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20110147 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How is the histology coded when no bone marrow examination is performed but the peripheral blood flow cytometry listed several differential diagnoses and the physician states the diagnosis is small lymphocytic lymphoma? See Discussion. | The peripheral blood flow cytometry results state, "findings consistent with a small mature B-cell neoplasm, differential - marginal zone lymphoma, lymphoplasmacytic lymphoma, and atypical CLL." The physician states the diagnosis is "SLL." No bone marrow examination or CT scan was done to assess whether the patient had lymphadenopathy.
Per Rule PH5, if the diagnosis is B-cell CLL/SLL and peripheral blood is involved, the histology is coded to B-CLL/SLL [9823/3]. Should the primary site and histology be coded to bone marrow [C421] and CLL/SLL [9823/3] per Rule PH5 despite the physician's diagnosis of SLL [9670/3]? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This is a single primary and the primary site and histology is coded as bone marrow [C421] and CLL/SLL [9823/3]. The code 9670/3 [malignant lymphoma, small B lymphocytes, NOS] used for SLL is now obsolete.
Per the Abstractor Notes section in the Heme DB indicates that SLL is, "usually associated with CLL and coded CLL/SLL 9823/3. Small lymphocytic lymphoma (SLL) is almost identical to CLL. A somewhat arbitrary distinction is drawn between them based on the relative degree of marrow and nodal involvement and the numbers of circulating cells."
Per the Definition section in the Heme DB it states that, "CLL by definition involves blood and bone marrow at time of diagnosis." Check the PRIMARY SITE and MODULE RULE sections that indicate the primary site is C421, Rule PH5. Per this rule, code the primary site bone marrow (C421) and code the histology B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) [9823/3] when the diagnosis is B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) AND peripheral blood is involved (the bone marrow may also be involved).
This may appear to contradict the physician's diagnosis, but the 2008 WHO no longer codes CLL and SLL as separate neoplasms, rather one neoplasm, CLL/SLL, which reflects the actual neoplastic process. Those patients with SLL usually manifest CLL during the neoplastic process and those patients with CLL usually manifest SLL during the neoplastic process. WHO recommends coding to CLL/SLL rather than coding two primaries when the other neoplasm manifests.
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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20120050 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what histology codes apply if a patient has a 1998 diagnosis of essential thrombocythemia and a recent clinical diagnosis of secondary myelofibrosis? See Discussion. | The patient has a history of essential thrombocythemia (ET) since 1998. This has been treated daily with aspirin. A recent bone marrow biopsy was consistent with myeloproliferative disorder with excess blasts, marked extensive reticulin marrow fibrosis with osteosclerosis, excess blasts (11%) in the marrow aspirate and peripheral blood. JAK2 mutation was present in a small minority of cells. The physician stated patient was, "considered to have secondary myelofibrosis and was started on Jakafi." | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Appendix F, a secondary myelofibrosis is not a reportable case.
Secondary myelofibrosis is not listed as a synonym for primary myelofibrosis in the Heme DB. The term "secondary myelofibrosis" means that the myelofibrosis was caused by, in this case, the essential thrombocythemia.
The diagnosis "consistent with myeloproliferative disorder" is also not a new reportable diagnosis. "Myeloproliferative disorder" refers to a group of diseases (an NOS category) that includes essential thrombocythemia, which was originally diagnosed in 1998, prior to reportability for this disease type.
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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20120076 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what histology codes are used for a 2005 diagnosis of nodular histiocytic lymphoma followed by a 2012 diagnosis of B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma? See Discussion. | Per the history and physical, patient was diagnosed in 2005 with nodular histiocytic lymphoma and had chemo at that time. Now the patient presents with enlarged right axillary lymph nodes. A lymph node core biopsy confirmed B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia. Flow cytometry was most consistent with B-cell chronic lymphocytic leukemia. | 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 M15. Code the histology for the first primary to 9698/3 [nodular histiocytic lymphoma. Per the Alternate Names section in the Heme DB, this histology is synonymous with follicular lymphoma, grade 3. Code the histology for the second primary to 9823/3 [B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma].
Nodular histiocytic lymphoma does not transform into CLL/SLL (Transformations to), nor does CLL/SLL transform to nodular histiocytic lymphoma (Transformations from). Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries in this case because none of the rules from 1-14 apply. Per the calculator, the CLL/SLL is 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. |
2012 |
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20100071 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed in February 2010 with a plasmacytoma of the frontal skull followed by a diagnosis of smoldering myeloma by bone marrow biopsy? See Discussion. | The patient had a diagnosis of solitary plasmacytoma of the right frontal skull in 2/2010 that was totally resected (the cranial specimen final diagnosis was plasmacytoma). The patient received radiation. While undergoing radiation, the patient was seen by a medical oncologist who did a bone marrow biopsy that revealed 10-15% plasma cells, and was called smoldering myeloma. Watchful waiting was recommended. In 8/2010, the patient had multiple lytic lesions and began systemic treatment.
