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20130058 | Reportability--Heme & Lymphoid Neoplasms: Is EBV-positive hemophagocytic lymphohistiocytosis (HLH) reportable when diagnosed in a 5 year old child and resulted in death in less than two months? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Hemophagocytic lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.
Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."
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. |
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20130057 | Histology--Heme & Lymphoid Neoplasms: How is the histology coded if the bone marrow biopsy favors lymphoplasmacytoid lymphoma and the physician states the diagnosis is lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia? See Discussion. | Bone marrow biopsy: Focal bone marrow involvement with B-cell lymphoproliferative disorder. Comment: This patient has 2 monoclonal proteins in serum, IgM kappa and IgG kappa clones. The marrow does have focal involvement with a small cell lymphoproliferative disorder. A lymphoplasmacytoid lymphoma is favored.
Flow Cytometry: Bone marrow reveals a low level, kappa-bearing-B-lymphoproliferative population that has an immunophenotype compatible with mantle cell lymphoma or related small, mature non-Hodgkin lymphoproliferative disorder.
Physician statement: lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia.
Per the Heme DB, the criteria to diagnosis WM is the serum paraprotein IgM. This patient's IgM was 6020 mg/dL. It was described as elevated per the physician. The physician also states the patient's IgG is elevated. According to the Heme DB, when both IgG and IgM are elevated it is indicative of LPL. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9671/3 [lymphoplasmactyic lymphoma (LPL)] per the Heme DB Abstractor Notes and Rule PH17. When IgG and IgM are elevated, code to lymphoplasmacytic lymphoma. Waldenstrom's macroglobulinemia is caused by increased lymphocytes which causes an increase in IgM. LPL has mixed abnormalities, both the lymphocytes and plasma cells are increased which results in an abnormally high IgM and IgG.
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. |
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20130056 | Primary site/Histology--Heme & Lymphoid Neoplasms: How are the site and histology fields coded if a bone marrow biopsy shows, "B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma," but the patient has no palpable lymphadenopathy and no scans were done? See Discussion. | Should the primary site be C779 or C421? Is the correct histology 9684/3 [malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS]? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site to C421 [bone marrow] and the histology to 9680/3 [diffuse large B-cell lymphoma] per Rule PH26. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is listed under Alternative Names section of the Heme BD for DLBCL [9680/3]. This patient has bone marrow involvement only. The Note for Rule PH26 instructs one to code the primary site to the bone marrow when all physical exams or work-up were negative for lymph node, tissue, or organ involvement OR no other work-up was done.
The histology is not coded 9684/3 [malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS]. This histology code became obsolete in 1/1/2010. Diffuse large B-cell lymphoma, immunoblastic variant is also listed under Alternative Names section of the Heme BD for DLBCL.
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. |
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20130055 | Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a lymphoma with multifocal bone and epidural involvement but no lymph node involvement if the physician does not clearly state the primary site? See Discussion. | MRI Lumbar spine: Bony metastatic disease most evident at L5, L3 and T10. There is marrow tumor in the posterior elements of T12 and T10. The 14 mm epidural mass represents epidural tumor, likely metastatic, extending into the left intervertebral foramen at T12-L1.
PET scan: Hypermetabolic activity corresponding to epidural mass at the level of T12 and L1 concerning for malignancy. Other small areas of hypermetabolic activity in the left mandible and both femoral necks. There is no hypermetabolic activity corresponding to the areas of abnormal marrow edema in the vertebral bodies which enhanced on MRI scan in the lumbar and lower thoracic spine. No lymph nodes mentioned.
Biopsy epidural mass: Diffuse large B-cell lymphoma with a background of follicular lymphoma, consistent with a large cell transformation. Flow cytometry confirms a mixed large and small cell population of lymphoma (55% large cells).
T12/L1 Bone Biopsy: Bone and marrow with atypical paratrabecular lymphoid infiltrates, suspicious for involvement by follicular lymphoma. Negative for large cell lymphoma. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site of the diffuse large B-cell lymphoma [9680/3] to C809 [unknown primary site] per Rule PH27. The patient has involvement of multiple bones and an epidural mass with no evidence of nodal involvement. Code the primary site to unknown [C809] when multiple organs are involved without any lymph node involvement, even when there is no statement from the physician regarding primary site.
