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20110148 | First course treatment/Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned and how is treatment coded when follicular lymphoma diagnosed in December 2009 is treated with CHOP and a subsequent December 2010 diagnosis of diffuse large B-cell lymphoma is treated with chemotherapy and a bone marrow transplant? See Discussion. | A follicular lymphoma [9690/3] involving multiple lymph nodes was diagnosed on 12/2/2009. The patient had no bone marrow involvement and was treated with CHOP as first course treatment. In October 2010, the patient was put on maintenance Rituxan but disease progression was noted in November 2010. A biopsy of a mesenteric lymph node in December 2010 showed diffuse large B-cell lymphoma [9680/3]. The patient subsequently had chemotherapy and an autologous bone marrow transplant.
According to the Multiple Primaries Calculator in the Heme DB, the DLBCL is a new primary but the physician calls the diagnosis of DLBCL a transformation from the follicular lymphoma diagnosed in December 2009. |
Updated May 2026
This case should be accessioned as two primaries: follicular lymphoma [9690/3] and diffuse large B-cell lymphoma [9680/3] per Rule M10. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (follicular lymphoma) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) after the initial clinical workup for the chronic neoplasm was completed.
Record the CHOP as the first course of treatment for the follicular lymphoma because this was the only treatment given for the chronic neoplasm (follicular lymphoma) prior to the transformation to the acute neoplasm (DLBCL). Record the chemotherapy and bone marrow transplant as first course treatment for the DLBCL.
As noted above, follicular lymphoma does transform to DLBCL. This "transformation" is actually a new disease. Follicular lymphoma is a disease in which the lymph nodes have a prominent follicular pattern; DLBCL is a disease with diffuse proliferation of large lymphoid cells. While it is true that follicular lymphoma will transform to DLBCL, this transformation indicates it becomes a different entity.
The DLBCL is coded as a second primary for several reasons: to determine the incidence of follicular lymphomas transforming to DLBCL; survival time can be calculated for the diagnosis of the more aggressive DLBCL; death will be attributed to the DLBCL (for mortality statistics) and not the 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. |
2011 |
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20130081 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is clinically stated to have Stage III follicular lymphoma following a diagnosis suspicious for B-cell lymphoma and is subsequently diagnosed with large B-cell lymphoma? See Discussion. | 01/27/2012 R neck mass FNA: Suspicious for B-cell non-Hodgkin lymphoma. 02/17/2012 Cervical node bx: In situ involvement by follicular-like B-cells of uncertain significance +CD10. Two other cervical biopsies show infarcted, extensively necrotic lymphoid tissue highly suspicious for B-cell lymphoma.
03/20/2012 Bone marrow: Low grade B-cell lymphoproliferative disorder with plasmacytic differential.
04/18/2012 Medical Oncology treats patient for Stage III follicular lymphoma. 10/16/2012 Cervical LN core bx: CD10+ large B-cell lymphoma.
Should Rule M4 (single primary) and Module 6, Rule PH11 apply to this case? |
Updated May 2026
This case should be accessioned as two primaries: follicular lymphoma [9690/3] diagnosed 02/17/2012 and diffuse large B-cell lymphoma [9680/3] diagnosed 10/16/2012 per Rule M10. This patient was diagnosed with a chronic neoplasm (follicular lymphoma) followed later by an acute neoplasm (DLBCL) after the completion of the initial clinical workup for the chronic neoplasm.
The follicular lymphoma was initially diagnosed on 02/17/2012. The cervical node biopsies were "highly suspicious for B-cell lymphoma" [9591/3]. While "suspicious" is a reportable ambiguous term used to accession cases, suspicious cytologies are not SEER reportable and, therefore, the diagnosis date cannot be 01/27/2012. The histology of the first primary would be updated to 9690/3 [follicular lymphoma] based on the Medical Oncology note on 04/18/2012 that confirmed the histology was follicular lymphoma and the patient was being treated for such.
