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

Use Rule PH21 to code the primary site to C778 [lymph nodes of multiple regions]. The spleen is not listed under the Primary Site(s) section in the Heme DB for diffuse large B-cell lymphoma. Per Rule PH21 code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. The spleen is a primary site for only a few lymphomas (noted in the Heme DB). Because the spleen filters blood, it is often reactive (splenomegaly) or frankly involved with the lymphoma. That reaction or involvement, however, does not affect the primary site coding. Only the involved nodes are used in coding primary site.

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 per Rule M1. According to our expert pathologist, "if multiple solid tissue tumors are present (sarcomas), then almost always there is one primary and the rest are metastases. There are infrequent occasions of multifocal liposarcoma or osteosarcoma occurring, but the patient would be treated as a patient with metastatic disease."

The steps used to arrive at this answer are:

Open the Multiple Primary and Histology Coding Rules manual. For a soft tissue primary, use one of the three formats (i.e., flowchart, matrix or text) under the Other Sites MP rules to determine the number of primaries because soft tissue primaries do not have site specific rules.

Go to the UNKNOWN IF SINGLE OR MULTIPLE TUMORS module, Rule M1.

Rule M1 states, "It is not possible to determine if there is a single tumor or multiple tumors, opt for a single tumor and abstract a single primary." Given the information from the expert pathologist, this case should be reported as a single primary applying this rule.

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.

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.

Per Rule M2, this is a single primary because there is a single histology mentioned. The histology is coded to 9699/3 [MALT lymphoma]. Code the primary site to C509 [breast] per Rule PH24 which states to code the primary site to the organ when lymphoma is present only in an organ.

Unless your software has edits that prevent coding laterality for lymphomas, code the laterality as bilateral. Up to half of extranodal, extragastric MALT lymphomas occur in multiple sites, particularly in paired sites (breast is an example).

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.

Code as a single primary. The histology is carcinoid.

Our expert pathology consultant replied as follows: "This patient clearly has a condition driving the proliferation of neuroendocrine cells. Possibilities include hypergastrinemia from a gastrinoma or from response of antral gastrin cells due to achlorhydria from long standing chronic atrophic gastritis, or multiple endocrine neoplasia (MEN1) syndrome (genetically driven). How should these cases be coded given we do not have a way to code the inciting situation. (I suspect the gastroenterologist knows what it is, but we haven't obtained that information.) We do not have an ICD-O-3 code for the underlying condition, MEN1 or hypergastrinemia. Therefore, the only choice is to code the resulting tumor, carcinoid [8240/3]."

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.

The expert pathologist recommends coding histology to 8980/3 [Carcinosarcoma] for this combination histology.

Expert consultation: The designation "carcinosarcoma" is given when the pathology shows differentiation in both the sarcomatous (rhabdomyoblastic) and carcinomatous (adenoca) elements. This is emphasized in the path for this case with the term "biphasic." The term "heterologous" mean that the sarcomatous component is of a type not normal to lung. Rhabdomyoblastic means skeletal muscle differentiation. Because skeletal muscle is not normally found in lung it is heterologous. If it were smooth muscle, it would be homologous because smooth muscle is found in lung (as a part of the bronchi).

The date of diagnosis is 2/25/10 and diagnostic confirmation is coded to 8 [clinical diagnosis only].

As the cytogenetics state, you need clinicopathologic correlation to get confirm a reportable diagnosis. There is no reportable diagnosis from the bone marrow biopsy. The cytogenetics were done (the pathologic part) and then the physician confirmed refractory cytopenia with multilineage dysplasia [9985/3] (the clinical part). The diagnostic process and the determination of a reportable diagnosis were completed when the clinician made the statement that this is refractory cytopenia with multilineage dysplasia.

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.

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (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.