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Report Produced: 10/20/2024 15:33 PM

Report Question ID Question Discussion Answer Year
20110155 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient shows evidence of "MDS as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera" 18 years after a diagnosis of "thrombocytosis and probable polycythemia that progressed to probable myelofibrosis"? See Discussion

Per consultation: an 83 year old patient started on hydroxurea 18 years ago following a diagnosis of thrombocytosis and probable polycythemia. It appears the polycythemia progressed to probable myelofibrosis. The possibility of an MDS needs to be considered.

Problem list: Polycythemia with probable progression to myelofibrosis or MDS.

Bone marrow biopsy two weeks later shows some progression of dysmegakaryocytopoiesis. Patient has evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera.

On follow-up visit six weeks later: Continue to manage patient with hydroxyurea.

An additional six months later: Diagnosis is polycythemia with thrombocytosis.

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. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].

The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.

The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."

According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.

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
20130044 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient has a biopsy diagnosis of extraosseous plasmacytoma followed fourteen days later with a diagnosis of multiple myeloma on imaging? See Discussion.

On 2/3/12 the patient was diagnosed via biopsy with an extraosseous (extramedullary) plasmacytoma filling both nasal cavities. On 2/16/12 a metastatic workup was performed and showed a lucent lesion in calvarium of Rt frontal bone. 2/17/12 radiation oncology consult states, "I believe this is most likely consistent with multiple myeloma." Subsequently, the 3/1/12 CT of the left shoulder showed a 3.6 cm lytic lesion of humeral head with cortical breakthrough consistent with metastasis or myeloma.

Per the Heme DB, extramedullary plasmacytoma can transform to multiple myeloma. Does that make this multiple primaries with the histologies coded to 9734/3 and 9732/3?

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, multiple myeloma [9732/3] diagnosed on 2/3/12 per Rule M8. Abstract the acute neoplasm as a single primary when both a chronic and acute neoplasm are diagnosed simultaneously or within 21 days AND there is documentation of only one positive tissue biopsy.

This patient only had a tissue biopsy of the nasal cavity proving the chronic neoplasm (extraosseous plasmacytoma). The acute neoplasm (multiple myeloma) was diagnosed clinically based on the scans.

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
20130051 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when biopsies of the left and right tonsils show diffuse large B-cell lymphoma and there is no other evidence of involvement? See Discussion. Scans are negative for lymphadenopathy and the bone marrow biopsy was benign. Radiation Oncology staged this as localized bilateral tonsil primary lymphoma.

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, diffuse large B-cell lymphoma [9680/3] of bilateral tonsils. Per Rule M2, a single histology is a single primary. Note 1 for Rule M2 states bilateral involvement of lymph nodes and/or organs is still a single 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.

2013
20120083 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient is diagnosed with follicular lymphoma, grade 3 in 2006 and is subsequently diagnosed with follicular lymphoma, grade 2 in 2011? See Discussion.

June 2006, the patient was diagnosed with follicular lymphoma, grade 3 by cervical lymph node biopsy and bone marrow biopsy. The patient refused treatment but was followed.

May 2007, the patient had another cervical LN biopsy with a diagnosis of follicular lymphoma, grade 2.

July, 2009, a neck mass excision was diagnosed as follicular lymphoma, grade 3.

June 2011, another neck lymph node was excised and diagnosed as follicular lymphoma, grade 2.

According to the MP calculator, FL grade 3 [9698/3] is a separate primary from FL grade 2 [9691/3]. Is the June 2011 diagnosis of FL grade 2 a new primary?

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 per Rule M15. The histology is coded to 9698/3 [follicular lymphoma, grade 3] diagnosed in 2006. The 2011 diagnosis of follicular lymphoma, grade 2 [9691/3] is not a new primary.

Follicular lymphoma, grade 2 [9691/3] is listed under the Same Primaries section of the Heme DB for 9698/3 [follicular lymphoma, grade 3]. To confirm this, Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries because none of the rules from 1-14 apply. Per the calculator, these histologies represent the same 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
20130060 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a diagnosis of bilateral extranodal orbital lymphoma when the same histology is present in both orbits?

