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Report Produced: 11/26/2024 21:21 PM

Report Question ID Question Discussion Answer Year
20061037 Multiple Primaries/Histology--Lymphoma: If a gastric biopsy demonstrates large B cell lymphoma arising in a low grade MALT lymphoma, how many tumors should be abstracted and how should the histology field(s) be coded? See Discussion.

Final path for gastric biopsy on 12/2005 is "consistent with malignant lymphoma" and Micro says "morphologic findings consistent with MALT lymphoma and an increased proportion of large atypical cells is concerning for large cell transformation. However, since the large cells are present only focally, a definitive diagnosis of large cell lymphoma cannot be rendered"

A second gastric biopsy a week later said: Final Path: Diffuse large B cell lymphoma arising in low grade MALT lymphoma. Micro says: "Compared to patient's previous biopsy...the current specimen contains a higher percentage of large atypical cells which stain positively for CD79a, a B cell marker. The morphologic and immunohistochemical findings are consistent with a large B cell lymphoma arising in a low grade MALT lymphoma."

These are different primaries according to the table of single versus subsequent primaries of lymphatic and hematopoietic diseases.

For cases diagnosed prior to 1/1/2010:

This is one primary. Code as 9699 [Marginal zone B-cell lymphoma, NOS].

The first biopsy was not conclusive. The biopsy one week later was more definitive. The reports are describing a difference between specimens, not a difference in disease.

According to the WHO classification, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is an extranodal lymphoma with B-cells, cells resembling monocytoid cells, small lymphocytes and scattered immunoblast and centroblast-like cells.

For cases diagnosed 2010 forward, 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.

 2006
20061010 Multiple Primaries/Histology--Lymphoma: If an oral mucosa, right hard palate biopsy contains a composite lymphoma [low-grade follicular + chronic lymphocytic leukemia], how many tumors should be abstracted and how should the histology field(s) be coded?

For cases diagnosed prior to 1/1/2010:This is one primary. Assign code 9590 [Malignant lymphoma, NOS]. This is a composite lymphoma. Code to lymphoma when there is any solid tumor (in lymph nodes, tissue, etc.) Code to lymphoma, NOS since this is not purely follicular and there is no code for composite lymphoma.

For cases diagnosed 2010 forward, 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.

 2006
20021158 Multiple Primaries/Histology--Lymphoma: What is the primary site(s) for a patient who had a lymph node biopsy with the histology of "large B cell lymphoma arising in the setting of low grade B cell lymphoma c/w marginal zone B cell lymphoma with plasmacytic features"? See discussion. This patient also had a bone marrow biopsy that demonstrated "low grade B cell lymphoma." Per the clinician, "Pt with discordant lymphoma. We will be approaching his lymphoma as two different diseases. The large B cell had cleared after chemotherapy and radiation therapy. The low grade lymphoma is incurable."

For cases diagnosed prior to 1/1/2010:

Code as two primaries with each arising in lymph nodes [C77._]. The histology for the first primary is 9699/3 [marginal zone B cell lymphoma]. The histology for the second primary is 9680/3 [large B cell lymphoma].

For cases diagnosed 2010 forward, 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.

 2002
20021061 Multiple Primaries/Histology--Mycosis Fungoides/Cutaneous T cell Lymphoma: Physicians often use the terms cutaneous T cell lymphoma (CTCL) and mycosis fungoides interchangeably and yet the SEER Single versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table indicates that these 2 diagnoses represent separate primaries. Do these cases represent one primary? If so, what histologic type should they be coded to?

For cases diagnosed prior to 1/1/2010:The patient does not have two different malignancies. Code the Histology field to 9700/3 [mycosis fungoides], the specific type of cutaneous T cell lymphoma. Mycosis fungoides is one of several types of cutaneous T cell lymphoma. Physicians often refer to mycosis fungoides by the "umbrella term" cutaneous T cell lymphoma.

The table indicates that the broad category of "T/NK-cell NHL" (which includes CTCL) and mycosis fungoides are presumably separate primaries because several entities are included in that broad category. In the specific case cited above, one entity (CTCL) within the broad category (T/NK-cell NHL) and mycosis fungoides are not separate primaries.

For cases diagnosed 2010 forward, 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.

 2002
20130050 Multiple Primaries/Primary site/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the primary site and histology for each if a 6/12/12 left shoulder mass specimen suspicious for large B-cell lymphoma is followed on 7/10/12 with three skin nodules excised from the back with a diagnosis of "composite lymphoma? See Discussion.

6/12/12 Excisional biopsy left shoulder soft tissue mass: Suspicious for large B-cell lymphoma.

7/10/12 Excisional biopsy three skin nodules of back: "Composite lymphoma" - primary cutaneous anaplastic large cell lymphoma (CD3 pos, CD4 pos, CD30 pos, ALK neg with partial loss of CD5) and CONCURRENT cutaneous follicular center lymphoma (CD20 pos, PAX5 pos, BCL-6 pos, partially CD10 pos) and flow cytometry revealed results compatible with involvement by a lymphoproliferative disorder of T-cell lineage.

Per imaging performed, there was no involvement of lymph nodes or other organs.

Is the primary site C449 Skin, NOS and histology 9718/3 [Lymphoma, primary cutaneous anaplastic large cell] be correct?

