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Report Produced: 09/07/2024 08:20 AM

Report Question ID Question Discussion Answer Year
20130215

Reportability--Heme & Lymphoid Neoplasms: Is hemophagocytic lymphohistiocytosis synonymous with an EBV-associated lymphoproliferative disorder in children reportable? See Discussion.

Pathology report states: Prominent T-cell infiltrate with frequent immunoblast-like cells. COMMENT: Findings consistent with an acute EBV-associated hemophagocytic process. In addition, there is a prominent CD8 + T-cell infiltrate with many large, activated forms. This T-cell process may represent an EBV-associated lymphoproliferative disorder in children.

EBV-associated lymphoproliferative disorder in children is listed in the Heme database. However, throughout multiple admissions, the oncologist states the diagnosis as "hemophagocytic lymphohistiocytosis". Are the two the same condition?

The patient is being treated with Etoposide.

Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

 2013
20130214 Primary site--Heme & Lymphoid Neoplasms: Does Rule PH20 apply if a patient with lymphoma has bilateral axillary and bilateral inguinal lymph node involvement? Rule PH20 states to code the primary site to the specific lymph node region when multiple lymph node chains within the same region as defined by ICD-O-3 are involved. Note 1 further states that one is to use this rule when there is bilateral involvement of lymph nodes.

Rule PH21 applies to this situation which states to code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. Axillary nodes are coded to C773 and inguinal nodes are coded to C774. There are two lymph node regions involved. Code the primary site to C778 [multiple lymph nodes].

If this patient had only bilateral axillary OR only bilateral inguinal nodes are involved, then PH20 would have applied and you would code to the specific lymph node region mentioned.

2013
20130213 Primary site--Heme & Lymphoid Neoplasms: How do you code the primary site for a marginal zone lymphoma involving bilateral axillary lymph nodes and inguinal lymph nodes, bone marrow and bilateral orbits that the physician refers to as a bilateral orbital lymphoma, Stage IV? See Discussion.

None of the rules seem to apply when the lymphoma is present in an organ, distant lymph nodes and bone marrow only. No regional nodes are involved.

Does rule PH22 infer that the organ should be coded as the primary site because it has been named by the physician?

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

Per Rule PH24, code primary site to orbit. According to Rule PH24, one is to code the primary site to the organ when lymphoma is present only in an organ. Note 2 under this rule also instructs one to capture the secondary involvement of distant lymph nodes and/or bone marrow in CS extension fields.

If the physician had not confirmed the primary site as orbit, you would have used Rule PH22 when the primary site is not indicated.

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
20130212 Reportability--Heme & Lymphoid Neoplasms: Is a case reportable in which the pathology report is negative for plasmacytoma but a subsequent physician's clinical diagnosis is plasmacytoma?

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

This case is reportable if the patient was treated for plasmacytoma. When the physician calls the case plasmacytoma and treats the patient accordingly, report the case.

See Case Reportability Instructions #6: Report the case when there is a clinical diagnosis (physician's statement) of a reportable hematopoietic or lymphoid neoplasm.

Note 1: The clinical diagnosis may be a final diagnosis found within the medical record or recorded on a scan (CT, MRI for example)

Note 2: Report the case even if the diagnostic tests are equivocal. A number of hematopoietic neoplasms are "diagnoses of exclusion" in which the diagnostic tests are equivocal and the physician makes the clinical diagnosis based on the equivocal tests and the clinical picture. See the Heme DB for definitive diagnostic methods for the specific neoplasm being abstracted.

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
20130211 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a bone marrow shows low grade mature B cell lymphoma with IgM paraprotein - macroglobulinemia? See Discussion.

Physician note: Bone marrow shows 10% involvement with low grade lymphoma. Assessment: Low grade mature B cell lymphoma with IgM paraprotein - macroglobulinemia.

The multiple primaries calculator indicates two primaries are to be reported. However, the physician stated that Waldenstrom's macroglobulinemia is another name for this patient's lymphoma.

There were no enlarged lymph nodes seen on the CT scan. The proposed treatment for this patient is Rituxan for the macroglobulinemia.

