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Report Produced: 07/31/2024 00:27 AM

Report Question ID Question Discussion Answer Year
20130086 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is diagnosed in 2008 with chronic myeloid leukemia, chronic phase and is subsequently diagnosed with both accelerated phase (2010) and blast crisis of CML (2012)? See Discussion.

Patient diagnosed in 1/2008 with CML, Chronic phase and had a complete remission following treatment.

In 3/2010 the patient was diagnosed with CML, Accelerated phase and again had a complete remission following treatment.

In 02/2012 the patient was diagnosed with CML, Blast crisis.

How do chronic and acute neoplasms (Rules M8 - M13) relate to histologies that are stated to have Chronic, Accelerated and Blast phases per the Heme DB? These histologies don't change, does this mean Rules M8 - M13 do not apply because there isn't a change in histology? How many primaries should be accessioned in this case?

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

This case is accessioned as a single primary, chronic myeloid leukemia, NOS [9863/3] diagnosed 01/2008 per Rule M2. The patient was diagnosed with CML, NOS [9863/3] in 2008 and again in 2010 and 2012. Abstract a single primary when there is a single histology.

CML, Chronic phase; CML, Accelerated phase; and CML, Blast phase (Blast crisis) are listed under the Alternate Names section for CML, NOS in the Heme DB.

Not all histologies have transformations. If a transformation is not listed in the Heme DB, Rules M8 - M13 do not apply.

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
20130081 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is clinically stated to have Stage III follicular lymphoma following a diagnosis suspicious for B-cell lymphoma and is subsequently diagnosed with large B-cell lymphoma? See Discussion.

01/27/2012 R neck mass FNA: Suspicious for B-cell non-Hodgkin lymphoma.

02/17/2012 Cervical node bx: In situ involvement by follicular-like B-cells of uncertain significance +CD10. Two other cervical biopsies show infarcted, extensively necrotic lymphoid tissue highly suspicious for B-cell lymphoma.

03/20/2012 Bone marrow: Low grade B-cell lymphoproliferative disorder with plasmacytic differential.

04/18/2012 Medical Oncology treats patient for Stage III follicular lymphoma.

10/16/2012 Cervical LN core bx: CD10+ large B-cell lymphoma.

Should Rule M4 (single primary) and Module 6, Rule PH11 apply to this case?

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: follicular lymphoma [9690/3] diagnosed 02/17/2012 and diffuse large B-cell lymphoma [9680/3] diagnosed 10/16/2012 per Rule M10. This patient was diagnosed with a chronic neoplasm (follicular lymphoma) followed greater than 21 days later by an acute neoplasm (DLBCL).

The follicular lymphoma was initially diagnosed on 02/17/2012. The cervical node biopsies were "highly suspicious for B-cell lymphoma" [9591/3]. While "suspicious" is a reportable ambiguous term used to accession cases, suspicious cytologies are not SEER reportable and, therefore, the diagnosis date cannot be 01/27/2012. The histology of the first primary would be updated to 9690/3 [follicular lymphoma] based on the Medical Oncology note on 04/18/2012 that confirmed the histology was follicular lymphoma and the patient was being treated for such.

The diagnosis of DLBCL was made 8 months later. Rule M4 cannot apply to this case because the follicular lymphoma and DLBCL were not diagnosed simultaneously. Rule M4 only applies when the two non-Hodgkin lymphomas are diagnosed simultaneously AND in the same location.

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
20130044 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient has a biopsy diagnosis of extraosseous plasmacytoma followed fourteen days later with a diagnosis of multiple myeloma on imaging? See Discussion.

On 2/3/12 the patient was diagnosed via biopsy with an extraosseous (extramedullary) plasmacytoma filling both nasal cavities. On 2/16/12 a metastatic workup was performed and showed a lucent lesion in calvarium of Rt frontal bone. 2/17/12 radiation oncology consult states, "I believe this is most likely consistent with multiple myeloma." Subsequently, the 3/1/12 CT of the left shoulder showed a 3.6 cm lytic lesion of humeral head with cortical breakthrough consistent with metastasis or myeloma.

