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This case is reportable.

It is possible that the polypectomy removed the entire tumor. Invasive carcinoma in a polyp does not mean that is has invaded the stalk of the polyp. If the stalk is not invaded, all of the cancer may have been removed by a polypectomy.

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

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 (i.e., at the same time or within 21 days of on another).

Apply Rule M3 to this case which states to abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Code the primary site to C421 [bone marrow] with histology coded to 9873/3 [acute myeloid leukemia, M1]. The FAB category is an older classification that is seldom used. Changes from FAB 1 to FAB 7 do not constitute 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.

Code histology to 8310/3 [clear cell adenocarcinoma]. The Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later.

The following steps are used to determine the histology code:

Open the Multiple Primary and Histology Coding Rules manual. For an endometrial primary, use the Other Sites Histo rules to determine the histology code because endometrium does not have site specific rules.

Go to the SINGLE TUMOR: INVASIVE ONLY module, which starts at Rule H8.

. Code clear cell adenocarcinoma [8310/3] because only one histologic type is identified.

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.

Do NOT use the Collaborative Stage Manual to determine the histology code. For CS STAGING purposes only, coding should be based on the squamous cell carcinoma component of this tumor.

The Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology. For cases diagnosed 2007 or later, the following steps are used to determine the histology code:

Open the Multiple Primary and Histology Coding Rules manual. For an esophagus primary, use the Other Sites Histo rules to determine the histology code because esophagus does not have site specific rules.

Start at Rule H8 because this is an invasive histology (assuming this is a single tumor). which states that one should code the appropriate combination/mixed code from Table 2 when there are multiple specific histologies.

Find Other Sites for Table 2 under the Terms & Definitions section of manual.

Locate the appropriate mixed code for squamous cell carcinoma and adenocarcinoma in column 1. Per column 3, the correct histology is adenosquamous carcinoma. Per column 4, the correct histology is 8560/3.

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

This is accessioned as one primary and the histology is coded to 9732/3 [multiple myeloma].

To arrive at this answer, it is important to first try to determine how many different unique neoplasms there are to correctly identify the number of primaries to report. Per the Heme DB, plasma cell leukemia is an obsolete term. The current term and histology code for this diagnosis is 9732/3 [plasma cell myeloma]. Plasma cell myeloma and multiple myeloma are synonyms per the Heme DB. Therefore, per Rule M2 a single primary exists when there is a single histology. That takes care of the multiple myeloma/plasma cell myeloma and plasma cell leukemia, but not the plasmacytoma.

In checking the Heme DB, the terms plasma cell myeloma and multiple myeloma are not synonyms for plasmacytoma. Therefore, we are left to determine whether the multiple myeloma/plasma cell myeloma vs the plasmacytoma represents one or two primaries.

Under the Transformation section of the Heme DB, it indicates that plasmacytoma (a chronic disease process) transforms to multiple myeloma (an acute disease process). Per Rule M9, abstract a single primary and code the acute histology when both a chronic and an acute neoplasm are diagnosed simultaneously. The histology is coded to the acute neoplasm when there is no information on the biopsy regarding which is the "later" histology. This update will be added to the Heme 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 prior to 2021

According to our expert pathologist consultant, code using the terms in the pathology report. Do not try to second guess the pathologist.

1. Thymomas are /1 and not reportable unless the pathologist states "malignant". This includes A, AB, B1, B2, and B3. Any one of these types of tumors may exhibit invasion but do not code /3 based only on invasion.