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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.

This is a single primary, epithelioid sarcoma of the left upper limb, diagnosed in 2003. The sarcoma progressed over the years and the patient was never free of disease -- positive margins were documented at each surgical event. Per the 2004 SEER Manual coding rules in place at the time of pre-2007 recurrences, they would not be multiple primaries according to Rule 5, exception 1.

The occurrence in 2010 is also not a new primary. The steps used to arrive at this decision are as follows.

Open the Multiple Primary and Histology Coding Rules manual. For a soft tissue primary, use one of the three formats (i.e., flowchart, matrix or text) under the Other Sites MP rules to determine the number of primaries because soft tissue primaries do not have site specific rules.

Start with the UNKNOWN IF SINGLE OR MULTIPLE TUMORS module, Rule M1. The rules are intended to be reviewed in consecutive order within the module that applies for this case. In this module there is only one rule.

. This patient was never disease free and it is unknown if this tumor was the same tumor (single tumor) or multiple tumors. Abstract a single primary for this patient.

Donor lymphocyte infusion (DLI) is coded as immunotherapy. The lymphocytes are donated by the same person who donated the original stem cell transplant. The lymphocyte infusion creates an immune response in which the T-cells are activated to attack the cancer cells.

See "Treatments" for CLL/SLL (9823/3)

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

Code histology to 9680/3 [diffuse large B-cell lymphoma]. Code 9684/3 [malignant lymphoma, diffuse large B-cell, immunoblastic NOS] is obsolete for cases diagnosed 2010 and later per the Heme DB.

Under the Definitions section in the Heme DB, it states that this is a lymphoma with diffuse proliferation of large neoplastic B lymphoid cells with nuclear size exceeding macrophage nuclei, more than twice size of normal lymphocytes. Normal architecture of node or extranodal tissue replaced in diffuse pattern. Morphologic variants: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.

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 for PanIN-III to 8148/2 [Glandular intraepithelial neoplasia, grade III]. 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 a pancreas primary, use the Other Sites Histo rules to determine the histology code because pancreas does not have site specific rules.

Go to the SINGLE TUMOR: IN SITU ONLY module, start at rule H1. Code 8148/2 [Glandular intraepithelial neoplasia, grade III]. There is only one histologic type identified.

In the next version of the MP/H rules, the H22 rule "Code 8148/2 (Glandular intraepithelial neoplasia grade III) for in situ glandular in sites such as the (PAIN III)" will be included under H2 as well. Currently the rule is only in the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module and should also be include under the SINGLE TUMOR: IN SITU only module.

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.

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

The histology is coded as Plasma cell myeloma [9732/3]. The diagnostic confirmation is coded to 1 [positive histology].

Under the Definitive Diagnostic Methods section in the Heme DB it indicates that a bone marrow aspiration and bone marrow biopsy are procedures used to diagnose this disease process. This patient's diagnosis was based on the pathology (presumably from a bone marrow biopsy).

NOTE: This is a reportable case. Ambiguous terminology is used to accession cases (determine reportability) because it has been used for over 30 years to do so. Any deviation from using ambiguous terminology to determine case reportability would cause the reporting of incidence counts to vary. In this case, there was a reportable, ambiguous terminology diagnosis of plasma cell myeloma on the pathology report; as well as a reportable physician's statement/diagnosis of plasma cell myeloma.

Ambiguous terminology, however, is not used to report a more specific diagnosis for the Heme & Lymphoid neoplasms. For example, if the pathology report final diagnosis was "Myeloproliferative neoplasm, probably Polycythemia Vera" the histology would be coded as myeloproliferative neoplasm, unclassifiable [9975/3]. The ambiguous terminology indicates that the genetic testing, immunophenotyping, etc., probably are not complete or are not diagnostic of the more specific disease. Wait to code the histology until there is a definite diagnosis given.

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.

The behavior code for hemangioblastoma can be coded to /3 when a pathologist indicates that the behavior is malignant. The behavior code should be based on a pathologist's opinion. It is usually not possible for a radiologist or patient care physician to make this determination clinically.

The histologic appearance of hemangioblastoma may resemble metastatic renal cell carcinoma; therefore, one will often see renal cell carcinoma listed as a possible diagnosis. This does not indicate that the hemangioblastoma is malignant. Do not code the behavior as /3 based on a differential diagnosis of renal cell carcinoma.