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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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20110143 | Multiple primaries--Heme & Lymphoid Neoplasms: How many and what primary site(s) are to be accessioned when biopsies of clavicular and neck skin lesions are both consistent with mycosis fungoides? See Discussion. |
Per the Heme DB and Manual, this is a single primary; however, per the MP/H Rules, this would be multiple primaries. Which rules apply to this case? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. When there is a question of whether the SEER MP/H Rules or Hematopoietic and Lymphoid Neoplasm Rules apply, check the histology and refer to the Case Reportability Instructions in the Hematopoietic and Lymphoid Neoplasm Manual. All ICD-O-3 morphology codes in the range 9590 - 9992 are included in the Hematopoietic Rules. Mycosis Fungoides [9700/3] is included in this range. Therefore, the SEER MP/H Rules do not apply to mycosis fungoides. This case should be accessioned as a single primary: mycosis fungoides [9700/3] of the skin, NOS [C449]. Per Rule M2 abstract a single primary when there is a single histology. Note that in the Primary Site(s) section of the Heme DB, it states the primary site must always be coded to skin (C440 - C449) for mycosis fungoides. Because the primary site is stated in this section of the Heme DB, it is not necessary to use the Primary Site Rules to determine the primary site. Code the primary site to C449 [skin, NOS] because the patient has multiple sites of skin involvement and there is no documentation indicating which subsite of skin was the origin of the mycosis fungoides. 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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20120021 | Multiple primaries--Heme & Lymphoid Neoplasms: How are the terms "chronic" and "acute" used to help determine the number of primaries to be abstracted and what rule applies when a diagnosis of diffuse large B-cell lymphoma is followed two years later by a diagnosis of follicular lymphoma, grade 3A of 3? See Discussion. |
7/31/08 Biopsy of the left supraclavicular lymph node diagnosed Stage IIIB DLBCL [9680/3] 10/14/10 Biopsy of a right supraclavicular lymph node diagnosed follicular lymphoma, grade 3A or 3 [9698/3]. Which multiple primary rule applies to determine the number of primaries to report? Is Rule M4 ignored? Does Rule M13 apply because follicular lymphoma normally transforms to DLBCL? Is this still a transformation because the follicular lymphoma came AFTER the DLBCL (the "acute" reverted to "chronic")? Or does Rule M15 apply, and the Multiple Primaries Calculator should be used to determine the number of primaries to report? Are "transformations" the acute phases of the more chronic disease? The Heme Manual and previous training sessions do not make this apparent. |
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 per Rule M13. Code the histology for the 7/31/08 diagnosis to 9680/3 [diffuse large B-cell lymphoma] and the code the histology for the 10/14/2010 diagnosis to 9698/3 [follicular lymphoma, grade 3A of 3]. Rule M13 applies to this case because the neoplasm was originally diagnosed in the blast or acute phase (DLBCL) and reverted to a less aggressive or chronic phase (follicular lymphoma) after treatment. Per the "Transformations to" section in the Heme DB for follicular lymphoma, grade 3 transforms to diffuse large B-cell lymphoma [9680/3]. This means that the follicular lymphoma is the chronic neoplasm and that DLBCL is the acute neoplasm. In this case, the chronic neoplasm was diagnosed after the acute neoplasm was diagnosed and treated (with chemotherapy). Do not Stop at Rule M4 because diffuse large B-cell lymphoma and follicular lymphoma (both NHL's) were not present in the same node(s) AT THE SAME TIME. 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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20160015 | Multiple primaries--Heme & Lymphoid Neoplasms: Could you please clarify Note 2 found in Rule M10, which is " 'Transformations to' (acute neoplasms) and 'Transformations from' (chronic neoplasms) are defined for each applicable histology in the database." Do the neoplasms being considered have to contain the words 'chronic' and/or 'acute'? |
Hematopoietic neoplasms that transform generally don't have 'chronic' or 'acute' as part of their preferred name. The 'chronic' and 'acute' designations are determined by the usual course of the neoplasm. Chronic neoplasms are generally slow growing while acute neoplasms grow fast and are more widespread. Not all Hematopoietic neoplasms transform. Each neoplasm that has the ability to transform has the transformations listed under the 'Transformations to' and/or 'Transformation from' sections in the Hematopoietic database.
For example, Diffuse large B-cell lymphoma (histology code 9680/3) has no histologies/neoplasms listed under 'transformations to.' This means that this neoplasm does not transform to any other neoplasm. There are multiple histologies/neoplasms listed under 'Transformations from' indicating the neoplasms listed under the Transformations from are the chronic neoplasms, and DLBCL is the acute neoplasm. If DLBCL (9680/3) occurs at the same time, within 21 days, or greater than 21 days of any of the histologies listed under 'Transformations From,' rules M8-M13 apply. If DLBCL (9680/3) occurred at the same time as a neoplasm not listed in the Transformations sections, the acute and chronic rules do not apply. |
2016 |
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