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. |
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20130054 | MP/H Rules/Multiple primaries--Lung: How many primaries are accessioned if a lobectomy has two tumors that are both stated to be adenocarcinoma but the pathologist states they are synchronous primaries? See Discussion. | Left upper lung lobectomy: Adenocarcinoma, poorly-differentiated (grade 3), tumor size 1.2 cm, confined to lung. Second primary lung tumor: adenocarcinoma, well-differentiated (grade 1), tumor size 0.9 cm, confined to lung. Diagnosis COMMENT: The two tumors, although both adenocarcinoma, show markedly different histologies and thus are classified as synchronous primaries. Multiple synchronous primaries are staged separately according to the 7th edition of AJCC.
The AJCC Staging Manual 7th ed states, "Multiple tumors may be considered to be synchronous primaries if they are of different histological cell types. When multiple tumors are of the same cell type, they should only be considered to be synchronous primary tumors if, in the opinion of the pathologist, based on features such as ..., they represent different subtypes of the same histologic cell type..."
In this case, the pathologist insists these are two synchronous primaries, although different subtypes are not given, because the tumors have different grades and look completely different under the microscope. The MP/H rules indicate this is a single primary. How many primaries are accessioned? |
For cases diagnosed 2007 or later, accession a single primary, adenocarcinoma [8140/3] of the left upper lobe lung [C341]. The steps used to arrive at this decision are:
Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Lung MP rules because site specific rules exist for this primary.
Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. The patient has two tumors in the same lung with the same histology.
Do not use the AJCC Manual to make multiple primary decisions. Use the MP/H Rules to determine the number of primaries to accession. |
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20130052 | Histology--Heme & Lymphoid Neoplasms: How is the histology coded if a biopsy final diagnosis is diffuse large B-cell lymphoma but the physician's final diagnosis favored anaplastic large cell lymphoma? See Discussion. | Patient has diffuse intrathoracic, intraabdominal and pelvic lymphadenopathy. An inguinal lymph node biopsy showed diffuse large B-cell lymphoma. The physician's final diagnosis favored anaplastic large cell lymphoma, but wanted to confirm this with FISH. The patient clinically deteriorated so the FISH studies were not done. Which histology is coded? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
The histology should be coded as diffuse large B-cell lymphoma [9680/3]. The biopsy pathology report definitively diagnosed DLBCL. The physician's diagnosis cannot be used because it is an ambiguous diagnosis only, "favored anaplastic large cell lymphoma." "Favor" is an ambiguous term.
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. |
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20130051 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when biopsies of the left and right tonsils show diffuse large B-cell lymphoma and there is no other evidence of involvement? See Discussion. | Scans are negative for lymphadenopathy and the bone marrow biopsy was benign. Radiation Oncology staged this as localized bilateral tonsil primary lymphoma. | 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, diffuse large B-cell lymphoma [9680/3] of bilateral tonsils. Per Rule M2, a single histology is a single primary. Note 1 for Rule M2 states bilateral involvement of lymph nodes and/or organs is still 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. |
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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. |
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20130047 | Date of diagnosis--Heme & Lymphoid Neoplasms: What is the diagnosis date for a patient with a mild thrombocytosis diagnosed in 2008, that was subsequently treated with Anagrelide in 11/2010 following an increase in platelet count, and later in 3/2011 was found to have positive JAK2 study physician refers to as essential thrombocythemia? See Discussion. | In 2008, patient diagnosed with mild thrombocytosis. The patient opted to be followed clinically with observation. In November 2010, a CBC showed an increased platelet count to 600,000. Anagrelide was started. The patient would never agree to a bone marrow biopsy. However, in 3/2011 a JAK2 study was performed and read as positive. Following the positive Jak2 study, physician stated the diagnosis was essential thrombocytosis and started the patient on a different drug. | Code the diagnosis date to 3/2011. It wasn't until 3/2011 that the physician documented a reportable diagnosis of essential thrombocytosis [9962/3].
Mild thrombocytosis is not reportable. Therefore, the case was not reportable in 2008. Although the patient was treated in 2010, there was no documentation of a reportable diagnosis. |
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20130045 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if subsequent to a bone marrow biopsy diagnosis of acute myeloid leukemia there is an oncology consult note that indicates the pathology finding is suggestive of an underlying myelodysplastic syndrome? See Discussion | 5/14/12 Bone marrow biopsy: Acute myeloid leukemia (AML).
5/21/12 Oncology consult: AML with 30-40% blasts and evidence of del(20q) and del(5q), is suggestive of an underlying myelodysplastic syndrome (MDS). Hence the patient has secondary AML.
If these are two primaries, how are the diagnosis dates coded? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case is accessioned as a single primary diagnosed on 5/14/12 as acute myeloid leukemia with myelodysplasia-related (e.g., del(5q)) changes [9895/3] per Rule M2. The patient was diagnosed with a single histology, acute myeloid leukemia with myelodysplasia-related changes per the submitted information.
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. |
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