Per the Heme DB, extramedullary plasmacytoma can transform to multiple myeloma. Does that make this multiple primaries with the histologies coded to 9734/3 and 9732/3?

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

This case should be accessioned as a single primary, multiple myeloma [9732/3] diagnosed on 2/3/12 per Rule M8. Abstract the acute neoplasm as a single primary when both a chronic and acute neoplasm are diagnosed simultaneously or within 21 days AND there is documentation of only one positive tissue biopsy.

This patient only had a tissue biopsy of the nasal cavity proving the chronic neoplasm (extraosseous plasmacytoma). The acute neoplasm (multiple myeloma) was diagnosed clinically based on the scans.

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
20100088 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient has 2005 diagnosis of multiple myeloma diagnosed returns in 2010 with extramedullary plasmacytoma and a bone marrow biopsy showing plasma cell dyscrasia that is clinically stated to "consistent with a relapse of myeloma"? See Discussion.

Patient was diagnosed in 2005 with multiple myeloma and following stem cell transplant 2005 was in complete remission.

On 2/1/10 an excisional biopsy of a soft tissue right flank mass showed plasmacytoma. On 3/2/10 the bone marrow biopsy was stated to be consistent with plasma cell dyscrasia. An outside attending physician stated the bone marrow biopsy was consistent with a relapse of myeloma. There was no radiologic evidence of disease elsewhere as of Feb 2010, only the soft tissue right flank mass. Patient initially presented for post-op radiation to the right flank and was treated 3/29/10. On 8/6/10 a biopsy of a right perinephric mass was positive for plasmacytoma. Subsequent xray on 8/16/10 of the right tibia and fibula showed lytic lesion consistent with progression of myeloma.

Using the Hematopoietic Database, the plasmacytoma in 2/1/10 is a second primary. How do the rules apply to the perinephric soft tissue disease and right tibia lesion? Are they separate new primaries? Or is all of this simply a recurrence of the original 2005 diagnosis as the attending physician states?

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

Accession a single primary with the histology coded to 9732/2 [multiple myeloma]. The disease discovered in 2010 represents further advancement of former disease. Per the Abstractor Notes section in the Heme DB, it states that bone marrow involvement, lytic bone lesions, and bone tumor masses of plasma cells are common. Under the Recurrence and Metastases section in the Heme DB it further states that extramedullary (in tissue other than the bone) involvement is a generally a manifestation of advanced disease. This case is an example of such a situation.

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
20120070 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a bone marrow biopsy shows myelodysplastic syndrome - refractory anemia with excess blasts type 2 (RAEB-2) and myelofibrosis? See Discussion. Should the myelofibrosis be accessioned as a second primary? Or is it a descriptor of the MDS/RAEB-2? The multiple primaries calculator shows 9983/3 and 9961/3 represent two primaries.

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

Accession a single primary per Rule M2 which indicates you are to abstract a single primary when there is a single histology. Code the histology to 9983/3 [refractory anemia with excess blasts type 2 (RAEB-2)].

Per Appendix F, myelofibrosis, NOS, is NOT a synonym for primary myelofibrosis. Myelofibrosis, NOS, if not specified to be myelofibrosis, therefore, is not reportable.

Per PH29, code the specific histology when the diagnosis is one non-specific (NOS) histology (MDS) and one specific histology (RAEB-2) AND the Multiple Primary Calculator confirms the specific histology and NOS histology are the same primary (which it does).

Myelodysplastic syndrome, NOS is a generic disease description. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. The more specific myelodysplastic syndromes are: refractory anemia; refractory neutropenia; refractory thrombocytopenia; refractory anemia with ring sideroblasts; refractory cytopenia with multilineage dysplasia; refractory anemia with excess blasts; and refractory cytopenia of childhood. 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.