The diagnosis of DLBCL was made 8 months later. Rule M4 cannot apply to this case because the follicular lymphoma and DLBCL were not diagnosed in the same biopsy specimen. Rule M4 only applies when the two non-Hodgkin lymphomas are diagnosed in the same biopsy specimen. |
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20100053 | Primary site--Heme & Lymphoid Neoplasms: How is primary site coded for a myeloid sarcoma (granulocytic sarcoma) arising in the chest wall in a patient that has a negative bone marrow biopsy? See Discussion. | Patient was diagnosed with Myeloid Sarcoma (granulocytic sarcoma) by chest wall biopsy. This is an extramedullary manifestation of acute leukemia and is not in the bone marrow (bone marrow is negative).
How should primary site be coded? The Heme DB states that almost any part of the body can be involved. It also states to not code primary site to C421. In this case the only involvement is the chest wall [C493]. However, use of the primary site code C493 triggers an edit error questioning this site/histology combination. If the primary site is coded to C421 [bone marrow], there is no edit error. Please explain the site code and rationale. |
Updated May 2026
Unless there are scans showing involvement of a lymph node or tissue other than the chest wall, the histology should be coded myeloid sarcoma [9930/3] and the primary site to C493 [soft tissue of chest wall]. Override the edit.
Per the Abstractor Notes section in Heme DB, for myeloid sarcoma [9930/3] the most frequently affected sites are skin, lymph nodes, gastrointestinal tract, soft tissue, and testis. |
2010 |
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20110010 | Multiple primaries--Heme & Lymphoid Neoplasms: Is a recently diagnosed granulocytic sarcoma followed by a diagnosis of AML two primaries? See Discussion. |
6/10/10 Axillary lymph node biopsy was compatible with AML. The physician noted that the patient was diagnosed with granulocytic sarcoma [9930/3] in the axillary node. 6/15/10 Bone marrow biopsy compatible with AML FAB M1 [9873/3]. After induction, a second bone marrow biopsy on 6/30/10 shows persistent/refractory AML. The physician noted that the second biopsy is compatible with AML FAB M7 [9910/3]. Is the granulocytic sarcoma a chronic form of the disease? If so, do we have one primary diagnosed 6/10/10 with primary site coded to C42.1 and histology coded to 9873/3? Does the second biopsy on 6/30/10 represent the same primary even though the persistent disease is now FAB M7? |
Updated May 2026 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 (i.e., within the initial clinical workup). Apply Rule M3 to this case which states to abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Code the primary site to C421 [bone marrow] with histology coded to 9873/3 [acute myeloid leukemia, M1]. The FAB category is an older classification that is seldom used. Changes from FAB 1 to FAB 7 do not constitute a new primary. |
2011 |
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20120072 | Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a diagnosis of multifocal Langerhans cell histiocytosis with involvement of the bone, liver, spleen and retroperitoneum? |
Updated May 2026 In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen). Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes. The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. The Abstractor notes also indicate that if it's not possible to determine primary site for LCH, default to Bone, NOS (C419) |
2012 | |
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20120039 | Primary site--Heme & Lymphoid Neoplasms: What primary site and heme rule applies when a PET scan shows bilateral renal masses, hypermetabolic liver lesions and retroperitoneal lymphadenopathy, a right kidney biopsy was positive for diffuse large B-cell lymphoma, and the bone marrow biopsy was negative? See Discussion. |
Patient has a history of chronic lymphocytic leukemia (CLL). February 2011 abdomen/pelvis x-ray showed development of bilateral renal masses. April 2011 PET scan showed intense areas of hypermetabolic activity corresponding to known bilateral renal masses, new hypermetabolic liver lesions, as well as left upper retroperitoneal lymphadenopathy. All findings are worrisome for malignancy. March 2011 right kidney mass biopsy was positive for diffuse large B-cell lymphoma (DLBCL). Bone marrow biopsy was negative for lymphoma. |
Updated May 2026 Per Rule PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney. The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred after the initial clinical workup for the CLL/SLL was completed, it is a new primary per Rule M10. 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 |
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20110017 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a patient originally diagnosed with CLL is subsequently diagnosed several months later on a bone marrow biopsy with Richter's syndrome that transformed into a large cell lymphoma? See Discussion. |
Per reviewed resources, the described condition is rare. Should the histology remain CLL or be changed to large cell lymphoma? |
