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Report Produced: 05/23/2025 08:21 AM

Report Question ID Question Discussion Answer Year
20110109

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted when a patient is simultaneously diagnosed with multiple myeloma/plasma cell myeloma, plasmacytoma and plasma cell leukemia?

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.

 2011
20130209

Multiple primaries--Heme & Lymphoid Neoplasms: Is a new bone marrow diagnosis of acute myelogenous leukemia that follows a 2007 treated diagnosis of a JAK-2 positive polycythemia vera a new primary?

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

Per Rule M10, abstract two primaries. Per the Heme DB, polycythemia vera [9950/3] transforms to an acute myelogenous leukemia [9861/3]. According to Rule M10, one is to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (e.g., polycythemia vera) AND there is a second diagnosis of an acute neoplasm (e.g., acute myelogenous leukemia) 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.

 2013
20110010

Multiple primaries--Heme & Lymphoid Neoplasms: Is a recently diagnosed granulocytic sarcoma followed by a diagnosis of AML two primaries? See Discussion.

6/10/10 Axillary lymph node biopsy was compatible with AML. The physician noted that the patient was diagnosed with granulocytic sarcoma [9930/3] in the axillary node.

6/15/10 Bone marrow biopsy compatible with AML FAB M1 [9873/3].

After induction, a second bone marrow biopsy on 6/30/10 shows persistent/refractory AML. The physician noted that the second biopsy is compatible with AML FAB M7 [9910/3].

Is the granulocytic sarcoma a chronic form of the disease? If so, do we have one primary diagnosed 6/10/10 with primary site coded to C42.1 and histology coded to 9873/3? Does the second biopsy on 6/30/10 represent the same primary even though the persistent disease is now FAB M7?

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

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 (i.e., at the same time or within 21 days of on another).

Apply Rule M3 to this case which states to abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Code the primary site to C421 [bone marrow] with histology coded to 9873/3 [acute myeloid leukemia, M1]. The FAB category is an older classification that is seldom used. Changes from FAB 1 to FAB 7 do not constitute a new 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
20150055

Multiple primaries--Heme & Lymphoid Neoplasms: Is this 2 primaries? In 2011, a patient had a spinal mass biopsied positive for DLBCL and follicular lymphoma. The heme rules make this one primary coded as DLBCL. Patient had 2 rounds of chemo, but in 2014, he had a recurrent tumor in the same location. The 2014 biopsy was follicular lymphoma. Is this a new primary -- conversion of acute to chronic after treatment? Or is it the same, since FL was diagnosed in the original specimen?

Rule M13 applies, abstract as two primaries. Since both DLBCL and FL were present in 2011, rule M2 does not fit -- not a single histology. Rule M13 reflects the situation in this case much better: an acute neoplasm which was treated and a chronic neoplasm diagnosed later.

2015
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
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
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.

 

 

 

 

2014
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
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
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