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SEER Inquiry System - Report
Produced: 05/15/2025 2:58 AM
Question 20110031
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Discussion:
The patient has a history of refractory anemia with excess blasts diagnosed in 2008. A vocal cord biopsy performed on 6/2/2010 stated, "in view of a previous history of myelodysplastic syndrome this is indicative of transformation to acute leukemia" and consistent with granulocytic sarcoma. A bone marrow biopsy done on 7/19/2010 stated this was compatible with refractory anemia with excess blasts in transformation.
Granulocytic sarcoma is a solid manifestation of AML. When these diagnoses occur more than 21 days apart, are they separate primaries?
According to the WHO definition, this is acute myeloid leukemia complicating myelodysplasia. Which rule applies for this case?
Answer:
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.
History:
This SINQ question has been updated to the Hematopoietic & Lymphoid Neoplasm Manual & Database published January 2014.
The original answer below was written based on the rules in 2010
For cases diagnosed 2010-2011, access the Hematopoietic Database at http://seer.cancer.gov/tools/heme/. Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. For 2010-2011 diagnoses, click on the "use the 2010 database" label in the upper right corner of the screen. The 2010 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.
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.
Enter in to the Heme DB to search for the histology. Click on the SEARCH button. The term "myeloid sarcoma" [9930/3] is highlighted on the screen under the RESULTS FOR ALL TERMS area.
Check the ABSTRACTOR NOTE section which states "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." Per the TRANSFORMATIONS section, there are not transformations from this disease process.
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. 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. The physician suspected that when he/she saw the deposit on the vocal cord and gave the provisional diagnosis of transformation to acute leukemia and the July bone marrow confirmed that provisional diagnosis.
Enter with excess blasts in to the Heme DB to search for the histology. Click on the SEARCH button. The term "refractory anemia with excess blasts" [9983/3] is highlighted on the screen under the RESULTS FOR ALL TERMS area.
Scroll down to the TRANSFORMATIONS section information. Refractory anemia with excess blasts (a chronic disease process) transforms to acute myeloid leukemia, NOS (an acute disease process). When a cell type is listed in the transformation section for a given malignancy, the disease process in the transformation section is considered the acute process and the disease listed at the top of the screen display is considered the chronic process. In this case, the chronic and acute disease processes were diagnosed at different times.
Determine the number of primaries. Click on the 2010 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, locate one of the three formats (i.e., flowchart, matrix or text) to check the Multiple Primary Rules. The rules are intended to be reviewed in consecutive order from Rule M1 to Rule M13. Stop at the first rule that applies to the case you are processing.
. 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.
