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20200007 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is simultaneously diagnosed with systemic mastocytosis and chronic myelomonocytic leukemia (CMML-0) on a single bone marrow biopsy? See Discussion. |
The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that, However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2. |
Abstract a single primary when the diagnosis is systemic mastocytosis with an associated clonal hematogoical non-mast cell lineage disease (SM-AHNMD) (9741/3). However, if the patient has a previous history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia, abstract the SM-AHNMD as a second primary as stated in the Heme DB. SINQ 20130172 represents a single primary as there is no mention of a prior history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia. |
2020 |
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20120044 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient is diagnosed with acute monocytic leukemia in 2009 and in 2011 has biopsy confirmed granulocytic sarcoma of the cerebellum? See Discussion. |
Is this a recurrence of the patient's leukemia? In 2011, the patient is found to have several masses in the cerebellum, biopsy confirmed granulocytic sarcoma. The physician stated this is an "extramedullary relapse of leukemia." The bone marrow biopsy in 2011 was negative.
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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accession a single primary per Rule M3. Code histology to 9891/3 [acute monocytic leukemia] diagnosed in 2009 and primary site to C421 [bone marrow].
Per Rule M3 a single primary is reported when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Histology 9891/3 [acute monocytic leukemia] is listed as one of the histologies in the "same lineage." Myeloid sarcoma (9930/3) diagnosed simultaneously with or after acute myeloid leukemia (9861/3) or another leukemia of the myeloid lineage (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3).
NOTE: Under the Alternate Names section of the Heme DB, granulocytic sarcoma is a synonym for myeloid sarcoma.
Per PH10, code the primary site C421 [bone marrow] and code the histology acute myeloid leukemia, NOS (9861/3) or any of the specific AML histologies (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3) when the diagnosis is myeloid sarcoma (9930/3) AND there is a simultaneous or previous diagnosis of acute myeloid leukemia.
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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20130113 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient diagnosed and treated for multiple myeloma is subsequently diagnosed with multiple large plasmacytomas involving the scalp and thorax? See Discussion |
The patient was diagnosed with multiple myeloma, underwent treatment and subsequently was in remission. The patient later presented with lesions on the scalp and thorax lesions. The final diagnosis on the pathology report for the scalp lesion was multiple myeloma with plasmablastic transformation (high grade). The physician states this is an aggressive, recurrent multiple myeloma with multiple large plasmacytomas involving the scalp and thorax. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Accession a single primary, multiple myeloma [9732/3] per Rule M2. The multiple myeloma is in an advanced stage when plasma cells are being deposited on the scalp and thorax. Clinically, those plasma cells are rightly called plasmacytomas by the physician. However, the patient has a late-stage multiple myeloma causing the plasma cells/plasmacytomas. Note that under the myeloma Recurrence and Metastases section of the Heme DB it indicates that extramedullary involvement (e.g., the scalp and thorax involvement) usually indicates advanced disease. Therefore, this scenario represents a case of a single histology that is accessioned as a single primary per Rule M2. 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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20130138 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a 2010 diagnosis of a preleukemic condition is subsequently diagnosed in 2012 with a specific leukemia that is not listed as a transformation? See Discussion. |
10/02/10 bone marrow biopsy showed myelodysplastic syndrome, unclassified [9989/3]. 6/19/12 bone marrow biopsy showed chronic myelomonocytic leukemia (CMML-2) [9945/3]. CMML-2 is not listed as an acute neoplasm for MDS. Is this the same disease? Per the pre-2010 rules, this would be the same disease. The current Heme DB indicates these are separate primaries. |
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, myelodysplastic syndrome (MDS) [9989/3] diagnosed 10/2/10 and chronic myelomonocytic leukemia (CMML-2) [9945/3] diagnosed 6/19/12 per Rule M15. Per Rule M15, use the Multiple Primaries Calculator when rules M1-M14 do not apply. When myelodysplastic syndrome (MDS) became reportable, the rules in effect at that time resulted in MDS often being the only diagnosis reported when both MDS and a leukemia were diagnosed. Statistics for some leukemias were impacted. Now we report both the MDS and the leukemia for greater accuracy. 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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20130109 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a 2004 diagnosis of acute myeloid leukemia is followed by a 2013 diagnosis of myeloid sarcoma? See Discussion. |
Patient was diagnosed in 2004 with acute myeloid leukemia [9861/3] and treated with chemotherapy and transplant. Now the patient has a biopsy of an umbilical mass that is positive for myeloid sarcoma (granulocytic sarcoma) [9930/3]. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accession a single primary, acute myeloid leukemia [9861/3] diagnosed in 2004 per Rule M3.
When there is a myeloid sarcoma diagnosed simultaneously or after a leukemia of the same lineage, it is a single primary. The myeloid sarcoma is actually caused by the AML progressing. The myeloid cells in the bone marrow or blood literally "seep out" and implant in the tissue.
