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Report Produced: 11/26/2024 19:45 PM

Report Question ID Question Discussion Answer Year
20100083 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient with a longstanding history of follicular cell non-Hodgkin lymphoma followed by a 2010 diagnosis of "B-cell lymphoma with prominent large cell component, compatible with primary cutaneous follicle center cell lymphoma"? See Discussion.

Patient has a history of follicular cell non-Hodgkin lymphoma dating back to the 1990s. The patient was treated with chemotherapy and bone marrow transplantation, radiation and rituximab. The patient had no evidence of recurrence. In April 2010 a lesion appeared on the side of the scalp above the left ear with a diagnosis of "B-cell lymphoma with prominent large cell component, compatible with primary cutaneous follicle center cell lymphoma." The oncology diagnosis is "primary cutaneous follicle center lymphoma."

Would the Multiple Primaries Calculator be used in this case?

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

Accession two primaries per Rule M15. Per the Multiple Primaries Calculator, primary cutaneous follicle center lymphoma [9597/3] following a diagnosis of follicular lymphoma, NOS [9690/3] is a new primary.

While the pathologic diagnosis was B-cell lymphoma "compatible with" primary cutaneous follicle center cell lymphoma and ambiguous terms cannot be used to identify a more specific histology, the physician confirmed the more specific diagnosis without ambiguous terminology. Therefore, this diagnosis should be coded.

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
20110053 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient with a several month history of refractory anemia with excess blasts (RAEB), that may or may not have been treated, who now presents with a bone marrow biopsy that is compatible with acute myeloid leukemia?

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

Per Rule M10, abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. Two primaries should be accessioned for this case: refractory anemia with excess blasts (RAEB) [9983/3] (a chronic neoplasm), and acute myeloid leukemia [9861/3] (an acute neoplasm).

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
20100087 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for one patient with history of marginal zone lymphoma initially diagnosed in 1994, followed by a 2010 diagnosis of large B-cell lymphoma and another patient with both B-cell chronic lymphocytic leukemia/small cell lymphoma (CLL/SLL) and diffuse large B-cell (DLBCL) in 2009? See Discussion.

Case 1 - Patient has a history of marginal zone lymphoma diagnosed in 1994 with recurrences in 2007 and 2009. The patient now presents for a bone marrow biopsy in May 2010 and is found to have large B-cell lymphoma, transformation. The first primary, marginal zone lymphoma, falls under the 2009 rules and the second primary, large B-cell lymphoma, falls under the 2010 and forward rules?

Case 2 - Patient was diagnosed with B-cell CLL/SLL and a DLBCL in 2009. If the 2009 rules only apply, these are a single primary. If the patient is admitted and treated in 2010 are the rules still based on the diagnosis date?

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

Case 1: Accession two primaries per Rule M10 when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The histology for the first primary is 9699/3 [marginal zone lymphoma] represents a chronic neoplasm and the second primary is 9680/3 [diffuse large B-cell lymphoma] is an acute neoplasm which was diagnosed more than 21 days after the first primary.

Case 2: Do not use the Heme DB and Manual rules for this case. Both diagnoses were made prior to 2010. The Heme DB and Manual are only effective for cases diagnosed 1/1/2010 and forward. Use the ICD-O-3 Hematopoietic Primaries Table to determine the number of primaries for this case. Per the Table, a second diagnosis of DLBCL [9680/3] following a diagnosis of CLL/SLL [9823/3] is one 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.

2010
20130111 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a 2008 diagnosis of extralymphatic follicular lymphoma in the breast is subsequently diagnosed in 2011 with ocular follicular lymphoma? See Discussion.

The patient was diagnosed with follicular lymphoma in the breast in 2008. Per notes, there was no evidence of disease again until 2011 when the patient presented with ocular lymphoma. The physician stated this was part of the same disease process as the prior breast diagnosis. The bone marrow was not involved in either case.

Is this a single primary (recurrence) of follicular lymphoma? Or are these multiple primaries because they arise in different extralymphatic sites?

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

Accession a single primary, follicular lymphoma [9690/3] of the breast diagnosed in 2008 per Rule M2.

Accession a single primary when there is a single histology. This is a recurrence of the patient's 2008 follicular lymphoma.

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
20130106 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a 2009 diagnosed Hodgkin lymphoma, nodular sclerosis type is treated and subsequently presents in 2010 with the same diagnosis? See Discussion.

2009 diagnosis of Hodgkin lymphoma, nodular sclerosis type involved the superior mediastinal nodes, AP window nodes, bilateral axillary nodes and pulmonary nodules. The patient received chemotherapy and went into remission.

Patient presents in 2010 with Hodgkin lymphoma, nodular sclerosing type in the superior mediastinum.

Does timing play any part in determining if this reported as one or two primaries? There is no timing rule in the Heme Manual.

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

Accession a single primary, Hodgkin lymphoma, nodular sclerosis type [9663/3] diagnosed in 2009 per Rule M2.

Accession a single primary when there is a single histology. Note 2 for Rule M2 indicates timing is not relevant. This is disease progression or recurrence and not 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.

2013
20130128 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient has a history of chronic myelomonocytic leukemia and a 12/08/2011 subsequent biopsy of the left leg that confirms leukemia cutis? See Discussion. Patient with a history of chronic myelomonocytic leukemia has been undergoing treatment with Dacogen for three years. On 12/8/11 the patient had a biopsy of the left leg that confirmed a diagnosis of leukemia cutis. How is the leukemia cutis coded?

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

Accession a single primary, chronic myelomonocytic leukemia [9945/3], per Rule M2. Accession a single primary when there is a single histology.

This is not a new primary. Leukemia cutis is the infiltration of neoplastic leukocytes into the skin from the existing leukemia. This is an advanced phase of the leukemia and has a poor prognosis.

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
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
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
20110031 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if patient initially diagnosed with granulocytic sarcoma on a vocal cord biopsy is subsequently diagnosed with acute myeloid leukemia more than 21 days later? See Discussion.

The patient has a history of refractory anemia with excess blasts diagnosed in 2008. A vocal cord biopsy performed on 6/2/2010 stated, "in view of a previous history of myelodysplastic syndrome this is indicative of transformation to acute leukemia" and consistent with granulocytic sarcoma. A bone marrow biopsy done on 7/19/2010 stated this was compatible with refractory anemia with excess blasts in transformation.

Granulocytic sarcoma is a solid manifestation of AML. When these diagnoses occur more than 21 days apart, are they separate primaries?

According to the WHO definition, this is acute myeloid leukemia complicating myelodysplasia. Which rule applies for this case?

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. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.

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