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20140053 | Multiple primaries--Heme & Lymphoid Neoplasms: Is this abstracted as one primary or two?
5/2/13 Bone Marrow biopsy: myelodysplastic syndrome with approaching to acute myeloid leukemia with del 5q and 7q deletions. FISH: deletion of chromosome 5q and deletion of chromosome 7q detected.
I checked the Heme DB manual and there is no term "With approaching to". I checked the Multiple Primary calculator and it says new primary. My interpretation is that the myelodysplastic syndrome is in the process of transforming to acute myeloid leukemia. |
Abstract a single primary, myelodysplastic syndrome with del 5q and 7q deletions (9986/3). This neoplasm can transform to acute myeloid leukemia (AML); however, "with approaching to" cannot be used to report this AML. |
2014 | |
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20110141 | Multiple primaries--Heme & Lymphoid Neoplasms: Should a 2010 diagnosis of central nervous system diffuse large B-cell lymphoma be abstracted as a new primary when the patient has a history of cutaneous T-cell lymphoma in the 1980's and a 1991 history of DLBCL of the bowel (NOS)? See Discussion. |
Patient presents in 2010 with the history of cutaneous T-cell lymphoma and DLBCL. The patient is stated to have been in remission from the DLBCL. However, a current CT scan of the brain is consistent with central nervous system DLBCL. Cerebrospinal fluid cytology is consistent with DLBCL. The CT scan of the torso showed no lymphadenopathy or suspicious findings. Does the recently discovered DLBCL disease process in the central nervous system represent a new third primary? Or is this disease recurrence/progression? The patient was referred to a cancer center and there is no additional information available regarding further workup or treatment. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. The patient only has two primaries: cutaneous T-cell lymphoma diagnosed in the 1980s and diffuse large B-cell lymphoma of the bowel diagnosed in 1991. The DLBCL of the brain does not represent a new primary. It is progression of the 1991 disease process with the same histology. Under the Alternate Names section in the Heme DB, one synonym for DLBCL is "Primary DLBCL of the CNS." The histology code for both the 1991 bowel neoplasm and the current CNS neoplasm is 9680/3. Per Rule M2, a single histology is a single 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. |
2011 |
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20140089 | Multiple primaries--Heme & Lymphoid Neoplasms: Should the 2014 diagnosis be abstracted as a new primary since it is not mantle cell lymphoma and all of the types listed in the differential diagnosis would be a new primary? See discussion. |
Mantle cell lymphoma diagnosed in 1997 which was treated with chemotherapy. Now in 2014 a 'relapse' of non-Hodgkin lymphoma. They do a biopsy of the pericardium, which is called low grade B cell non Hodgkin lymphoma. See comment. The comment says histochemical stains are reviewed and findings are consistent with involvement by a CD5 positive low grade B cell lymphoma. Lack of cyclin D1 and SOX-11 positivity as well as negative IGH-CCND1 FISH analysis essentially rule out mantle cell lymphoma. The morphologic and immunophenotypic features of this disorder are not specific for any lymphoma subtype. The differential includes CLL, marginal zone lymphoma, and lymphoplasmacytic lymphoma. If this is coded NHL, NOS (9591) it is the same primary as seq. 1 and would not be abstracted. |
This is the same primary, the mantle cell lymphoma.
Differential diagnoses cannot be used to assign histology. For the 2014 diagnosis, the only histology that can be assigned is 9591/3 for non-Hodgkin lymphoma, NOS. (CLL, mantle cell lymphoma and lymphoplasmacytic lymphoma are all NHL's.)
Compare the 1997 diganosis of mantle cell lymphoma with the 2014 diagnosis of non-Hodgkin lymphoma. Start with Rule M1. The first rule that applies is Rule M15, which instructs you to use the multiple primaries calculator. Enter 9673/3 and then 9591/3 and then calculate. The result is same primary.
If at a later time one of the differential diagnoses is confirmed, apply the rules again.