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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20210002 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed with therapy-related myelodysplastic syndrome (t-MDS) (9920/3) in 2015 followed by a 2020 diagnosis of myelodysplastic syndrome, NOS (MDS, NOS) (9989/3)? See Discussion. |
Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified. MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS. The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary. |
Abstract separate primaries using Rule M15 of the Hematopoietic and Lymphoid Neoplasms (Heme) Manual. The Heme Database states: Excluded from this category are progression of myeloproliferative neoplasms (MPNs) and evolution of primary MDS or primary MDS/MPN to acute myeloid leukemia (AML); in each of these latter cases evolution to AML is part of the natural history of the primary disease and it may be impossible to distinguish natural progression from therapy-related changes. There is no indication of transformation. |
2021 |
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20200012 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed with myelodysplastic syndrome (MDS) with ring sideroblasts in 2005, and stated to have progressed to high risk disease/early evolving acute myeloid leukemia (AML) in 09/2019? See Discussion. |
The bone marrow biopsy proved bone marrow with blasts comprising 15-19%. Neither the pathologist nor the physician specifically diagnosed this as AML, calling this only high risk disease or early evolving AML prior to starting the patient on Vidaza. No further information can be obtained from the pathologist or the physician for this case. Should this early evolving AML be accessioned as an additional primary per Rule M10, or is this the same MDS that is now high risk as the blast count is up to 19%, but has not yet reached the threshold of 20% blasts usually required for AML per the Hematopoietic and Lymphoid Neoplasm Database? |
Abstract a single primary as we do not abstract early/evolving AML. This is still one primary until there is a confirmed diagnosis of AML. |
2020 |
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20130035 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a subsequent diagnosis of diffuse large B-cell lymphoma (95%) and follicular lymphoma, grade 3 (5%) is made following an original diagnosis of low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL) ? See Discussion. |
In 2011, patient presented with a large mesenteric mass, numerous other smaller mesenteric lymph nodes, moderate retroperitoneal and extensive iliac chain adenopathy greater on right; small inguinal nodes are also present mostly on right side and splenomegaly per the CT scan. Abdominal pelvic mass needle biopsies showed low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL). The patient was treated with R-CVP with unknown response. In June 2012, patient presented again for laparoscopy and lymph node biopsy for stated recurrence of lymphoma found on CT scan. A large mass was seen in mesentery of bowel. Abdominal mass biopsy showed diffuse large B-cell lymphoma (DLBCL). Abdominal mass #2 excisional biopsy showed diffuse large B-cell lymphoma, 95%, and follicular lymphoma grade 3, 5%. The majority of the tumor is now DLBCL. |
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, diffuse large B-cell lymphoma diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s). Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was "most consistent with follicular lymphoma." The term "consistent with" is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011. 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. Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is a new primary per rule M12. |
2013 |
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20130040 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a patient has a history of chronic myeloid leukemia diagnosed in 1993 followed by a diagnosis of acute myeloid leukemia arising in chronic myelogenous leukemia, blast phase? See Discussion. |
12/1993 Bone marrow biopsy: Chronic myeloid leukemia t(9;22) (q34;q11).
09/2011 Bone marrow biopsy: Abnormal cytogenetic & FISH support persistent involvement by chronic myelogenous leukemia.
12/2011 Peripheral blood, flow cytometry: Involvement by acute myeloid leukemia arising in chronic myelogenous leukemia (CML, blast phase, 30% blasts by manual diff.).
Is the 12/2011 diagnosis a new primary? If not, why don't Rules M8-M13 apply when the Heme DB Abstractor Notes section for CML indicates that when there is a chronic, accelerated and blast phase that develops later in the course of the disease, change the histology code to the more specific diagnosis?
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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as a multiple primary: chronic myelogenous leukemia t(9;22) (q34;q11) [9863/3] diagnosed in 1993 and acute myeloid leukemia [9861/3] diagnosed in 2011 per Rule M15.
Use the diagnosis date to determine the appropriate manual and rules to follow to determine the histologies for this case. To determine the histology of the 1993 diagnosis, use the ICD-O-2. The Heme Manual & DB will be used to determine the number of primaries and the histology of the 2011 diagnosis of AML.
Rules M8-M13 in the Heme Manual cannot be applied to this case because no transformation occurred. CML does not transform to another neoplasm.
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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20200005 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what M rule applies when a patient is diagnosed with both plasmablastic lymphoma and at least one plasmacytoma? See Discussion. |
The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019. In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma. Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma. Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process. |
Abstract multiple primaries using the Heme and Lymphoid Rule M15. The Multiple Primaries Calculator shows that the plasmablastic lymphoma (9735/3) and extraosseus plasmacytoma (9734/3) are separate primaries. We also checked with our expert pathologist who concurs as the spinal lesion was not reviewed to prove that it is plasmablastic lymphoma, therefore, the diagnosis as per pathology remains plasmacytoma. |
2020 |
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