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

There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics."

Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.]

The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML.

It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML.

The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the 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.

This case should be accessioned as two primaries per Rule M13. Code the histology for the 7/31/08 diagnosis to 9680/3 [diffuse large B-cell lymphoma] and the code the histology for the 10/14/2010 diagnosis to 9698/3 [follicular lymphoma, grade 3A of 3]. Rule M13 applies to this case because the neoplasm was originally diagnosed in the blast or acute phase (DLBCL) and reverted to a less aggressive or chronic phase (follicular lymphoma) after treatment.

Per the "Transformations to" section in the Heme DB for follicular lymphoma, grade 3 transforms to diffuse large B-cell lymphoma [9680/3]. This means that the follicular lymphoma is the chronic neoplasm and that DLBCL is the acute neoplasm. In this case, the chronic neoplasm was diagnosed after the acute neoplasm was diagnosed and treated (with chemotherapy).

Do not Stop at Rule M4 because diffuse large B-cell lymphoma and follicular lymphoma (both NHL's) were not present in the same node(s) AT THE SAME TIME.

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 the primary site to main bronchus [C340].

Primary small cell carcinoma in the thymus/mediastinum is rare. A bronchial lesion with extension into the mediastinum is much more likely. In a case like this, it is difficult to be sure exactly where the tumor arose, however, it is recommended the default site be the main bronchus when there is no information to the contrary.

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

Ambiguous terms are not used to code a specific histology. This includes ambiguous terminology used as a result of immunophenotyping or genetic studies. However, a definitive clinical diagnosis can be used to code a more specific histology.

In this example, the histology is coded to non-Hodgkin lymphoma, NOS [9591/3] because the pathology final diagnosis was non-Hodgkin lymphoma, NOS even though it was followed by further genetic and immunohistochemistry studies that were "compatible with" (ambiguous terminology) follicular lymphoma.

However, if there was a subsequent non-ambiguous clinical diagnosis, the histology would be coded to the more specific diagnosis. For example, if the pathology final diagnosis was non-Hodgkin lymphoma, NOS, and there was a subsequent clinical diagnosis of follicular lymphoma or the patient was treated for follicular lymphoma, then the histology should be coded to 9690/3 [follicular lymphoma, NOS]. Document either of these in a text field to support the histology code chosen. Follicular lymphoma is a specific type of non-Hodgkin lymphoma. If you do have a confirmed diagnosis of follicular lymphoma, code that specific cell type per rule PH29.

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 9591/3 [B-cell lymphoma, NOS] per Rule PH28 which states that one is to code the histology when the diagnosis is

• specific histology

• specific histologies
• Heme DB Multiple Primaries Calculator documents the specific histologies and NOS are the

There is only one non-specific histology code mentioned, low grade B-cell lymphoma. This term is synonymous with B-cell lymphoma, NOS.

Per the Multiple Primaries Calculator, when comparing the histology 9591/3 [B-cell lymphoma, NOS] and 9671/3 [lymphoplasmacytic lymphoma], it is the same primary. When comparing the histology 9591/3 [B-cell lymphoma, NOS] and 9699/3 [marginal zone lymphoma], it is 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.

Code the primary site to C179 [small intestine, NOS] for multiple invasive tumors of the small intestine accessioned as a single primary.

The steps used to arrive at this decision are:

Step 1: Go to the Primary Site subsection located in Section IV of the 2012 SEER Manual titled "Description of This Neoplasm."

Step 2: Apply instruction 5. "Code the last digit of the primary site code to '9' for single primaries, when multiple tumors arise in different subsites of the same anatomic site and the point of origin cannot be determined." Code the primary site to C179 [small intestine, NOS].

When multiple tumors arising in different subsites are accessioned as a single primary, the primary site is coded to the NOS code, in this case small intestine, NOS [C179]. The level of invasion does not determine the primary site, unless one or more of the tumors is in situ and another is invasive.

For cases diagnosed 2007 or later, code the histology as lobular carcinoma, in situ [8520/2].

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Breast Histo rules because site specific rules exist for this primary.

Start at the SINGLE TUMOR: IN SITU CARCINOMA ONLY module, Rule H1. The rules are intended to be reviewed in consecutive order. Stop at the first rule that applies to the case you are processing. Code the histology to lobular carcinoma in situ [8520/2] because this is the only histologic type identified.

Pleomorphic lobular carcinoma is a variant of lobular carcinoma which does not have an ICD-O-3 code. It is still a lobular carcinoma. The identification of the variants of lobular carcinoma was a relatively recent discovery and the information was not available when the 2007 MP/H Rules were written. All of the lobular variants will be included in the next revision of the MP/H Rules.

For cases diagnosed 2021 or later, VIN II-III is reportable. Similarly, AIN II-III, VAIN II-III, etc. are reportable. For cases diagnosed 2021 or later, the primary resource for reportability is ICD-O-3.2. Squamous intraepithelial neoplasia, grade II is listed in ICD-O-3.2 as 8077/2 making it reportable. This applies to the various sites of intraepithelial neoplasia grade II including anus, vulva, and vagina.

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

Per Rule M14, accession this is a single primary. Per PH27, code the primary site to C809 [unknown} and per PH1, code the histology to 9680/3 [diffuse large B-cell lymphoma].

Per Rule M14, abstract as a single primary when post-transplant lymphoproliferative disorder is diagnosed simultaneously with any B-cell lymphoma, T-cell lymphoma, Hodgkin lymphoma or plasmacytoma/myeloma.

Per PH1, code the histology of the accompanying lymphoma or plasmacytoma/myeloma when the diagnoses of post-transplant lymphoproliferative disorder and any B-cell lymphoma, T-cell lymphoma, Hodgkin lymphoma, or plasmacytoma/myeloma occur simultaneously.

Per PH27, code the primary site to C809 [unknown primary site] because there is no lymph node involvement, but there is involvement of two extranodal 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 2021 and later

VIN II-III is reportable based on ICD-O-3.2 which lists squamous intraepithelial neoplasia, grade II as 8077/2 making it reportable. Also see SINQ 20120094.