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Code as a single primary. The histology is carcinoid.

Our expert pathology consultant replied as follows: "This patient clearly has a condition driving the proliferation of neuroendocrine cells. Possibilities include hypergastrinemia from a gastrinoma or from response of antral gastrin cells due to achlorhydria from long standing chronic atrophic gastritis, or multiple endocrine neoplasia (MEN1) syndrome (genetically driven). How should these cases be coded given we do not have a way to code the inciting situation. (I suspect the gastroenterologist knows what it is, but we haven't obtained that information.) We do not have an ICD-O-3 code for the underlying condition, MEN1 or hypergastrinemia. Therefore, the only choice is to code the resulting tumor, carcinoid [8240/3]."

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

Code the histology to 9837/3 [adult T cell leukemia/lymphoma] and the primary site to C778 [lymph nodes, multiple regions]. Per Rule PH8, code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. A statement of "mediastinal mass" and lymphadenopathy for lymphoma primaries is equivalent to lymph node involvement. To identify the more specific primary site, you need to move to Rule PH21 that indicates you are to code the primary site as multiple lymph node regions, NOS [C778] when multiple lymph node regions, as defined by ICD-O-3, 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.

Assuming the same primary site for the 2008 lesion, according to the current MP/H rules the high grade fibrosarcoma [8810/3] is a new primary per Head & Neck MPH rule 11 because it is a different histology.

The revised MP/H rules will include tables to define tumors that de-differentiate (transform) and recur with what is seemingly a different histology. Although the rules will be changed in the future, we must use the rules in place at this time for this case.

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

This should be accessioned as a single primary per Rule M8 which states to abstract as a single primary and code the acute neoplasm when both a chronic (MDS) and an acute (AML) neoplasm are diagnosed simultaneously or within 21 days AND there is documentation of only one positive bone marrow biopsy, lymph node biopsy, or tissue biopsy. Code the histology to 9895/3 [acute myeloid leukemia with myelodysplasia-related changes].

NOTE: When you search with quotation marks around the phrase, the database will only return results with that exact wording. To only return results for the expression trisomy 13, enter in the Heme DB. In this case, a search for "trisomy 13" returns no results. Therefore, it does not impact the coding of histology for this case.

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 histology to 8244/3 [composite carcinoid]. Rule H9 applies: Code 8244 [composite carcinoid] when the diagnosis is adenocarcinoma and carcinoid tumor. WHO describes these tumors as "mixed adenoneuroendocrine carcinoma (MANEC)." They have components of adenocarcinoma mixed with high-grade neuroendocrine carcinoma (NEC), which can be either small cell or large cell.

The next version of the MP/H rules for colon will make this clear by adding a note regarding this issue to Rule H9.

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.

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

"Thrombocytopenia of unknown etiology" is not reportable. Thrombocytopenia refers to a low platelet count which causes bleeding. Thrombocytopenia can be caused by viral infections, excessive alcohol usage, HIV, and other causes (including chemotherapy). If the diagnosis is not "refractory thrombocytopenia" the case is not reportable. Appendix F lists this term as non-reportable.

If you do not see the term in the Heme DB under either the Name column or the Alternative Names section for the results returned, it is not reportable. The only reportable term that contains the word thrombocytopenia is refractory thrombocytopenia. Therefore, thrombocytopenia of unknown etiology is not reportable.

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 should be accessioned as two primaries per Rule M15. Rule M15 instructs one to use the Heme DB Multiple Primaries Calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14. Code the histology for the 1999 primary to 9687/3 [Burkitt high grade B cell lymphoma] and code primary site to C739 [thyroid.] Code the second primary to 9680/3 [diffuse large B-cell lymphoma] with primary site coded to C079 [parotid gland] per Rule PH24 which instructs one to code the to the when lymphoma is present only in an .

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 should be accessioned as two primaries. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic 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.