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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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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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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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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. |
2013 |
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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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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? |
Updated May 2026
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 when they are both diagnosed during the initial clinical workup 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. |
2013 |
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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? |
Updated May 2026
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.
In addition, the following note was added to the Heme DB for all the Follicular Lymphomas (9690/3, 9691/3, 9695/3, 9698/3) in 2025. Follicular lymphomas are always the same primary. When there are multiple diagnoses of Follicular lymphoma, update the histology (as needed) to always reflect the highest grade. Known grade always takes priority over the NOS histology. (See Rule M2 in the Hematopoietic manual). Priority order: 1. 9698/3: Follicular lymphoma, Grade 3, 3A, 3B 2. 9691/3: Follicular lymphoma, Grade 2 3. 9695/3: Follicular lymphoma, Grade 1 4. 9690/3: Follicular lymphoma, NOS |
2012 |
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20100037 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be accessioned for a patient diagnosed with essential thrombocythemia [9962/3] in 2002 who had a 2010 biopsy consistent with the fibrotic stage for a chronic myeloproliferative disorder that "suggests the patient is transforming to an acute myeloid leukemia"? See Discussion. |
Patient had a diagnosis of essential thrombocythemia [9962/3] in 2002 and was treated with Hydroxyurea. In 2010, the patient was admitted with severe bone pain and a diagnosis described as, "The overall features of the biopsy are consistent with a fibrotic stage of a chronic myeloproliferative disorder. The presence of up to 15% CD34+ immature cells seen in the biopsy suggests that the patient is transforming to an acute myeloid leukemia." In addition, cytogenetic studies and molecular testing for JAK2 were ordered. These findings confirmed a myeloproliferative disorder. JAK2 mutation was not detected. The patient died within 2 weeks. Is this a new primary?
Was this patient diagnosed with AML (which requires 20% or more blasts and this is only 15%)? If this is a new primary, is the histology 9861/3 [AML, NOS] or 9895/3 [AML with myelodysplasia-related changes]? Was the second diagnosis of AML definitively diagnosed? |
Updated May 2026
This case is a single primary, essential thrombocythemia [9962/3] in 2002. The 2010 diagnosis is chronic myeloproliferative disorder [9960/3].
According to Rule M15, the Multiple Primaries Calculator is to be used to first determine the number of primaries. Per the calculator, essential thrombocythemia and chronic myeloproliferative disorder are the same primary. (Acute myeloid leukemia is not used as the second histology because it is preceded by a non-reportable ambiguous term, "suggests." "Suggests" is not on the list of reportable ambiguous terms in the Hematopoietic and Lymphoid Neoplasm Coding Manual.
In 2010, this patient was in a late stage of ET. When any of the specific MPN neoplasms such as ET are in the late stage of disease, the characteristics of the specific disease (ET) will no longer be detectable. Accordingly, for this patient the diagnostic testing was positive for MPN, unclassifiable. In this case, do not change the diagnosis from the more specific disease (ET) to the NOS (MPN, unclassifiable) |
2010 |
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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? |
Updated May 2026
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) after the initial clinical workup of the chronic neoplasm is completed.
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. |
2010 |
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20100040 | Histology--Heme & Lymphoid Neoplasms: How is this field coded for a patient with a negative bone marrow and multiple plasmacytomas in different bone sites (e.g., thoracic vertebrae and left femur)? | Updated May 2026
The original answer for this question was to code the primary site to C419 [Bone, NOS] and histology to 9731/3 [solitary plasmacytoma] In 2021, we received clinical guidance from the WHO 4th edition which stated the presence of two plasmacytomas is multiple myeloma (9732/3), even if the bone marrow is negative or not evaluated. Registrars were not required to go back and review older cases. This case would be coded as a single primary, 9732/3 and primary site C421. |
2010 |
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