Code primary site to C445 [skin, back] and histology to 9718/3 [cutaneous anaplastic large cell lymphoma] .

Per Rule M6, abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location. For this case, there is cutaneous follicular (follicle) center lymphoma (9597/3) and cutaneous anaplastic large cell lymphoma (9718/3).

Per Rule PH22, code the primary site to the site or origin (skin, back) and the histology to the NHL with the numerically highest ICD-O-3 code. In this case, that would be 9718/3.

2013
20041047 Multiple primaries (Pre-2007)/EOD-Extension--Fallopian Tube: How many primaries are coded when endometrioid adenocarcinoma involves bilateral fallopian tubes? See Discussion. The pathologist states "because of the intimate association with the luminal line of the fallopian tube it is felt that this represents synchronous primaries rather than mets." The SEER Code Manual only lists ovary, retinoblastomas, and Wilms Tumors under the bilateral code stated to be a single primary.

For tumors diagnosed prior to 2007:

Complete two abstracts, one for left fallopian tube and one for right fallopian tube. This case has been determined to be two primaries by the pathologist. Bilateral involvement of paired sites (other than ovary, retinoblastoma and Wilms tumor) with the same histology within two months requires a determination of whether there are one or two primaries. The pathologist in the case above has made this determination.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

 2004
20091042 Multiple primaries--Hematopoietic, NOS: How many primaries should be coded when a patient has multiple occurrences of plasmacytoma followed by a diagnosis of multiple myeloma? See Discussion.

Example: Patient had a diagnosis on February 2003, plasmacytoma of the sinus; June 2003, plasmacytoma of the alveolar ridge; July 2003, plasmacytoma of the skin; and June 2004, multiple myeloma.

If this represents a transformation of plasmacytomas to multiple myeloma, will the information on multiple myeloma be available for statistical and research purposes?

For cases diagnosed prior to 1/1/2010:Accession this case as plasmacytoma diagnosed in Feb. 2003. Each of the subsequent diagnoses are not abstracted as new primaries. They are the "same," one primary only, according to the Definition of Single and Subsequent Primaries for Hematologic Malignancies (the tri-fold heme table).

The 2003 diagnosis is a classic example of extraosseous plasmacytoma (9734/3).

Plasmacytoma and multiple myeloma would be two primaries in the new hematopoietic rules taking effect in 2010.

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.

 2009
20130179 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries and what is the histology for each primary if a diffuse large B-cell lymphoma [9680/3] and a focus of splenic marginal zone lymphoma [9689/3] occur in a splenectomy specimen? See Discussion.

Patient presents with a huge mass in the spleen with direct extension to gastric fundus.

12/1/12 Splenectomy: Macroscopic nodules compatible with diffuse large B-cell lymphoma [9680/3]. Further, in the white pulp there are changes compatible with focus of splenic marginal zone lymphoma [9689/3].

Under the Transformations To section in the Heme DB, splenic marginal zone lymphoma transforms to diffuse large B-cell lymphoma.

Per Rule M4, this is a single primary. According to Rule M4, one is to abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s).

Per Rule PH11, code the histology to 9680/3 [diffuse large B-cell lymphoma] and the primary site to C422 [spleen]. According to PH11, one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

2013
20130172 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the histology for each if a bone marrow diagnosis reveals co-existent systemic mastocytosis and a lymphoplasmacytic neoplasm? See Discussion.

11/7/12 Peripheral blood flow cytometry: small population of clonal CD5- CD10- B-cells consistent with a B-cell lymphoproliferative process.

1/16/13 Bone marrow final diagnosis: co-existent systemic mastocytosis and lymphoplasmacytic neoplasm.

B-cell component of lymphoplasmacytic neoplasm constitutes 20% of bone marrow cellularity and the plasma cell component approximately 20%. The differential diagnosis includes marginal zone lymphoma with plasmacytic differentiation and lymphoplasmacytic lymphoma.

Flow cytometry: kappa monotypic B-cells and plasma cells.

Comment: Co-existence of systemic mastocytosis and mature B-cell lymphoma meets the criteria for Systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD).

From our physician's progress note: KIT-D816V-positive, CD117+/CD25+ /SM-AHNMD(40% of the nucleated cells as spindled mast cells) but also seemingly two distinct lymphoid neoplasms, a CD5-negative/CD10-negative B-cell lymphoproliferative neoplasm consistent with occupying another 20% of the nucleated marrow space, together with an IgG-kappa-restricted (non-reportable diagnosis) occupying another 20% of the nucleated marrow space (and an accompanying 2.0 g/dl M-spike without hypercalcemia or anemia).

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

Under the Alternate Names section of the Heme DB, systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD) is a synonym for systemic mastocytosis. Per Rule M2, this is one primary. Abstract a single primary when there is a single histology. Code the histology to 9741/3 [systemic mastocytosis].

Per the pathology report, the two diagnoses of systemic mastocytosis and mantle cell lymphoma meet the criteria for SM-AHNMD. The B-cell lymphoma is a symptom/marker of the AHNMD. In systemic mastocytosis with AHNMD, a myeloid or lymphatic malignancy is diagnosed with the SM. The prognosis is usually dominated by the non-mast cell malignancy.

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
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