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2014 |
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20210029 | Multiple primaries--Heme and Lymphoid Neoplasms: Is a patient with peripheral blood initially showing chronic myelogenous leukemia (CML), lymph node biopsy showing granulocytic sarcoma (9930/3), and bone marrow biopsy showing acute myeloid leukemia (AML) one or two primaries? See Discussion. |
1. 12/11/2020 Peripheral blood revealing what was thought to be chronic myelogenous leukemia BCR/ABL1 positive (9875/3). Patient was started on Hydrea while waiting for further tests on 12/12/2020. 2. 12/14/2020 Lymph node biopsy showed granulocytic sarcoma (9930/3), but flow cytometry states it is similar to that seen in the patient's peripheral blood and is consistent with nodal involvement by myeloblasts. 3. 12/15/2020 Bone marrow biopsy reads acute myeloid leukemia (9861/3), likely arising from BCR/ABL1 positive chronic myeloid leukemia. There is a note on this pathology from medical oncologist that says: This will dramatically change the course of his treatment, likely with a TKI. 4. 12/17/2020 Sprycel started. Patient was weaned off Hydrea. According to Rule M3, abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. It lists 9930/3 when simultaneously (or after) with 9861/3. Technically, it was two days before, but I feel like I should and could count that as simultaneously because of Note 1 that says: These sarcomas are solid manifestations of the associated leukemia. For example, when acute myeloid leukemia and myeloid sarcoma are diagnosed simultaneously, the myeloid sarcoma is the result of myeloid cells migrating from the bone marrow or blood into tissue. It is part of the disease process for the acute leukemia. Also, the providers never mention granulocytic sarcoma Based on that, I think that #2 & #3 above are the same primary, which would be acute myeloid leukemia (9861/3). Per the hematopoietic database, 9875/3 transforms to 9861/3. Therefore, Rule M8 is confusing with the "only one" biopsy. Does this rule apply because the 9875/3 was from peripheral blood only? But peripheral blood is coded in Diagnostic Confirmation as histology. Rule M9 reads: The two diagnoses are likely the result of an ongoing diagnostic work-up. The later diagnosis is usually based on all of the test results and correlated with any clinical information. Because that is truly what I think is happening here though that rule states there is no available documentation. If you do not have any documentation, how would you know you are dealing with a chronic and an acute diagnosis? M10 does not apply. According to Rule M11, abstract as multiple primaries when both a chronic and an acute neoplasm are diagnosed simultaneously or within 21 days and there is documentation of two biopsies. The chronic myelogenous leukemia only had peripheral blood and not a bone marrow, lymph node or tissue, but that is counted as positive histology in diagnostic confirmation, but I don't know if that is kept as a separate field/thought. I would not code a peripheral blood smear as with a surgical code or a surgical diagnostic and staging procedure code, so maybe that is what I should be thinking about and therefore would probably say Rule M8 and one primary. |
This is one primary based on Rule M3. Abstract as a single primary site for the granulocytic sarcoma and AML since they are both evaluating the blood/bone marrow, which are counted as one site. To count them twice would result in over counting primaries. For Rule M9: This would not apply to your situation since you do have information on both the CML and the AML. We had to write in this rule for cases where you do not always have the information available. In terms of the peripheral blood versus actually biopsy: In this case, do not count the peripheral blood as a separate site. Rule M8 does fit your case, coding this as the AML and having this as one primary. |
2021 |
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20081053 | Multiple primaries--Lymphoma: Is a splenectomy done for non-Hodgkin lymphoma diffuse large B-cell of the spleen a composite histology and a single primary if a perihilar lymph node with Hodgkin lymphoma classic type is found at the time of this surgery? |
For cases diagnosed prior to 1/1/2010:This is two primaries -- Non-Hodgkin lymphoma (NHL) in the spleen and Hodgkin lymphoma (HD) in a lymph node. Composite lymphoma is NHL and HD both in a single lymph node. For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ. |
2008 | |
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20091043 | Multiple primaries--Lymphoma: Should a second primary lymphoma be accessioned if the reporting hospital disagrees with the final diagnosis stated on a review of slides? See Discussion. |
Example: Patient had an original diagnosis of small lymphocytic lymphoma (9670/3) of lung in 1986 and later presents with small B-cell non-Hodgkin lymphoma (9670/3) of small bowel in 2008 at Hospital A. Slides sent for review at Hospital B where patient was also seen. Slides there read as low grade B-cell lymphoma most consistent with extranodal marginal B-cell lymphoma of mucosal associated tissue (MALT Lymphoma). Hospital A's pathology report stated that immunostains would exclude mantle cell lymphoma and MALT lymphoma and the original pathology report has not been amended to match the outside path diagnosis. Is this a second primary of MALT lymphoma (9699)? |
For cases diagnosed prior to 1/1/2010:The 2008 diagnosis is not a new primary according to the Definitions of Single and Subsequent Primaries for Hematologic Malignancies (the tri-fold heme table) using the pathology report diagnosis from the facility where the procedure was performed (Hospital A). Since Hospital A disagreed with the slide review and did not amend their diagnosis based on the slide review, do not use the slide review diagnosis in this case. For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ. |
