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Report malignant myopericytoma as 8824/3 for cases diagnosed 2021 and later.

This case should be accessioned as one primary.

Reportability is determined by the year of diagnosis. The original DLBCL was diagnosed in 2003 and the follicular lymphoma in 2009. The pre-2010 rules are used for both cases. Per the Single Versus Subsequent Primaries Table, these are the same primary. It is reported with the histology 9680/3 [diffuse large B-cell lymphoma]

Do not compare the DLBCL diagnosed in 2003 and the follicular lymphoma diagnosed in 2010 because the determination of the number of primaries for the two specific histologic types was done (as it should have been) using the rules in effect in 2009 when the follicular lymphoma was first diagnosed. The determination of a single or multiple primaries is made the first time the patient presents with the two different diseases; it is not changed when the same disease process reappears after 2010.

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

This case should be accessioned as a single primary. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].

The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.

The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."

According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.

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, determine the histology when rules PH1-PH29 do not apply. Code diffuse large B-cell lymphoma, germinal cell type to 9680/3 [diffuse large B-cell lymphoma (DLBCL)][9680/3]. Under the Alternate Names section of the Heme DB, these two terms are synonyms that share the same histology code.

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 as Myelodysplastic syndrome, unclassifiable [9989/3].

Ambiguous terminology is used to accession cases (determine reportability). While ambiguous terminology is generally not used to code a specific histology, it can be used to code histology if it is the .

The statement that you do not use ambiguous terms to code histology is intended for those NOS histologies with an ambiguous term being used to describe the subtype. For example, if the physician states this is a myelodysplastic syndrome, NOS, refractory thrombocytopenia. The correct histology would be MDS, NOS [9989/3] and not refractory thrombocytopenia [9992/3].

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 hard-to-treat diseases such as DLBCL, the treatment plan outlined prior to treatment beginning may indicate, "The first course of treatment will be radiation and R-CHOP. If the R-CHOP does not achieve remission, we will use RICE." In other words, the first course treatment plan includes a second round of chemotherapy if the patient has not achieved a complete response after the R-CHOP and radiation. If the treatment plan was documented like this for the patient, the first course treatment includes R-CHOP, involved field radiation and RICE.

However, if there is no initial treatment plan in the medical record, all treatment provided after the date when "residual disease" or "failed to achieve remission" is documented in the medical record is either second or a subsequent course of therapy.

Code the histology to 8082/3 [urothelial carcinoma, plasmacytoid].

The Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later. Unfortunately, in this case there is no current rule that directs you appropriately to Table 1 from Rule H7 to find this histology combination. We need to add an example under Rule H7 that instructs you to "See Table 1" for an urothelial carcinoma diagnosis that mentions a more specific cell type (e.g., plasmacytoid). We will add a reference to Table 1 in Rule H7 in the updates to MP/H Rules.

According to the MP/H rules, code histology to 8010/2 [intraepithelial carcinoma] for cases diagnosed 2007-2014. Borderline mucinous tumor is not reportable to SEER.

The steps used to arrive at this decision are:

Go to the Other Sites Histo rules found in the Multiple Primary and Histology Coding Rules Manual.

Start at the SINGLE TUMOR: IN SITU ONLY module, rule H1. Code the histology when only one histologic type is identified. The only reportable histology in this case is intraepithelial carcinoma [8010/2].

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

Idiopathic thrombocytic purpura (ITP) is not a synonym for refractory thrombocytopenia (RT). ITP is not a reportable disease. See Appendix F.

Under the Alternate Names section in the Heme DB, the only synonym for refractory thrombocytopenia is "RT." ITP is not listed as a synonym for refractory thrombocytopenia.

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.