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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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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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20130085 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient was treated in 1999 with Vidaza for myelodysplastic syndrome and had a recent biopsy that demonstrated a transformation to acute myeloid leukemia? |
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, acute myeloid leukemia [9861/3]. MDS diagnosed prior to 1/1/2001 is not a reportable disease process. However, because MDS is currently a reportable disease process, it must be considered when trying to determine whether the AML represents a separate primary.
Rule M2 does not apply to this case because more than one histology is mentioned in the scenario. According to the Heme DB, MDS can transform to AML. Rules M8-M13 apply to cases involving transformation. In this case, Rule M10 applies because the patient was diagnosed with a chronic neoplasm (myelodysplastic syndrome) followed greater than 21 days later by an acute neoplasm (AML). 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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20130031 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a plasmacytoma of the intervertebral disc is diagnosed in 2010 followed by a diagnosis of immature plasma cell myeloma by a right hip biopsy in 2011? See Discussion. |
The patient was diagnosed with intervertebral disc plasmacytoma and had radiation therapy to the pelvic bones in 2010. In 2011 (more than 21 days later) a right hip biopsy revealed immature plasma cell myeloma. There is clinical documentation that this is progression into myeloma. Per the Heme DB (Primary Site(s) and Definition sections) and Rule PH30, in the Heme Manual, the primary site is coded to C421 [bone marrow] and the histology is coded 9732/3 [plasma cell myeloma] when there is a clinical diagnosis of multiple myeloma and/or there is no documentation of a bone marrow biopsy or the results are unknown. This patient did have a bone marrow biopsy that indicates there are an increased plasma cells present; plasma cells represent less than 10%. The skeletal survey and bone scan did not reveal any further lesions. Is this progression of disease because there is only one lesion in the right hip 8 months after the diagnosis of plasmacytoma? Or is this a second primary based on the right hip biopsy that showed plasma cell myeloma and the physician's documentation of disease progression? Plasmacytomas are usually single lesions. Would this disease process have multiple lesions if they are diagnosed at different times? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. This case is accessioned as two primaries: Plasmacytoma diagnosed in 2010 and plasma cell myeloma diagnosed in 2011 per Rule M10. The patient has a diagnosis of a solitary plasmacytoma (chronic neoplasm) followed by a diagnosis of plasma cell myeloma (acute neoplasm) diagnosed greater than 21 days later. The physician is calling this a progression to plasma cell myeloma even though the bone marrow has less than 10% plasma cells, take this statement as progression or a clinical diagnosis of plasma cell myeloma. 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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20130002 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned, and what is the year of diagnosis, when the patient was initially diagnosed with poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] in 1991, followed by multiple recurrences and transformations? See Discussion. |
5/1991 Left groin biopsy: Poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3]. Subsequently, the patient had multiple recurrences. 7/1/08 Left axillary biopsy: Disease transformed to malignant lymphoma, large B-cell and a small focus of follicular lymphoma. Patient was followed until there was no evidence of disease. 4/22/10 Left axillary biopsy: Recurrence of follicular lymphoma, grade 1. No large cell component was found. The bone marrow biopsy was negative for lymphoma. The patient was on observation. 11/02/10 MD note indicates the disease progressed to follicular lymphoma, grade 3. No large cell component was identified. The patient clinically has no evidence of disease on maintenance Rituxan. |
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, non-Hodgkin lymphoma (previously called poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell) [9591/3] diagnosed in 1991. Determining the number of primaries is based on the rules in effect at the time of each diagnosis. The original lymphoma was diagnosed in 1991 and the first transformation to follicular lymphoma in 2008. The pre-2010 rules for coding histology and determining multiple primaries must be applied first because the rules changed for diagnoses occurring 2010 or later. Per the Single Versus Subsequent Primaries Table, poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] is the same primary as follicular lymphoma [9690]. The Heme DB and Manual are used to confirm that the 2010 recurrences of follicular lymphoma, grade 1 [9695/3], and follicular lymphoma, grade 3 [9698/3], are the same primary according to the Heme Calculator check required per Rule M15. Per the Heme DB page, the diagnoses follicular lymphoma, grade 3 [9698/3] and follicular lymphoma, grade 1 [9695/3] are comparable to follicular lymphoma [9690] as stated in the section. 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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20130167 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a 2013 diagnosis of right leg skin nodules, consistent with plasmacytoma/plasma cell myeloma, follows a 3/20/07 biopsy diagnosis of multiple myeloma? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Abstract this case as a single primary. Code the histology to 9732/2 [multiple myeloma]. Review the Abstractor Notes section in the Heme DB for multiple myeloma. It states that in multiple myeloma there is generalized bone marrow involvement and that extramedullary involvement is diagnostic of advanced disease. This is a case of advanced multiple myeloma. |
2013 | |
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20110017 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a patient originally diagnosed with CLL is subsequently diagnosed several months later on a bone marrow biopsy with Richter's syndrome that transformed into a large cell lymphoma? See Discussion. |
Per reviewed resources, the described condition is rare. Should the histology remain CLL or be changed to large cell lymphoma? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm. Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3]. 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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20110055 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted for a bone marrow biopsy diagnosis of "acute myeloid leukemia (non-M3 type; favor FAB M1), probably arising in myelodysplastic syndrome;" and flow cytometry studies performed the same day were consistent with acute myeloid leukemia? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Ambiguous terminology is NOT used to determine histology for hematopoietic or lymphoid neoplasms. Therefore, the comment that the AML is "probably" arising in myelodysplastic syndrome is not used to determine the histology code. The term "favor" is also an ambiguous term and cannot be used to code histology.
This is a single histology per M2, abstract a single primary when there is a single histology. The histology is coded to 9861/3 [acute myeloid leukemia, NOS]
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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20110018 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted for a case with a history of follicular lymphoma, grade 2 and a subsequent splenectomy diagnosis of diffuse large B-cell lymphoma? See Discussion. |
The patient was treated over a period of time for follicular lymphoma, grade 2. The oncologist thought the spleen was congested and removed it. The diagnosis was DLBCL. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.. This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is follicular lymphoma, grade 2 [9691/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute 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. |
2011 |
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20100049 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted when a lymph node biopsy reveals "malignant lymphoma, peripheral T-cell type, with some features of angioimmunoblastic T-cell lymphoma and follicular T-cell lymphoma," the bone marrow biopsy was negative for involvement, and the oncologist states this patient has "peripheral T-cell lymphoma"? See Discussion. |
CT scan showed retroperitoneal and inguinal adenopathy. Right inguinal lymph node biopsy revealed "malignant lymphoma, peripheral T-cell type, with some features of angioimmunoblastic t-cell lymphoma and follicular t-cell lymphoma." Flow cytometry studies showed no evidence of B-cell lymphoma and atypical CD3+/CD10+/CD7-/CD4+/CD56+ T cells are detected (19%). The bone marrow biopsy was negative for involvement. Patient was staged as Stage II Peripheral T-Cell lymphoma by the oncologist and started chemotherapy. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the oncologist's clinical diagnosis of peripheral T-cell lymphoma.
The definition for this neoplasm is "A large group of lymphomas which we collectively refer to as peripheral T-cell lymphomas with the optional addition of "unspecified" to emphasize that these cases do not belong to any better defined entities. Attempts to distinguish between them on morphological basis have met with poor reproducibility."
Per the Abstractor Notes in the Heme DB: Patients present with peripheral LN involvement. The diagnosis of PTCL, NOS is made ONLY when other specific entities have been explored.
This fits your case; attempts to find a more specific disease (flow cytometry; BM biopsy) were negative and gave no further information that could be used to assign a more specific classification.
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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