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SEER Inquiry System - Report
Produced: 01/21/2026 3:16 PM
Question 20120011
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Is there a timing rule used to recode histology should a more specific diagnosis of refractory anemia with excess blasts (RAEB) be confirmed after an initial diagnosis of myelodysplastic syndrome (MDS)? How many primaries are abstracted if RAEB subsequently evolves toward an acute myeloid leukemia? See Discussion.
Discussion:
Facility A: 4/8/2010 Bone Marrow biopsy: Features most compatible with MDS. (No treatment administered.) 7/2/2010 Peripherial Blood: Transforming Myelodysplastic Syndrome (MDS). COMMENT: Clonal abnormality compatible with MDS/acute myeloid leukemia (AML) in all metaphases examined. (Still no treatment administered.)
Facility B: 10/6/2010 Patient now presents for evaluation and treatment. Patient started on Vidaza. 10/07/10 Bone Marrow biopsy: Refractory anemia with excess blasts (RAEB-2) COMMENT: Evolution towards AML with myelodysplasia related changes considered; cytogenetic analysis reveals abnormalities most compatible with MDS and/or AML.
Based on the Heme Manual and DB, the 4/8/2010 diagnosis of MDS, NOS (9989/3) is the first primary. Should the 7/2/2010 diagnosis of transforming MDS to AML (9861/3) be a new, second primary?
Based on the Abstractor Note for MDS in the Heme DB for MDS, "If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis." Based on this note, should the MDS histology code [9989/3] be changed to refractory anemia with excess blasts (RAEB-2) [9983/3] from the biopsy taken on 10/7/2010 (one day after treatment began) that revealed RAEB-2 with evolution towards AML?
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics."
Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.]
The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML.
It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML.
The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second 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.
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) will appear which indicates the correct version of the program is available now for query.
The Abstractor Note for MDS was interpreted correctly. Update the histology from MDS, NOS [9989/3] to refractory anemia with excess blasts (RAEB-2) [9983/3] when a more specific subtype of MDS is diagnosed after the NOS histology.
There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics."
Abstract a single primary, the diagnosis of MDS/RAEB-2 [9983/3]. The steps used to arrive at this decision are:
Enter in the Heme DB to search for the histology code. Click on the SEARCH button. Ensure that 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. Note that RAEB-2 can transform to AML.
The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability but it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML.
See the following explanation of why care must be taken when using ambiguous terminology.
The patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML if left untreated is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML. The other problem with this case is is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." That means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second primary.
Abstract a single primary, RAEB-2 [9983/3] diagnosed 4/8/2010.
