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For cases diagnosed 2007 or later, accession two primaries, ductal with apocrine features in the left breast and lobular carcinoma in the right breast.

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 MP rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. The patient has tumors in both the right and left breasts.

Rule M6 does not apply because inflammatory carcinoma involves only the left breast and the patient has a different histology in the right breast and there is no mention of inflammatory carcinoma in that breast. In this situation continue to the next applicable rule.

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

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. Per Appendix F, HLH is caused by an over stimulated immune system (infection, etc.). It is a clinical syndrome associated with a variety of underlying conditions. To be reportable, a child's diagnosis must state "fulminant hemophagocytic syndrome" to be reportable (9724/3). This is not the situation in this case.

"Hemophagocytic lymphohistiocytosis" is also listed in Appendix F: Non-Reportable List for Hematopoietic Diseases.

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.

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.

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 primary site to C119 [nasopharynx] per Rule PH25.

Per our subject matter expert, use Module 7 Rule PH25 to code the primary site to an organ (nasopharynx and maxillary sinus) because an organ(s) and its regional lymph nodes are involved. The distant lymph nodes are simply part of the staging (the lymphoma has progressed to another lymph node region).

Diffuse large B cell lymphoma originating in the oral cavity and maxillofacial region is rare, but documented. The most common sites for this rare neoplasm are Waldeyer ring, tonsils, nasopharynx, base of tongue, and palatine tonsil. There are also rare cases of diffuse large B cell lymphoma originating in the maxillary sinus. The percentage of cases arising in the nasopharynx is greater than those originating in the maxillary sinus.

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 M3. Code histology to 9891/3 [acute monocytic leukemia] diagnosed in 2009 and primary site to C421 [bone marrow].

Per Rule M3 a single primary is reported when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Histology 9891/3 [acute monocytic leukemia] is listed as one of the histologies in the "same lineage." Myeloid sarcoma (9930/3) diagnosed simultaneously with or after acute myeloid leukemia (9861/3) or another leukemia of the myeloid lineage (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3).

NOTE: Under the Alternate Names section of the Heme DB, granulocytic sarcoma is a synonym for myeloid sarcoma.

Per PH10, code the primary site C421 [bone marrow] and code the histology acute myeloid leukemia, NOS (9861/3) or any of the specific AML histologies (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3) when the diagnosis is myeloid sarcoma (9930/3) AND there is a simultaneous or previous diagnosis of acute myeloid leukemia.

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 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.

Abstract two primaries for this scenario per Rule M7. The first primary is the renal pelvis in Oct. 2006; the second primary is the ureter in Nov. 2009. The bladder tumor in July 2008 is not a new primary per Rule M8.

Compare the diagnosis date of the current (most recent) tumor to the diagnosis date of the original tumor. This applies even if the patient had six occurrences in-between these dates; you still compare the current tumor to the diagnosis date of the original tumor and ignore recurrences in this process.

See slide 6 of the Beyond the Basics presentation,

http://www.seer.cancer.gov/tools/mphrules/training_adv/SEER_MPH_Gen_Instruc_06152007.pdf.