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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.

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.

Code the primary site to C421 [bone marrow] and histology to 9823/3 [CLL/SLL]. Per Rule there may be involvement of bone marrow AND lymph node(s), lymph node region(s), organ(s), or tissue(s) but as long as the peripheral blood and/or bone marrow are involved, the primary site is bone marrow (C421).

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 primary site to the involved lymph nodes [C770-C779]. Per Rule PH 8, it indicates you are to code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. Note 2 further states that the bone marrow may or may not be involved. If the bone marrow is involved, code this information in the CS Extension field.

Per the Abstractor Notes section in the Heme DB, this is a systemic disease with widespread lymph node involvement as well as involvement of the peripheral blood. In addition, systemic involvement of extranodal sites (including lung) are often involved.

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, accession two primaries, invasive mucinous carcinoma [8480/3] and in situ ductal carcinoma, solid and cribriform types [8523/2].

The steps used to arrive at this decision are:

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual after determining the histology of each tumor (8480/3 and 8523/2).

Start at the MULTIPLE TUMORS module, rule M4. These tumors have ICD-O-3 histology codes that are different at the second (xxx) and third (xxx) number and are, therefore, multiple primaries.

Code histology to 8153/3 [malignant gastrinoma].

According to the WHO Classification of Tumors of the Digestive System, pages 64-65, carcinoid is a synonym for gastric neuroendocrine tumor (NET) and gastrinoma is synonymous with gastrin-producing NET. Gastrin-producing NET (gastrinoma) is coded 8153/3.

Record the neoadjuvant therapy only for the first primary and do not record the neoadjuvant therapy for the incidental new primary found on surgery. 

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

When there is a question of whether the SEER MP/H Rules or Hematopoietic and Lymphoid Neoplasm Rules apply, check the histology and refer to the Case Reportability Instructions in the Hematopoietic and Lymphoid Neoplasm Manual. All ICD-O-3 morphology codes in the range 9590 - 9992 are included in the Hematopoietic Rules. Mycosis Fungoides [9700/3] is included in this range. Therefore, the SEER MP/H Rules do not apply to mycosis fungoides.

This case should be accessioned as a single primary: mycosis fungoides [9700/3] of the skin, NOS [C449]. Per Rule M2 abstract a single primary when there is a single histology.

Note that in the Primary Site(s) section of the Heme DB, it states the primary site must always be coded to skin (C440 - C449) for mycosis fungoides. Because the primary site is stated in this section of the Heme DB, it is not necessary to use the Primary Site Rules to determine the primary site. Code the primary site to C449 [skin, NOS] because the patient has multiple sites of skin involvement and there is no documentation indicating which subsite of skin was the origin of the mycosis fungoides.

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.