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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 M15. Code the histology for the first primary to 9698/3 [nodular histiocytic lymphoma. Per the Alternate Names section in the Heme DB, this histology is synonymous with follicular lymphoma, grade 3. Code the histology for the second primary to 9823/3 [B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma].

Nodular histiocytic lymphoma does not transform into CLL/SLL (Transformations to), nor does CLL/SLL transform to nodular histiocytic lymphoma (Transformations from). Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries in this case because none of the rules from 1-14 apply. Per the calculator, the CLL/SLL is a new 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 primary site to C77.8 [multiple lymph node regions, NOS].

Per Rule PH6, code the primary site to the involved lymph node region(s) when there is no bone marrow involvement or when it is unknown whether the bone marrow is involved. To determine the more specific lymph node subsite to code, use Rule PH21. It indicates one is to code the primary site to C778 [multiple lymph node regions, NOS] when multiple lymph node regions, as defined by the ICD-O-3 (see Table C1: Lymph Node/Lymph Node Chain Reference Table in Appendix C), are involved and it is not possible to identify the lymph node region where the lymphoma originated.

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 a single primary. Code the histology to 9680/3 [diffuse large B-cell lymphoma] and diagnosis date to 2004. Per Rule M2, abstract as a single primary when there is a single histology.

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. The Heme DB indicates the primary site for systemic mastocytosis is always coded to C421 [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.

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.

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

Apply rule PH22 to code the primary site to C779 [lymph nodes, NOS].

While the pathology does not indicate that this particular case represents a B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt variant, in the Abstractor Notes section of the Heme DB for diffuse large B-cell lymphoma it does indicate that its presentation, "may have lesions in ileocecal region or jaws. Bone marrow and peripheral blood may be involved. Patients present with lymphadenopathy or mass lesions in extranodal site." This patient does have involvement of two extranodal sites and involvement of regional lymph nodes for only one of those sites.

PH22 indicates one is to code the primary site to C77.9 [lymph nodes, NOS] when lymphoma is present in multiple organs and lymph nodes that are not regional for that organ and the origin cannot be determined even after consulting the physician. There are two extranodal sites of involvement and only one chain of lymph nodes is regional to one of those sites so this rule applies.

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.

Accession a single primary per Rule M2 which indicates you are to abstract a single primary when there is a single histology. Code the histology to 9983/3 [refractory anemia with excess blasts type 2 (RAEB-2)].

Per Appendix F, myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS, if not specified to be myelofibrosis, therefore, is not reportable.

Per PH29, code the specific histology when the diagnosis is one non-specific (NOS) histology (MDS) and one specific histology (RAEB-2) AND the Multiple Primary Calculator confirms the specific histology and NOS histology are the same primary (which it does).

Myelodysplastic syndrome, NOS is a generic disease description. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. The more specific myelodysplastic syndromes are: refractory anemia; refractory neutropenia; refractory thrombocytopenia; refractory anemia with ring sideroblasts; refractory cytopenia with multilineage dysplasia; refractory anemia with excess blasts; and refractory cytopenia of childhood. If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis.

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].

After searching the Heme DB for the term , no B-cell leukemia/lymphoma NOS code is found. However, the diagnosis of B-cell lymphoblastic leukemia/lymphoma is found. This case scenario does not specify that this is a lymphoblastic leukemia/lymphoma; therefore, the histology code 9811/3 [B-cell lymphoblastic leukemia/lymphoma, NOS] cannot be applied.

A subsequent search of the Heme DB for the term returns "Non-Hodgkin lymphoma, NOS" [9591/3]. Under the Alternative Names section of the Heme DB, B-cell lymphoma, NOS, is a synonym for Non-Hodgkin lymphoma, NOS. Therefore, the B-cell lymphoma NOS code [9591/3] is the most appropriate histology code to use for this case.

This will be added to the next revision of the Heme DB and Manual.

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 2007 or later, code the histology as papillary carcinoma, poorly differentiated [8260/33].

The WHO classification lists grade III papillary carcinoma as one of the synonyms for poorly differentiated thyroid carcinoma.

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 Other Sites Histo rules because site specific rules have not been developed for this primary.

Start with the SINGLE TUMOR: INVASIVE ONLY module, rule H8. The rules are intended to be reviewed in consecutive order within a module. Per rule H13 "phenotype" is not a term used to code a more specific histology. Moving to Rule H14 the histology is coded 8260/3 [papillary adenocarcinoma].

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.