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Report Produced: 03/06/2025 22:28 PM

Report Question ID Question Discussion Answer Year
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
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
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
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
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
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
20120010 Multiple primaries/Behavior--Ovary: What is the diagnosis date and histology for the primary(ies) abstracted for a patient with a mucinous cystic borderline tumor of the ovary in 2003 and a metastatic ovarian adenocarcinoma in 2011? See Discussion.

The 2011 pathology report: Spine at L3 biopsy: metastatic adenocarcinoma. Per addendum: Prior total abdominal hysterectomy specimen from 2003 was reviewed and showed an ovarian mucinous cystic tumor of borderline malignancy which has a similar morphology to the invasive adenocarcinoma seen on current specimen.

Abdominal tissue and omental biopsy: invasive and non-invasive glandular implants compatible with origin from ovarian mucinous borderline tumor.

The final diagnosis per radiation oncologist was, "recurrent ovarian cancer."

This is a single primary. The diagnosis date is coded to 2003 and the histology is mucinous cystadenocarcinoma [8470/3]. The bone, abdominal tissue and omentum are metastatic sites. The MP/H Rules do not apply to metastases.

This is a case where an invasive or microinvasive element was missed in the original pathology. Because the entire tumor was not sectioned and placed on slides, the pathologist used their expertise when sectioning and selecting tissue to be examined. It is not a matter of poor judgment, just a fact that it is impossible to review the tissue from the entire tumor. The behavior must be changed to malignant [/3].

2012
20081083 Multiple primaries--Lymphoma: Is mediastinal large B-cell lymphoma followed by classical Hodgkin lymphoma reportable as one or two primaries? See Discussion. Diagnosed 06/06/2006 with mediastinal large B-cell lymphoma, 9679/36. On 05/10/2007, another mediastinal lymph node biopsy done and the diagnosis was recurrent malignant lymphoma, classical Hodgkin's. A Hematopatholgy Consultant states, "it appears likely that the preceding mediastinal diffuse large B-cell lymphoma and the current classical Hodgkin's lymphoma are clonally related and represent different manifestations of the same entity. One might also place this in the spectrum of 'mediastinal gray zone lymphoma' described by Dr. Jaffee and colleagues."

For cases diagnosed prior to 1/1/2010:Report this case as two primaries. Report non-Hodgkin lymphoma followed by Hodgkin lymphoma as separate primaries.

According to the Table of Single and Subsequent Primaries for Hematologic Malignancies, mediastinal large B-cell lymphoma and Hodgkin disease are "D" - Different disease processes.

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.

 2008
20100077 Multiple primaries--Heme & Lymphoid Neoplasms: Would it be correct to apply rule M5 for a recurrence and abstract a single primary when a patient has a history of Hodgkin disease diagnosed in 2005 followed by a diagnosis of "recurrent Hodgkin and EBV+ Diffuse large B-cell lymphoma" in 2010? See Discussion.

Does Rule M5 only apply if both diseases are present at the original diagnosis, or does it also take into account a recurrence of an old disease? The answer to this question makes a difference between stopping at rule M5 and abstracting as one disease, or going on to rule M15 to query the Hematopoietic Database to determine whether the patient has two separate primaries.

Example: Patient had Stage II Hodgkin disease in 2005 (all lymph nodes above diaphragm, supraclavicular LN biopsied at diagnosis), treated and patient achieved complete remission. In 2010, the patient is admitted for suspected recurrence. A supraclavicular lymph node biopsy showed, "Recurrent Hodgkin" AND "EBV+ Diffuse Large B-cell Lymphoma," both in the same lymph node. Applying rule M5, this is a single primary and states not to query the DB. However, this doesn't seem correct as it does not account for the new DLBCL.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

You must first determine the histology codes for each occurrence of lymphoma. The 2005 diagnosis was stated to be Hodgkin disease (NOS) [9650/3]. The 2010 diagnosis was Hodgkin and EBV + diffuse large B-cell lymphoma (two histologies). Per Rule M5 the 2010 diagnosis is a single primary because the Hodgkin and the non-Hodgkin (DLBCL) were simultaneously present in the same lymph node. Per Rule PH14, a Hodgkin and non-Hodgkin simultaneously present in the same location should be coded to 9596/3 [B-cell lymphoma, unclassifiable].

Ultimately, there is a diagnosis of 9596/3 in 2010 that followed a diagnosis of 9650/3 in 2005. Per Rule M15, use the Multiple Primary Calculator to determine the number of primaries, which indicates the 9596/3 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. https://educate.fhcrc.org/LandingPage.aspx.

 2010
20140010 Multiple primaries--Heme & Lymphoid Neoplasms: Is this one primary or two? Follicular lymphoma grade 1 (9695/3) on 8/23/12 from an abdominal lymph node. On 1/6/14 an abdominal lymph node biopsy showed diffuse large b cell lymphoma arising from high grade follicle center cell lymphoma. Patient has been on observation.

1st primary, 8/23/12: Follicular lymphoma, grade 1 2nd primary, 1/6/14: Diffuse Large B Cell Lymphoma

Apply the multiple primary rules twice for this case. The 2012 diagnosis is follicular lymphoma. There are two histologies in 2014: diffuse large b cell lymphoma and follicle center cell lymphoma diagnosed at the same time in the same location. This is one primary per rule M4.

Then compare the 2012 diagnosis to the 2014 diagnosis.

Per the Hematopoietic Database, follicular lymphoma (all types) transforms to DLBCL. Per Rule M10, the DLBCL would be a second primary.

2014