lymphoma) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.

Record the CHOP as the first course of treatment for the follicular lymphoma because this was the only treatment given for the chronic neoplasm (follicular lymphoma) prior to the transformation to the acute neoplasm (DLBCL). Record the chemotherapy and bone marrow transplant as first course treatment for the DLBCL.

As noted above, follicular lymphoma does transform to DLBCL. This "transformation" is actually a new disease. Follicular lymphoma is a disease in which the lymph nodes have a prominent follicular pattern; DLBCL is a disease with diffuse proliferation of large lymphoid cells. While it is true that follicular lymphoma will transform to DLBCL, this transformation indicates it becomes a different entity.

The DLBCL is coded as a second primary for several reasons: to determine the incidence of follicular lymphomas transforming to DLBCL; survival time can be calculated for the diagnosis of the more aggressive DLBCL; death will be attributed to the DLBCL (for mortality statistics) and not the follicular 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.

This is a single primary. The only rules used to determine the number of primaries are the MP/H rules for cases diagnosed 2007 or later. Do not use other information such as Oncotype Dx to determine the number of primaries for a patient. Oncotype is used to determine whether the cancer is likely to recur AND whether the cancer would benefit from chemotherapy.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. Once in the manual, locate the Breast MP rules under one of the three formats (i.e., flowchart, matrix or text).

Start with the MULTIPLE TUMORS module, Rule M4. The rules are intended to be reviewed in consecutive order within the module from Rule M4 to Rule M13. You stop at the first rule that applies to the case you are processing.

The patient has two tumors in the same breast with the same histology. Abstract a single primary for this patient.

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

This case should be accessioned as one primary per Rule M15, 9680/3 [diffuse large B-cell lymphoma]. Per Rule M15 one is to use the Heme DB Multiple Primaries Calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14. The 12/2010 liver diagnosis of NHL, NOS [9591/3] is the same primary per the Multiple Primaries Calculator.

There are no timing rules for lymphoma other than rules M8-M13 which deal with the timing of chronic and acute diagnoses.

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: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

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 is a single primary per Rule M4 which states 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). The histology is coded to 9680/3 per PH11which states to code 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.

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

This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm.

Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3].

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 is a single primary per Rule M2 which indicates to abstract a single primary when there is a single histology. Code the histology to 9590/3 [lymphoma] and the primary site to C629 [testes. Unless your software has edits that prevent coding laterality for lymphomas, code the laterality as bilateral. Up to half of extranodal lymphomas occur in multiple sites, particularly in paired 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.

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.

Histology is sarcomatoid carcinoma [8033/3]. This case was sent to the lung physician experts because of the difficulty in trying to apply the current MP/H rules. Their rationale for the coding decision follows:

"This pathologist has diagnosed a sarcomatoid carcinoma, and then listed all of the subtypes associated with that diagnosis. I would go with the primary diagnosis, sarcomatoid carcinoma. The inclusion of squamous cell differentiation would exclude spindle cell and giant cell as diagnoses, so the pathologist is using them descriptively. We have no basis for picking one of the subtypes and sarcomatoid carcinoma covers all of the diagnoses given."

See the glossary in the Lung Equivalent Terms and Definitions for Sarcomatoid carcinoma: A group of tumors that are non-small cell in type and contain spindle cells and/or giant cells. Depending on the histologic features the tumor may be designated: pleomorphic carcinoma [8022/3]; spindle cell carcinoma [8032/3]; giant cell carcinoma [8031/3], carcinosarcoma [8980/3]; or pulmonary blastoma [8972/3].