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This is a single primary, essential thrombocythemia [9962/3] diagnosed in 2007. The 2010 Heme DB and Manual should not have been used to determine the number of primaries in this case. The Heme DB applies only to cases diagnosed 2010 and later.

In order to determine the number of primaries, use the rules in place at the time of the subsequent 2009 diagnosis of primary myelofibrosis. Per the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, a diagnosis of essential thrombocythemia [9962/3] followed by a diagnosis of primary myelofibrosis [9961/3] is a single primary.

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.

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

Per Rule PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.

In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).

Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.

The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.

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 histology as adenocarcinoma, NOS [8140/3].

For cases diagnosed 2007 or later, 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) and go to the Colon Histology rules to determine the histology code for this case. The Module you use depends on the behavior and number of tumors identified in the primary site.

Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY Module Rule H15. The rules are intended to be reviewed in consecutive order from Rule H15 to Rule H24. Stop at the first rule that applies to the case you are processing. Code the histology when only one histologic type is identified. In this case, the only histology present was adenocarcinoma, NOS [8140/3].

Rules H17 through H21 do not apply in this case because there is no malignancy arising in any of the adenomas or polyps scattered throughout the colon.

Code the histology to malignant melanoma, superficial spreading type [8743/3] based on the final diagnosis. For cases diagnosed 2007 or later, the steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. For a melanoma primary, use the Melanoma Histology rules to determine the histology code because there are site specific rules for cutaneous melanomas.

Start at Rule H1. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H10. The rules are intended to be reviewed in consecutive order within the applicable Module. Code the more specific histologic term when the diagnosis is melanoma, NOS [8720] with a single specific type (i.e., superficial spreading) mentioned in the final diagnosis.

The final diagnosis takes precedence over the CAP protocol. The CAP protocol may be used when it provides additional or noncontradictory information, but that does not apply in this case.

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.

Pancreatic (neuro)endocrine neoplasms (PanNETs) are reportable. The correct histology code is 8240/3. The grade is coded as 1 [well differentiated].

This is a reportable case. Code the histology as malignant gastrinoma [8153/3].

Gastrinomas are usually malignant. This one is apparently present in a metastatic site (periportal lymph node) which confirms the malignancy.

For cases diagnosed 2007 or later, accession a single primary.

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 Head and Neck MP rules after determining the histology of each tumor - (8070/3 [squamous cell carcinoma] and 8010/3 [carcinoma, NOS]) because site specific rules have been developed for this primary.

Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. Abstract a single when one tumor is carcinoma, NOS [8010] and another tumor is a specific carcinoma, squamous cell carcinoma [8070] because the ethmoid sinus (site of origin) is not a paired site per the MP/H rules.

We will review the list of paired organs for the next edition of the MP/H Rules.

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

Code the histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)].

For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.

Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.

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.