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20130050 | Multiple Primaries/Primary site/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the primary site and histology for each if a 6/12/12 left shoulder mass specimen suspicious for large B-cell lymphoma is followed on 7/10/12 with three skin nodules excised from the back with a diagnosis of "composite lymphoma? See Discussion. | 6/12/12 Excisional biopsy left shoulder soft tissue mass: Suspicious for large B-cell lymphoma.
7/10/12 Excisional biopsy three skin nodules of back: "Composite lymphoma" - primary cutaneous anaplastic large cell lymphoma (CD3 pos, CD4 pos, CD30 pos, ALK neg with partial loss of CD5) and CONCURRENT cutaneous follicular center lymphoma (CD20 pos, PAX5 pos, BCL-6 pos, partially CD10 pos) and flow cytometry revealed results compatible with involvement by a lymphoproliferative disorder of T-cell lineage.
Per imaging performed, there was no involvement of lymph nodes or other organs.
Is the primary site C449 Skin, NOS and histology 9718/3 [Lymphoma, primary cutaneous anaplastic large cell] be correct? |
Code primary site to C445 [skin, back] and histology to 9718/3 [cutaneous anaplastic large cell lymphoma] .
Per Rule M6, abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location. For this case, there is cutaneous follicular (follicle) center lymphoma (9597/3) and cutaneous anaplastic large cell lymphoma (9718/3).
Per Rule PH22, code the primary site to the site or origin (skin, back) and the histology to the NHL with the numerically highest ICD-O-3 code. In this case, that would be 9718/3. |
2013 |
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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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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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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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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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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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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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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. | Updated May 2026 Rule M15 applies to this case which instructs you to use the Multiple Primaries Calculator. The result for 9695/3 and 9714/3 is "New Primary."
Abstract the anaplastic T cell lymphoma as a new primary. Code the histology to 9714/3 [Anaplastic large cell lymphoma, ALK-positive].
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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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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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