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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.

For cases diagnosed 2007 or later, code the primary site to ovary [C56.9] and the histology to papillary carcinoma, follicular variant [8340/3].

The steps used to arrive at this decision are:

Refer to the 2012 SEER Manual for help to determine the primary site. This neoplasm is arising in a teratoma of the ovary. Per the 2012 SEER Manual, in this case the site is coded to ovary [56.9] because that is where the tumor originated. Although the teratoma contains thyroid tissue, it arose in the ovary. Teratomas are unusual in that they contain all three germ cell layers from which an embryo forms. It is not unusual to have malignancies that are usually primary to the thyroid, liver, brain, lung, etc., originate in a teratoma.

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 Histology 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. Code the histology as papillary carcinoma, follicular variant [8340/3].

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.

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.

Arimidex has not been approved to treat endometrial cancer. It is not prescribed for pre-menopausal women. Clarify with the physician why the drug was being used. If the physician states Arimidex was given to reduce tumor burden, code as hormone therapy.

See the SEER*Rx interactive database, http://seer.cancer.gov/seertools/seerrx/

For cases diagnosed 2007 or later, accession two primaries, right breast cancer diagnosed in June 2006 and a subsequent right breast primary diagnosed in December 2011.

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. Accession two primaries, tumors diagnosed more than five (5) years apart are multiple primaries.

If the pathology report stated the tumor originated in residual breast tissue, then this is a new tumor and, therefore, a new primary per rule M5. If the pathology report stated the tumor arose in the chest wall and/or there is no designation of residual breast tissue, then this is a regional metastasis and not a new primary.

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.

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.

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.