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 a single histology. The bone marrow initially showed a non-specific B-cell lymphoma. WM is a type of B-cell neoplasm. After immunophenotyping, a more specific histologic diagnosis of WM was made. In this case a single histology (WM) is diagnosed by the definitive diagnostic method (serum paraprotein demonstrating IgM), so it accessioned as a single primary.

Per PH16, code the histology to 9761/3 [Waldenstrom Macroglobulinemia (WM)] and the primary site to C420 [blood].

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
20130210

Primary site--Heme & Lymphoid Neoplasms: Does Rule PH27 apply meaning that primary site is coded to C809 or would it be more appropriate to code to C269 GI Tract NOS since all disease involves the GI tract and this is more specific?

Extranodal lymphoma first diagnosed in the stomach (fundus and antrum) which upon further investigation also involved the small bowel (MALT Lymphoma) in the absence of lymph node findings. MD staged this IIE. Initial thought was Gastric, but PET/CT indicated abnormal uptake involving loop of distended small bowel in the pelvis.

Assign C269 for Gastrointestinal tract, NOS. Apply Rule PH24, code to the organ when only an organ is involved. This rule can be used for NOS sites such as GI tract, NOS.

Based on the information provided, this lymphoma is confined to the GI tract -- stomach and small bowel.

2013
20130209

Multiple primaries--Heme & Lymphoid Neoplasms: Is a new bone marrow diagnosis of acute myelogenous leukemia that follows a 2007 treated diagnosis of a JAK-2 positive polycythemia vera a new primary?

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

Per Rule M10, abstract two primaries. Per the Heme DB, polycythemia vera [9950/3] transforms to an acute myelogenous leukemia [9861/3]. According to Rule M10, one is to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (e.g., polycythemia vera) AND there is a second diagnosis of an acute neoplasm (e.g., acute myelogenous leukemia) 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.

 2013
20130208

Histology--Heme & Lymphoid Neoplasms: How is histology coded when a bone marrow shows slightly hypercellular marrow with acute myeloid leukemia, non-M3 type and the flow cytometry is also consistent with acute myeloid leukemia, non-M3 type?

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

Without further information as to the type of acute myeloid leukemia, code the histology to 9861/3 [acute myeloid leukemia, NOS]. If further information on the specific acute myeloid leukemia becomes available, update the histology code.

Document that the pathology report states the acute myeloid leukemia is a "non-M3 type" in a text field. This documentation will help explain the choice of 9861/3 for this case. M3 refers to one of the eight FAB subtypes described by a group of French, American, and British leukemia experts in the 1970's who divided acute myeloid leukemias into subtypes, M0 through M7. They classified the disease based on the type of cell from which the leukemia developed and how mature the cells were. This was based largely on how the leukemia cells looked under the microscope after routine staining.

In this case, all we know is that the histology does not pathologically represent the M3 (acute promyelocytic leukemia (APL)) form of acute myeloid leukemia. We do not know which type of acute myeloid leukemia it does represent.

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
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
20130206 Primary site--Heme & Lymphoid Neoplasms: What rule applies to code a primary site for a peripheral blood diagnosis of marginal zone lymphoma that has a positive flow cytometry/FISH analysis when no biopsies are performed, scans show no evidence of disease, exam indicates no lymph nodes are palpable and the physician's clinical diagnosis "marginal zone lymphoma, unspecified site, stage 1"? See Discussion.

PE: No palpable lymph nodes.

PET scan: No spleen or lymph node uptake; no uptake anywhere in the body.

Peripheral blood and flow cytometry/FISH analysis diagnosis: Marginal zone lymphoma.

No bone marrow or biopsy of any lymph nodes done. Doctor states "marginal zone lymphoma, unspecified site, stage 1."

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

Per Rule PH27, code the primary site to C809 [unknown primary]. According to Rule PH27 one is to code the primary site to unknown primary site C809 when there is no evidence of lymphoma in lymph nodes AND the physician documents in the medical record that he/she suspects that the lymphoma originates in an organ(s) OR multiple organ involvement without any nodal involvement.

If further workup is done and a primary site is determined, update the primary site for this case.

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