2009 |
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20210039 | Multiple primaries/Heme & Lymphoid Neoplasms--Lymphoma: Is a 2021 right tongue base biopsy showing diffuse large B-cell lymphoma (DLBCL) (9680/3) a new primary following a prior history of hairy cell leukemia-variant (HCL-v) (9591/3) in 2011? See discussion. |
Patient was diagnosed with low-grade non-Hodgkin lymphoma in 2011, later classified as hairy cell leukemia-variant. Right cervical node biopsy in 2020 proved HCL-v and a subsequent 2021 right tongue base biopsy showed DLBCL. The tongue base biopsy path includes the comment, patient has history of HCL-v, but the morphology and flow cytology features are different from the patient's previous right cervical node biopsy. This DLBCL likely represents a second de novo lymphoma, but cannot exclude an unusual transformation of the prior HCL-v. Per Heme Rule M7, abstract a single primary when a more specific histology is diagnosed after an NOS if the Heme DB confirms the same primary. The histology code for HCL-v, 9591/3 is a non-specific code, but it seems like a specific histology. The Heme Calculator does say 9591 and 9680 are the same primary, but we are unsure if that is correct for this case of HCL-v followed by DLBCL. |
Abstract two primaries. This is a transformation from a chronic disease (the Hairy Cell Variant) to an acute disease (DLBCL). Although this rare situation is not clearly covered in the Hematopoietic rules, the fact that this was originally a Hairy Cell Leukemia variant means that the DLBCL is a new primary. |
2021 |
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20130142 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are reported if a 2010 inguinal lymph node biopsy diagnosis of follicular lymphoma, grade 1 is subsequently diagnosed in 2012 with a 50% follicular, grade 3 and 50% diffuse large B-cell via a biopsy of an axillary mass? |
In 2010 a left inguinal lymph node biopsy revealed follicular lymphoma, grade 1. There were no other suspicious lymph nodes in the body. In 2012 a biopsy of a large axillary mass revealed a a 50% follicular, grade 3 and 50% diffuse large B-cell. According to the rules, the transformation to a B-cell is new primary. Is the mixed cell neoplasm a single primary? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. There are two reportable primaries for this case -- follicular lymphoma in 2010 and DLBCL in 2012. First determine the histologies needed to to determine the number of primaries to report. We determined the histologies are follicular lymphoma, grade 1 for 2010 and DLBCL for 2012 as follows:
Per the Hematopoietic database, follicular lymphoma (all types are chronic) transforms to DLBCL (acute). Per Rule M 10 instructions, "Abstract as multiple primaries when a neoplasm is as a neoplasm there is a of an neoplasm after the chronic diagnosis." Therefore, abstract the DLBCL as a 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. |
2013 |
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20100037 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be accessioned for a patient diagnosed with essential thrombocythemia [9962/3] in 2002 who had a 2010 biopsy consistent with the fibrotic stage for a chronic myeloproliferative disorder that "suggests the patient is transforming to an acute myeloid leukemia"? See Discussion. |
Patient had a diagnosis of essential thrombocythemia [9962/3] in 2002 and was treated with Hydroxyurea. In 2010, the patient was admitted with severe bone pain and a diagnosis described as, "The overall features of the biopsy are consistent with a fibrotic stage of a chronic myeloproliferative disorder. The presence of up to 15% CD34+ immature cells seen in the biopsy suggests that the patient is transforming to an acute myeloid leukemia." In addition, cytogenetic studies and molecular testing for JAK2 were ordered. These findings confirmed a myeloproliferative disorder. JAK2 mutation was not detected. The patient died within 2 weeks. Is this a new primary?
Was this patient diagnosed with AML (which requires 20% or more blasts and this is only 15%)? If this is a new primary, is the histology 9861/3 [AML, NOS] or 9895/3 [AML with myelodysplasia-related changes]? Was the second diagnosis of AML definitively diagnosed? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case is a single primary, essential thrombocythemia [9962/3] in 2002. The 2010 diagnosis is chronic myeloproliferative disorder [9960/3].
According to Rule M15, the Multiple Primaries Calculator is to be used to first determine the number of primaries. Per the calculator, essential thrombocythemia and chronic myeloproliferative disorder are the same primary. (Acute myeloid leukemia is not used as the second histology because it is preceded by a non-reportable ambiguous term, "suggests." "Suggests" is not on the list of reportable ambiguous terms in the Hematopoietic and Lymphoid Neoplasm Coding Manual.
In 2010, this patient was in a late stage of ET. When any of the specific MPN neoplasms such as ET are in the late stage of disease, the characteristics of the specific disease (ET) will no longer be detectable. Accordingly, for this patient the diagnostic testing was positive for MPN, unclassifiable. In this case, do not change the diagnosis from the more specific disease (ET) to the NOS (MPN, unclassifiable).
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. |
2010 |
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20120011 | 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. |
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? |
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. |
2012 |
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