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 lymphoma of bilateral orbits per Rule M2. Abstract a single primary when there is a single histology. Both orbits showed the same histology. Note 1 for Rule M2 states bilateral involvement of lymph nodes and/or organs is a single 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.

2013
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
20130212 Reportability--Heme & Lymphoid Neoplasms: Is a case reportable in which the pathology report is negative for plasmacytoma but a subsequent physician's clinical diagnosis is plasmacytoma?

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

This case is reportable if the patient was treated for plasmacytoma. When the physician calls the case plasmacytoma and treats the patient accordingly, report the case.

See Case Reportability Instructions #6: Report the case when there is a clinical diagnosis (physician's statement) of a reportable hematopoietic or lymphoid neoplasm.

Note 1: The clinical diagnosis may be a final diagnosis found within the medical record or recorded on a scan (CT, MRI for example)

Note 2: Report the case even if the diagnostic tests are equivocal. A number of hematopoietic neoplasms are "diagnoses of exclusion" in which the diagnostic tests are equivocal and the physician makes the clinical diagnosis based on the equivocal tests and the clinical picture. See the Heme DB for definitive diagnostic methods for the specific neoplasm being abstracted.

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
20100018 Reportability/Heme & Lymphoid Neoplasms--Hematopoietic, NOS: Is light chain disease reportable if it is treated with chemotherapy agents? See Discussion.

A patient was diagnosed in 2010 with light chain disease based on SPEP and urine testing. Bone marrow aspiration and biopsy were done. Flow cytometry, cytogenetic studies and FISH for plasma cell disorders are all normal. Medical oncologist states diagnosis is light chain disease. Patient was started on Revlimid, dexamethasone and Velcade.

In reviewing the case reportability instructions, this seems to fall under Instruction 1, note 1. Immunoglobulin deposition disease, preferred term for light chain disease, is coded as 9769/1. This is normally a non-reportable diagnosis, but the patient was given cancer-directed treatment. Would this case be accessioned using the above morphology code and primary site of bone marrow [C42.1]?

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

This case is not reportable. The histology is 9769/1 [light chain disease] in the Heme DB.

Light chains are produced in neoplastic plasma cells (multiple myeloma) and are called Bence-Jones proteins. The physician did the cytogenetic studies and FISH to rule out plasma cell disease. 50-60% of people with Light-chain deposition disease (LCDD) have an associated lymphoproliferative disorder, most commonly multiple myeloma. The remaining patients develop LCDD in the setting of progression of monoclonal gammopathy of unknown significance (MGUS) with no evidence of neoplastic plasma cell proliferation. This patient falls in this category, MGUS, which is not reportable.

2010
20130101 Reportability--Heme & Lymphoid Neoplasms: Is plasma cell dyscrasia, favor MGUS vs. smoldering myeloma reportable? See Discussion. The pathology report states, "plasma cell dyscrasia, favor MGUS vs. smoldering myeloma." The patient then died of a heart attack and no further information is available. If this is reportable, what histology code applies?

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

This case is not reportable. Neither plasma cell dyscrasia nor MGUS are reportable. Smoldering myeloma was given as a possible diagnosis, but never confirmed.

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
20120074 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient is diagnosed in 2004 with extranodal diffuse large B-cell lymphoma (DLBCL) of the stomach followed by a 2011 diagnosis of DLBCL involving abdominal lymph nodes? See Discussion. In 2004 a patient's extranodal DLBCL was treated with a partial gastrectomy at another facility. A recurrence of DLBCL was diagnosed in 2011 by a fine needle aspiration of abdominal lymph nodes. The patient presented to this facility for chemotherapy.

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

This case is accessioned as a single primary. Code the histology to 9680/3 [diffuse large B-cell lymphoma] and diagnosis date to 2004. Per Rule M2, abstract as 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.

2012