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
20100096 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a 9/30/10 biopsy diagnoses follicular lymphoma, grade 1 and the patient is subsequently diagnosed on a 10/11/10 biopsy with large B-cell lymphoma which is stated to be a transformation of the prior lymphoma?

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

Per Rule M11, this case is to be accessioned as two primaries; follicular lymphoma, grade 1 [9695/3] and diffuse large B-cell lymphoma (DLBCL) [9680/3]. The case represents a chronic neoplasm (follicular lymphoma, grade) and an acute neoplasm (diffuse large B-cell lymphoma) diagnosed within 21 days of one another and there is documentation of two biopsies, one confirming the chronic disease and the other confirming the acute disease.

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
20130045 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if subsequent to a bone marrow biopsy diagnosis of acute myeloid leukemia there is an oncology consult note that indicates the pathology finding is suggestive of an underlying myelodysplastic syndrome? See Discussion

5/14/12 Bone marrow biopsy: Acute myeloid leukemia (AML).

5/21/12 Oncology consult: AML with 30-40% blasts and evidence of del(20q) and del(5q), is suggestive of an underlying myelodysplastic syndrome (MDS). Hence the patient has secondary AML.

If these are two primaries, how are the diagnosis dates coded?

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

This case is accessioned as a single primary diagnosed on 5/14/12 as acute myeloid leukemia with myelodysplasia-related (e.g., del(5q)) changes [9895/3] per Rule M2. The patient was diagnosed with a single histology, acute myeloid leukemia with myelodysplasia-related changes per the submitted information.

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
20110031 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if patient initially diagnosed with granulocytic sarcoma on a vocal cord biopsy is subsequently diagnosed with acute myeloid leukemia more than 21 days later? See 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?

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.

2011
20110155 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient shows evidence of "MDS as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera" 18 years after a diagnosis of "thrombocytosis and probable polycythemia that progressed to probable myelofibrosis"? See Discussion

Per consultation: an 83 year old patient started on hydroxurea 18 years ago following a diagnosis of thrombocytosis and probable polycythemia. It appears the polycythemia progressed to probable myelofibrosis. The possibility of an MDS needs to be considered.

Problem list: Polycythemia with probable progression to myelofibrosis or MDS.

Bone marrow biopsy two weeks later shows some progression of dysmegakaryocytopoiesis. Patient has evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera.

On follow-up visit six weeks later: Continue to manage patient with hydroxyurea.

An additional six months later: Diagnosis is polycythemia with thrombocytosis.

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

This case should be accessioned as a single primary. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].

The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.

The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."

According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.

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
20120083 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient is diagnosed with follicular lymphoma, grade 3 in 2006 and is subsequently diagnosed with follicular lymphoma, grade 2 in 2011? See Discussion.

June 2006, the patient was diagnosed with follicular lymphoma, grade 3 by cervical lymph node biopsy and bone marrow biopsy. The patient refused treatment but was followed.

May 2007, the patient had another cervical LN biopsy with a diagnosis of follicular lymphoma, grade 2.

July, 2009, a neck mass excision was diagnosed as follicular lymphoma, grade 3.

June 2011, another neck lymph node was excised and diagnosed as follicular lymphoma, grade 2.

According to the MP calculator, FL grade 3 [9698/3] is a separate primary from FL grade 2 [9691/3]. Is the June 2011 diagnosis of FL grade 2 a new primary?

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

This case should be accessioned as a single primary per Rule M15. The histology is coded to 9698/3 [follicular lymphoma, grade 3] diagnosed in 2006. The 2011 diagnosis of follicular lymphoma, grade 2 [9691/3] is not a new primary.

Follicular lymphoma, grade 2 [9691/3] is listed under the Same Primaries section of the Heme DB for 9698/3 [follicular lymphoma, grade 3]. To confirm this, Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries because none of the rules from 1-14 apply. Per the calculator, these histologies represent the same 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