Updated May 2026 This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more after the initial clinical workup of the chronic disease is completed. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm. Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3]. |
2011 |
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20130031 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a plasmacytoma of the intervertebral disc is diagnosed in 2010 followed by a diagnosis of immature plasma cell myeloma by a right hip biopsy in 2011? See Discussion. |
The patient was diagnosed with intervertebral disc plasmacytoma and had radiation therapy to the pelvic bones in 2010. In 2011 (more than 21 days later) a right hip biopsy revealed immature plasma cell myeloma. There is clinical documentation that this is progression into myeloma. Per the Heme DB (Primary Site(s) and Definition sections) and Rule PH30, in the Heme Manual, the primary site is coded to C421 [bone marrow] and the histology is coded 9732/3 [plasma cell myeloma] when there is a clinical diagnosis of multiple myeloma and/or there is no documentation of a bone marrow biopsy or the results are unknown. This patient did have a bone marrow biopsy that indicates there are an increased plasma cells present; plasma cells represent less than 10%. The skeletal survey and bone scan did not reveal any further lesions. Is this progression of disease because there is only one lesion in the right hip 8 months after the diagnosis of plasmacytoma? Or is this a second primary based on the right hip biopsy that showed plasma cell myeloma and the physician's documentation of disease progression? Plasmacytomas are usually single lesions. Would this disease process have multiple lesions if they are diagnosed at different times? |
Updated May 2026 This case is accessioned as two primaries: Plasmacytoma diagnosed in 2010 and plasma cell myeloma diagnosed in 2011 per Rule M10. The patient has a diagnosis of a solitary plasmacytoma (chronic neoplasm) followed by a diagnosis of plasma cell myeloma (acute neoplasm) diagnosed after the initial clinical workup was completed for the chronic (plasmacytoma) neoplasm. The physician is calling this a progression to plasma cell myeloma even though the bone marrow has less than 10% plasma cells, take this statement as progression or a clinical diagnosis of plasma cell myeloma. |
2013 |
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20110142 | Reportability--Heme & Lymphoid Neoplasms: Is the pathologic final diagnosis of "follicular lymphoma, WHO grade 1-2, findings may represent in situ follicular lymphoma" reportable if the clinician also states this may be an "in situ follicular lymphoma"? See Discussion. |
2/16/11 mesentery biopsy showed "follicular lymphoma, WHO grade 1-2, findings may represent an "in situ" follicular lymphoma." 3/7/11 clinician note stated, "nodularity of the mesentery which upon biopsy may be in situ follicular lymphoma. No treatment is necessary. This is not a proven malignancy. It may evolve into one. Plan 6 month follow-up and CT scans. Do the notes from the oncologist and pathologist stating that this "may be" or "may represent" an in situ lymphoma make this case non-reportable? |
Updated May 2026 This case should not be accessioned. In situ lymphoma is not reportable for any of the standard setters (CoC, NPCR, or SEER) for any diagnosis year. In the Case Reportability Instructions, it states "Do report in situ (/2) lymphomas." |
2011 |
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20110143 | Multiple primaries--Heme & Lymphoid Neoplasms: How many and what primary site(s) are to be accessioned when biopsies of clavicular and neck skin lesions are both consistent with mycosis fungoides? See Discussion. |
Per the Heme DB and Manual, this is a single primary; however, per the MP/H Rules, this would be multiple primaries. Which rules apply to this case? |
Updated May 2026 When there is a question of whether the SEER MP/H Rules (2007-2017), Solid Tumor Rules (2018+) or Hematopoietic and Lymphoid Neoplasm Rules (2010+) apply, check the histology and refer to the Case Reportability Instructions in the Hematopoietic and Lymphoid Neoplasm Manual. All ICD-O-3 morphology codes in the range 9590 - 9992 are included in the Hematopoietic Rules. Mycosis Fungoides [9700/3] is included in this range. Therefore, the SEER MP/H Rules do not apply to mycosis fungoides. This case should be accessioned as a single primary: mycosis fungoides [9700/3] of the skin, NOS [C449]. Per Rule M2 abstract a single primary when there is a single histology. Note that in the Primary Site(s) section of the Heme DB, it states the primary site must always be coded to skin (C440 - C449) for mycosis fungoides. Because the primary site is stated in this section of the Heme DB, it is not necessary to use the Primary Site Rules to determine the primary site. Code the primary site to C449 [skin, NOS] because the patient has multiple sites of skin involvement and there is no documentation indicating which subsite of skin was the origin of the mycosis fungoides. 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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