| Report | Question ID | Question | Discussion | Answer | Year |
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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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20061037 | Multiple Primaries/Histology--Lymphoma: If a gastric biopsy demonstrates large B cell lymphoma arising in a low grade MALT lymphoma, how many tumors should be abstracted and how should the histology field(s) be coded? See Discussion. | Final path for gastric biopsy on 12/2005 is "consistent with malignant lymphoma" and Micro says "morphologic findings consistent with MALT lymphoma and an increased proportion of large atypical cells is concerning for large cell transformation. However, since the large cells are present only focally, a definitive diagnosis of large cell lymphoma cannot be rendered" A second gastric biopsy a week later said: Final Path: Diffuse large B cell lymphoma arising in low grade MALT lymphoma. Micro says: "Compared to patient's previous biopsy...the current specimen contains a higher percentage of large atypical cells which stain positively for CD79a, a B cell marker. The morphologic and immunohistochemical findings are consistent with a large B cell lymphoma arising in a low grade MALT lymphoma." These are different primaries according to the table of single versus subsequent primaries of lymphatic and hematopoietic diseases. |
For cases diagnosed prior to 1/1/2010: This is one primary. Code as 9699 [Marginal zone B-cell lymphoma, NOS]. The first biopsy was not conclusive. The biopsy one week later was more definitive. The reports are describing a difference between specimens, not a difference in disease. According to the WHO classification, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is an extranodal lymphoma with B-cells, cells resembling monocytoid cells, small lymphocytes and scattered immunoblast and centroblast-like cells. For cases diagnosed 2010 forward, 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. |
2006 |
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20061010 | Multiple Primaries/Histology--Lymphoma: If an oral mucosa, right hard palate biopsy contains a composite lymphoma [low-grade follicular + chronic lymphocytic leukemia], how many tumors should be abstracted and how should the histology field(s) be coded? | For cases diagnosed prior to 1/1/2010:This is one primary. Assign code 9590 [Malignant lymphoma, NOS]. This is a composite lymphoma. Code to lymphoma when there is any solid tumor (in lymph nodes, tissue, etc.) Code to lymphoma, NOS since this is not purely follicular and there is no code for composite lymphoma. For cases diagnosed 2010 forward, 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. |
2006 | |
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20021158 | Multiple Primaries/Histology--Lymphoma: What is the primary site(s) for a patient who had a lymph node biopsy with the histology of "large B cell lymphoma arising in the setting of low grade B cell lymphoma c/w marginal zone B cell lymphoma with plasmacytic features"? See discussion. | This patient also had a bone marrow biopsy that demonstrated "low grade B cell lymphoma." Per the clinician, "Pt with discordant lymphoma. We will be approaching his lymphoma as two different diseases. The large B cell had cleared after chemotherapy and radiation therapy. The low grade lymphoma is incurable." | For cases diagnosed prior to 1/1/2010: Code as two primaries with each arising in lymph nodes [C77._]. The histology for the first primary is 9699/3 [marginal zone B cell lymphoma]. The histology for the second primary is 9680/3 [large B cell lymphoma]. For cases diagnosed 2010 forward, 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. |
2002 |
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20021061 | Multiple Primaries/Histology--Mycosis Fungoides/Cutaneous T cell Lymphoma: Physicians often use the terms cutaneous T cell lymphoma (CTCL) and mycosis fungoides interchangeably and yet the SEER Single versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table indicates that these 2 diagnoses represent separate primaries. Do these cases represent one primary? If so, what histologic type should they be coded to? | For cases diagnosed prior to 1/1/2010:The patient does not have two different malignancies. Code the Histology field to 9700/3 [mycosis fungoides], the specific type of cutaneous T cell lymphoma. Mycosis fungoides is one of several types of cutaneous T cell lymphoma. Physicians often refer to mycosis fungoides by the "umbrella term" cutaneous T cell lymphoma.
The table indicates that the broad category of "T/NK-cell NHL" (which includes CTCL) and mycosis fungoides are presumably separate primaries because several entities are included in that broad category. In the specific case cited above, one entity (CTCL) within the broad category (T/NK-cell NHL) and mycosis fungoides are not separate primaries. For cases diagnosed 2010 forward, 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. |
2002 | |
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20110039 | Multiple primaries/Primary site--Heme & Lymphoid Neoplasms: What are the primary sites and how many primaries are abstracted, when 2004 diagnosis of grade 2 follicular lymphoma of the bilateral breasts is subsequently diagnosed with a 2010 diagnosis of diffuse large B-cell lymphoma (40%) and grade 3a follicular lymphoma (60%) of a left arm nodule? See Discussion. | Follicular lymphoma was diagnosed 7/2004, grade 2 per biopsy of the bilateral breasts. Bone marrow biopsy was positive for lymphoma involving 10% of bone marrow. Imaging showed extensive lymphadenopathy mainly in abdomen/pelvis with an 8 cm mass in the right pelvis. Smaller lymph nodes were noted in the left periaortic region and also some small precarinal lymph nodes. This was a stage IVA lymphoma. The patient had six cycles of CHOP/R with an excellent response. Per the clinician's notes on 12/2005, there was no evidence of recurrence or no sign of active disease. The plan was to follow up with the patient in 6 months.
08/22/06 imaging showed new disease in the bilateral chest wall. 8/2006 bilateral breast nodule biopsies, are positive for grade 1-2 follicular lymphoma. The patient was treated with Rituxan. Per clinician's 3/2007 note, no active disease is noted. Patient was regularly followed with no evidence of disease until 10/2010. At that time, the patient had a left arm nodule biopsy which was positive for diffuse large B cell lymphoma (40%) CD positive and grade 3a follicular lymphoma (60%). RICE was recommended due to "transformation" per oncologist. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M10, this case should be accessioned as two primaries when a neoplasm is originally diagnosed as a chronic neoplasm (is follicular lymphoma (FL), grade 2) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.
Code the histology for the first primary to 9691/3 [follicular lymphoma (FL), grade 2] and the primary site to bilateral C509 [breast, NOS]. FL can start as an extranodal disease; breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes.
Per Rule M4, abstract the 2010 disease process as a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Per Rule PH11 the primary site is coded to C779 [lymph nodes, NOS] and the histology is coded to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Rule PH11 states one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.
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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