Per rule M15 and the Multiple primary calculator, 9731/3 [plasmacytoma] and 9732/3 [smoldering myeloma] is accessioned as two primaries. When the manual states, "Use the Hematopoietic Database to determine the primary site and histology when PH1-PH29 do not apply," does this mean to use the calculator not the database itself? By the old rules this was one primary. Did this change for cases diagnosed 1/1/10 and later? Which M rule is the correct rule to apply? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
The smoldering myeloma is a second primary per Rule M10. Accession as multiple primaries because this case was originally diagnosed as a chronic neoplasm (plasmacytoma)phase and there was a second diagnosis of an acute neoplasm (multiple myeloma) more than 21 days after chronic diagnosis. See note 1 which indicates, "This is a change from previous rules." Note that the MP rules and the MP calculator in the Heme DB agree.
When the rules tell you to go to the DB to determine the histology and primary site, you use the DB information. (Don't forget to check the Abstractor Notes). The multiple primaries calculator is used to determine the number of primaries to abstract. Always use the M rules before using the MP calculator.
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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20110065 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted when a skin (right thigh) biopsy is consistent with mycosis fungoides (cutaneous T-cell lymphoma)? See Discussion. | Applying rule M15 (multiple primaries calculator) indicates this is two primaries. Is this correct? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site to C447 [skin of lower limb] and code histology to 9700/3 [mycosis fungoides]. he pathologist wrote in parentheses that this was cutaneous (i.e. primary site is skin) and that it is a T-cell lymphoma (mycosis fungoides is a T-cell lineage). So the parenthetical statement was not a separate diagnosis; rather a general classification of the mycosis fungoides. "CTCL" is listed under the Alternate Names section of the Heme DB. CTCL is an abbreviation for cutaneous T-cell lymphoma. CTCL is a synonym for mycosis fungoides. This is a single primary per M2 which states to abstract a single primary when there is a single histology.
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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20100075 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned when a 1/27/10 bone marrow biopsy, FISH and cytogenetics reveals chronic myelogenous leukemia (CML), BCR/ABL positive, t(9;22)(q34;q11) and a 4/15/10 bone marrow biopsy reveals B-acute lymphoblastic leukemia (Blast phase of CML)? | 1/27/10 BM biopsy: CML BCR/ABL+ FISH positive for BCR/ABL and cytogenetics showing the t(9;22)q34q11.2 translocation. Treated with Imatinib. 4/15/10 BM biopsy: B-acute lymphoblastic leukemia (Blast phase of CML). Would the term "blast phase of CML" indicate the 4/15/10 bone marrow biopsy showed CML or would a new primary be abstracted with histology coded 9811/3 [B lymphoblastic leukemia/lymphoma, NOS]?
Applying rule M10, this is a new primary, but note 2 states transformations are defined in the Heme DB. The Abstractor Notes section indicates CML has three phases: chronic, accelerated, and the blastic phase or blast crisis. The accelerated phase can last weeks to months. In the chronic phase the involvement is usually limited to blood, bone marrow and spleen although the liver may be infiltrated. During the blastic phase, lymph nodes and tissue may be involved. The blastic phase is a disease progression from the chronic phase. The disease, however, remains the same histology, chronic myelogenous leukemia. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case represents a multiple primary per Rule M15 which states you are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14.
The histology for the first primary is coded to 9875/3 [chronic myelogenous leukemia, BCR-ABL1 positive].
The histology for the second primary is 9811/3 [B lymphoblastic leukemia/lymphoma, NOS] in the absence of further documentation that the B-ALL was also positive for the t(9;22) translocation.
The histology code 9806/3 [Mixed phenotype acute leukemia with t(9;22)(q34;q11.2); BCR-ABL1] cannot be used for the second primary because there is no documentation that the B-ALL diagnosed on 04/15/2010 also had the t(9;22) translocation and this histology cannot be used in patients ." Per the Definition section in the Heme DB, in order to use histology code 9806/3 "This leukemia meets the criteria for mixed phenotype acute leukemia (MPAL) in which the blasts also have t(9;22) translocation of BCR-ABL1 rearrangement. Some patients with chronic myeloid leukemia may develop or even present with a mixed blast phase that would meet criteria for MPAL; however, this diagnosis should not be made in patients known to have had CML."
