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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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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. |
2013 | |
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20130136 | Multiple primaries--Heme & Lymphoid Neoplasms: If a neoplasm is listed under the Transformations section in the Heme DB, is this always a new primary? See Discussion. | Where are the instructions for coding transformations? When a disease is listed under the transformations, the Multiple Primaries Calculator states it is a new primary. Is this a new primary when the physician calls it a transformation?
For example, patient was diagnosed in 2000 with chronic lymphocytic leukemia (CLL). A biopsy of a stomach mass on 4/26/12 was positive for diffuse large B-cell lymphoma. DLBCL is listed under the Transformations To section in the Heme DB for CLL. Is this a new primary because it is a transformation? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Transformations do not always indicate a multiple primary is to be reported. Always apply the M Rules to determine the number of primaries. Refer to Rules M8-M13 in the Heme Manual address to determine the number of reportable primaries when chronic and acute neoplasms (transformations) are indicated on a case. Do not use the MP Calculator to determine the number of primaries unless the M Rules direct you to use it.
This case should be accessioned as two primaries, chronic lymphocytic leukemia [9823/3] diagnosed in 2000, and diffuse large B-cell lymphoma [9680/3] diagnosed on 04/26/2012 per Rule M10. Abstract a new primary when a neoplasm is originally diagnosed as a chronic (less aggressive) neoplasm (CLL) and there is a second diagnosis of an acute neoplasm (DLBCL) more than 21 days later.
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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20130009 | Grade--Pancreas: Can the grade be coded when a biopsy is taken from the part of a primary tumor that has contiguously extended into an adjacent organ or structure? See Discussion. | The grade rule states to code grade from tissue removed from the primary tumor only, never from a metastatic site or a site of recurrence. There is no mention of whether the grade can be coded if only the contiguous site of involvement is biopsied when a single tumor directly extends to an adjacent tissue or organ. For example, is grade coded to 2 when a pancreatic tumor extends into the duodenum, and the duodenal biopsy confirms moderately differentiated adenocarcinoma consistent with a pancreatic primary? Or does the primary organ/site have to be biopsied in order to be able to code grade? | For one tumor involving a contiguous site, when there is no tissue specimen available from the primary site, you may code the grade based on the tissue from the tumor in the contiguous site.
This instruction is included in the upcoming grade instruction document. |
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20130184 | Reportability--Appendix: Are low-grade appendiceal mucinous neoplasms reportable? |
For cases diagnosed prior to 1/1/2022 A low-grade appendiceal mucinous neoplasm (LAMN) is not reportable. The WHO classification designates LAMN with the behavior code /1 [uncertain whether benign or malignant]. |
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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. |
2013 |
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20130035 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a subsequent diagnosis of diffuse large B-cell lymphoma (95%) and follicular lymphoma, grade 3 (5%) is made following an original diagnosis of low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL) ? See Discussion. |
In 2011, patient presented with a large mesenteric mass, numerous other smaller mesenteric lymph nodes, moderate retroperitoneal and extensive iliac chain adenopathy greater on right; small inguinal nodes are also present mostly on right side and splenomegaly per the CT scan. Abdominal pelvic mass needle biopsies showed low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL). The patient was treated with R-CVP with unknown response. In June 2012, patient presented again for laparoscopy and lymph node biopsy for stated recurrence of lymphoma found on CT scan. A large mass was seen in mesentery of bowel. Abdominal mass biopsy showed diffuse large B-cell lymphoma (DLBCL). Abdominal mass #2 excisional biopsy showed diffuse large B-cell lymphoma, 95%, and follicular lymphoma grade 3, 5%. The majority of the tumor is now DLBCL. |
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 diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s). Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was "most consistent with follicular lymphoma." The term "consistent with" is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011. 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. Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is a new primary per rule M12. |
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20130121 | Reportability--Heme & Lymphoid Neoplasms: Is "early essential thrombocythemia" reportable? See Discussion. | The bone marrow biopsy diagnosis was, "Combined bone marrow morphologic, flow cytometric, immunohistochemical, molecular and cytogenetic findings are most consistent with early or evolving essential thrombocythemia with low level JAK2 V617F mutation documented on molecular testing." The physician is calling this a benign process. Is this reportable as essential thrombocythemia? Are the terms early or evolving ignored? Does the presence of a JAK2 mutation make this reportable? Without JAK2 testing is this case reportable? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Yes, this is a reportable case. The histology is coded to 9962/3 [essential thrombocythemia]. The positive JAK2 mutation testing and bone marrow biopsy results taken together support the diagnosis of essential thrombocythemia in this case.
In the Abstractor Notes section of the Heme DB, it indicates that only 50-60 percent of patients with essential thrombocythemia will have a positive JAK2 mutation. A diagnosis of essential thrombocythemia can still be made in the absence of a JAK2 mutation. For example, if the bone marrow biopsy final diagnosis or a physician's clinical diagnosis is essential thrombocythemia, despite a negative JAK2 mutation test, the neoplasm is still reportable.
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 |
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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 |
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20130156 | Other therapy--Heme and Lymphoid Neoplasms: Based on the hematopoietic manual instructions, is plasmapheresis coded as treatment for Waldenstrom macroglobulinemia? See Discussion. | A patient, who was diagnosed with Waldenstrom macroglobulinemia at another facility, presented to our facility for plasmapheresis on 12/27/2012. No other treatment was given.
How is the plasmapheresis coded for treatment? |
Do not code plasmapheresis as treatment. It does not modify the neoplasm. | 2013 |
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