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Report Produced: 07/04/2024 04:28 AM

Report Question ID Question Discussion Answer Year
20110066 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned for a patient with a history of CLL undergoing chemotherapy who is subsequently diagnosed on a liver biopsy with diffuse large B-cell lymphoma (Richter transformation)?

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

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).

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.

2011
20110146 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned when a patient was diagnosed in 2003 with malignant lymphoma, mixed cell type, follicular in the inguinal lymph nodes and was recently diagnosed with follicular lymphoma (by a neck lymph node biopsy) involving the neck and mediastinal lymph nodes?

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: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

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
20120058 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned when the patient is diagnosed with an acute neoplasm (diffuse large B-cell lymphoma) per a pathology report and is subsequently diagnosed clinically with a chronic neoplasm (chronic lymphocytic leukemia/small lymphocytic lymphoma) less than 21 days later? See Discussion.

The patient was diagnosed with an extranodal DLBCL on a biopsy of the stomach. A bone marrow biopsy performed 16 days later showed no DLBCL, but demonstrated an abnormal CD5-positive B-cell population that was subsequently referred to as CLL/SLL by the physician. The peripheral blood was negative and showed only moderate thrombocytopenia.

Does rule M10 apply in this case? Abstract the acute neoplasm as a single primary (DLBCL) as there was only one pathology specimen (stomach biopsy) proving DLBCL and the bone marrow did not definitively identify CLL/SLL.

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 M11. Code the histology of one primary to 9680/3 [diffuse large B-cell lymphoma], the acute neoplasm. Code the histology for the second primary to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma], the chronic neoplasm.

Per Rule M11, abstract as multiple primaries when both a chronic and acute neoplasm are diagnosed simultaneously or less than or equal to 21 days apart AND there is documentation of two pathology specimens, one confirming the chronic neoplasm (bone marrow biopsy) and one confirming the acute neoplasm (stomach biopsy).

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
20110126 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned, and what rule applies, when the patient has a history of diffuse large B-cell lymphoma diagnosed in 2003, a follicular lymphoma diagnosed in 2009, and another diagnosis of follicular lymphoma in 2010? Is the application of the multiple primary rules effected if it is unknown whether the patient was ever disease free? See Discussion.

Patient has a history of diffuse large B-cell lymphoma involving multiple lymph node regions (site C778) with bone marrow involvement diagnosed in 2003 and a history of follicular lymphoma confined to the thyroid and neck lymph nodes diagnosed in 2009. In 2010 the patient was diagnosed with follicular lymphoma in the inguinal and abdominal lymph nodes.

The 2003 diagnosis of DLBCL and the 2009 diagnosis of follicular lymphoma are the same primary according to the 2009 rules, the Single Versus Subsequent Primaries Table.

What rule is used to determine whether the 2010 diagnosis of follicular lymphoma represents a new primary? Which histologies are compared using the rules: the 2010 follicular lymphoma diagnosis to the 2009 follicular diagnosis or the 2010 follicular lymphoma diagnosis to the 2003 DLBCL diagnosis?

This case should be accessioned as one primary.

Reportability is determined by the year of diagnosis. The original DLBCL was diagnosed in 2003 and the follicular lymphoma in 2009. The pre-2010 rules are used for both cases. Per the Single Versus Subsequent Primaries Table, these are the same primary. It is reported with the histology 9680/3 [diffuse large B-cell lymphoma]

Do not compare the DLBCL diagnosed in 2003 and the follicular lymphoma diagnosed in 2010 because the determination of the number of primaries for the two specific histologic types was done (as it should have been) using the rules in effect in 2009 when the follicular lymphoma was first diagnosed. The determination of a single or multiple primaries is made the first time the patient presents with the two different diseases; it is not changed when the same disease process reappears after 2010.

2011
20130136 Multiple primaries--Heme & Lymphoid Neoplasms: If a neoplasm is listed under the Transformations section in the Heme DB, is this always a new primary? See Discussion.

Where are the instructions for coding transformations? When a disease is listed under the transformations, the Multiple Primaries Calculator states it is a new primary. Is this a new primary when the physician calls it a transformation?

For example, patient was diagnosed in 2000 with chronic lymphocytic leukemia (CLL). A biopsy of a stomach mass on 4/26/12 was positive for diffuse large B-cell lymphoma. DLBCL is listed under the Transformations To section in the Heme DB for CLL. Is this a new primary because it is a transformation?

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

Transformations do not always indicate a multiple primary is to be reported. Always apply the M Rules to determine the number of primaries. Refer to Rules M8-M13 in the Heme Manual address to determine the number of reportable primaries when chronic and acute neoplasms (transformations) are indicated on a case. Do not use the MP Calculator to determine the number of primaries unless the M Rules direct you to use it.

This case should be accessioned as two primaries, chronic lymphocytic leukemia [9823/3] diagnosed in 2000, and diffuse large B-cell lymphoma [9680/3] diagnosed on 04/26/2012 per Rule M10. Abstract a new primary when a neoplasm is originally diagnosed as a chronic (less aggressive) neoplasm (CLL) and there is a second diagnosis of an acute neoplasm (DLBCL) more than 21 days later.

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
20100101 Multiple primaries--Heme & Lymphoid Neoplasms: Is a 10/2010 diagnosis of accelerated phase of CML following a 4/2010 diagnosis of blast phase CML a new primary? See Discussion.

Patient was diagnosed in the blast phase of CML on a 4/2010 bone marrow biopsy. Pt failed Gleevec and progressed to the accelerated phase of CML in 10/2010.

Is this a single primary? This is not addressed in the hematopoietic rules. If this is a multiple primary, what rule should be applied?

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

Per Rule M2 this is a single primary because there is only a single histology represented for this case.

Under the Alternate Names section in the Heme DB for chronic myelogenous leukemia (CML), NOS [9863/3 and chronic myelogenous leukemia, BCR-ABL1 positive [9875/3] it indicates CML-blast phase, CML-accelerated phase and CML-chronic phase are all synonyms for CML, NOS. Any combination of these terms diagnosed represents one disease process. The Gleevec was given to prevent or delay progression to the accelerated phase.

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
20130207 Multiple primaries--Heme & Lymphoid Neoplasms: Is a new primary reported for the diagnosis of plasmacytoma associated with a pathological fracture if it follows a diagnosis five years ago of multiple myeloma? See Discussion.

Multiple myeloma was diagnosed more than 5 years prior to admission. The patient underwent multimodality treatment.

Currently, the patient suffered a fracture. The pathology report diagnosis was "plasmacytoma." The discharge summary states, "multiple myeloma advanced with multiple lytic lesions".

Does this scenario represent a single primary dating back to the original diagnosis? Or does the diagnosis of plasmacytoma on the recent biopsy indicate a new primary because it was originally diagnosed as acute and reverts to a chronic neoplasm after treatment more than 21 days later?

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

Per the Abstractor Notes section, this case represents a single primary. Histology is coded to 9732/2 [multiple myeloma], which is now advanced.

Review the Abstractor Notes section in the Heme DB for multiple myeloma. It states that in multiple myeloma there is generalize bone marrow involvement. It further states that lytic bone lesions and bone tumor masses of plasma cells (plasmacytomas) are signs of advanced disease. According to the Discharge Summary, this patient had multiple lytic lesions and plasmacytoma which indicates advanced disease.

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
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
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
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