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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20110105 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be reported for a bone marrow biopsy diagnosis of "lymphoproliferative disorder, small cell lymphocytic lymphoma/small cell lymphocytic leukemia consistent with marginal zone lymphoma"? | According to our hematopoietic/lymphoid neoplasm physician expert, abstract one primary with the histology code 9699/3 [marginal zone lymphoma]. The pathologist is using the expression "small lymphocytic lymphoma" in a descriptive manner (marginal zone lymphoma is comprised of small lymphocytes) rather than in a "diagnostic" manner. | 2011 | |
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20100022 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Is a 2010 diagnosis of ALK+ anaplastic T cell lymphoma following a 2008 diagnosis of follicular B cell lymphoma, grade 1 a new primary? If so, how is the histology coded? See Discussion. | A patient has a history of Stage 4 follicular B cell lymphoma, grade 1 [9695/3] diagnosed in 2008. The patient was treated with Adriamycin, Cytoxan, Rituxan, and Prednisone. In 2010, the medical oncologist states the patient has progression/recurrence of lymphoma with pathology that has changed to anaplastic T cell lymphoma ALK+. There was immunophenotyping, but there was no more specific diagnosis made. The patient died within 3 months. | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Abstract the anaplastic T cell lymphoma as a new primary. Code the histology to 9714/3 [Anaplastic large cell lymphoma, ALK-positive].
Rule M15 applies to this cases which instructs you to use the Multiple Primaries Calculator. The result for 9695/3 and 9714/3 is "New Primary."
Apply Rule PH30 to code histology which instructs you to use the Heme DB to determine the histology when rules PH1-PH29 do not apply. In searching the Heme DB for "anaplastic" the first term returned is Anaplastic large cell lymphoma, ALK-positive [9714/3].
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. |
2010 |
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20100081 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Should a single primary be accessioned with the histology coded 9732/3 [multiple myeloma] when a patient is diagnosed initially with a plasmacytoma on an excision and a single bone marrow biopsy showed only 4% plasma cells, then the subsequent workup led to a clinical diagnosis of multiple myeloma? See Discussion. | This patient had a plasmacytoma removed from the sphenoid sinus and was started on Dexamethasone. The patient had a bone marrow biopsy with 4% plasma cells. A statement in the hematology notes read, "it can increase the rate of false negative results with a bone marrow biopsy." The bone marrow biopsy was done 15 days after the surgery for the plasmacytoma.
Workup yielded the diagnosis of multiple myeloma. Per a statement in hematology notes, "I found her having 4% blasts, atypical plasma cells in the bone marrow biopsy and also lytic lesions involving the T7 and lucencies involving L4 and L5 vertebral bodies and also the upper sacrum. The PET-CT scan did not show significant metabolic activities in those lesions. The patient had a small amount of Bence-Jones in the urine and also an abnormal kappa to lambda ratio in the serum. The ratio was 12 to 1. The beta 2 microglobulin was 1.4. The albumin in the serum was 3.4. Based on that, the patient has been diagnosed with Durie-Salmon stage III in ISS stage II multiple myeloma."
The abstractor notes for multiple myeloma state that the diagnosis is made when the proportion of plasma cells in the bone marrow is 10% or greater. Should a diagnosis of MM be accessioned and coded when the bone marrow is less than 10% plasma cells, but a clinical diagnosis of MM is made? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accept the physician's diagnosis of multiple myeloma [9732/3]. Code the multiple myeloma as a single primary using rule M8 if there was only ONE positive biopsy. Code as multiple primaries (both the solitary plasmacytoma and multiple myeloma) using Rule M11 if there are TWO positive biopsies, one confirming the chronic neoplasm and the other confirming the acute neoplasm.
Per the Heme DB Abstractor Notes: The registrar DOES NOT CODE plasma cell myeloma based on the percentage of plasma cells. There must be a diagnosis of plasma cell myeloma. In addition, a clinical diagnosis of plasma cell myeloma may be made based on amyloidosis with associated renal impairment, anemia and/or hypercalcemia supported by radiologic evidence of multiple lytic bone lesions. he biopsy confirmed plasma cell malignancy (plasmacytoma) and the clinical workup confirmed 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. |
2010 |
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20071105 | Multiple Primaries/Histology--Lymphoma/Leukemia: How many primaries and what histologies are coded when a path diagnosis for a cervical/neck mass demonstrates classical Hodgkin's lymphoma on a background of chronic lymphocytic leukemia? | For cases diagnosed prior to 1/1/2010:Hodgkin disease and chronic lymphocytic leukemia are separate primaries according to our current instructions. Abstract and code them separately.
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. |
2007 |
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