Report | Question ID | Question | Discussion | Answer | Year |
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20210029 | Multiple primaries--Heme and Lymphoid Neoplasms: Is a patient with peripheral blood initially showing chronic myelogenous leukemia (CML), lymph node biopsy showing granulocytic sarcoma (9930/3), and bone marrow biopsy showing acute myeloid leukemia (AML) one or two primaries? See Discussion. |
1. 12/11/2020 Peripheral blood revealing what was thought to be chronic myelogenous leukemia BCR/ABL1 positive (9875/3). Patient was started on Hydrea while waiting for further tests on 12/12/2020. 2. 12/14/2020 Lymph node biopsy showed granulocytic sarcoma (9930/3), but flow cytometry states it is similar to that seen in the patient's peripheral blood and is consistent with nodal involvement by myeloblasts. 3. 12/15/2020 Bone marrow biopsy reads acute myeloid leukemia (9861/3), likely arising from BCR/ABL1 positive chronic myeloid leukemia. There is a note on this pathology from medical oncologist that says: This will dramatically change the course of his treatment, likely with a TKI. 4. 12/17/2020 Sprycel started. Patient was weaned off Hydrea. According to Rule M3, abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. It lists 9930/3 when simultaneously (or after) with 9861/3. Technically, it was two days before, but I feel like I should and could count that as simultaneously because of Note 1 that says: These sarcomas are solid manifestations of the associated leukemia. For example, when acute myeloid leukemia and myeloid sarcoma are diagnosed simultaneously, the myeloid sarcoma is the result of myeloid cells migrating from the bone marrow or blood into tissue. It is part of the disease process for the acute leukemia. Also, the providers never mention granulocytic sarcoma Based on that, I think that #2 & #3 above are the same primary, which would be acute myeloid leukemia (9861/3). Per the hematopoietic database, 9875/3 transforms to 9861/3. Therefore, Rule M8 is confusing with the "only one" biopsy. Does this rule apply because the 9875/3 was from peripheral blood only? But peripheral blood is coded in Diagnostic Confirmation as histology. Rule M9 reads: The two diagnoses are likely the result of an ongoing diagnostic work-up. The later diagnosis is usually based on all of the test results and correlated with any clinical information. Because that is truly what I think is happening here though that rule states there is no available documentation. If you do not have any documentation, how would you know you are dealing with a chronic and an acute diagnosis? M10 does not apply. According to Rule M11, abstract as multiple primaries when both a chronic and an acute neoplasm are diagnosed simultaneously or within 21 days and there is documentation of two biopsies. The chronic myelogenous leukemia only had peripheral blood and not a bone marrow, lymph node or tissue, but that is counted as positive histology in diagnostic confirmation, but I don't know if that is kept as a separate field/thought. I would not code a peripheral blood smear as with a surgical code or a surgical diagnostic and staging procedure code, so maybe that is what I should be thinking about and therefore would probably say Rule M8 and one primary. |
This is one primary based on Rule M3. Abstract as a single primary site for the granulocytic sarcoma and AML since they are both evaluating the blood/bone marrow, which are counted as one site. To count them twice would result in over counting primaries. For Rule M9: This would not apply to your situation since you do have information on both the CML and the AML. We had to write in this rule for cases where you do not always have the information available. In terms of the peripheral blood versus actually biopsy: In this case, do not count the peripheral blood as a separate site. Rule M8 does fit your case, coding this as the AML and having this as one primary. |
2021 |
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20210023 | Reportability/Terminology--Head & Neck: Is an "evolving" squamous cell carcinoma of the vermillion border of the left lower lip reportable? |
For solid tumors, ignore the term "evolving" and apply the registry rules for reportability to this case. Squamous cell carcinoma of the vermillion border of the lower lip (C001) is reportable. |
2021 | |
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20210060 | Reportability/Histology--Thymus: Is a 2021 diagnosis of a type A microscopic thymoma reportable? See Discussion. |
ICD-O-3.2 lists microscopic thymoma as benign (8580/0) and thymoma, type A as malignant (8581/3). January 2021: Left central neck node dissection for thyroid carcinoma with thymic tissue showing an incidental type A microscopic thymoma, described as a small (<0.2 cm) focus. Diagnosis comments further indicate this is morphologically consistent with a microscopic thymoma (type A). |
Report this case as type A thymoma. We consulted an expert physician and his advice on this specific case is to interpret it as a malignancy and report. Use text fields to record the details of this case. |
2021 |
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20210027 | Reportability--Heme and Lympoid Neoplasms--Polycythemia vera: Is secondary polycythemia vera reportable? See Discussion. |
A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea. According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia. |
Abstract as a new primary for polycythemia vera, 9950/3. JAK2 is commonly used to assess suspected polycythemia vera and in this case, the mutation is positive for V617F. Based on the JAK2 results, this looks like a true polycythemia vera and not a secondary polycythemia. |
2021 |
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20210022 | Solid Tumor Rules (2018/2021)/Multiple primaries--Prostate: Is basal cell carcinoma with focal squamous differentiation and a small focus of infiltrating prostatic adenocarcinoma one or two primaries and if one, is the histology 8147/3? See Discussion. |
Scenario: Patient had a transurethral resection of the prostate on 8-29-19, positive for basal cell carcinoma with focal squamous differentiation involving approximately 50% of tissue (determined not to be mets by consult). On 11-14-19, the patient had a prostatectomy positive for residual basal cell carcinoma and a small focus of infiltrating prostatic adenocarcinoma. According to AJCC, 8th edition, page 724, basal cell carcinoma of the prostate is 8147/3 and we ignored the small focus of adenocarcinoma. The above scenario was reported as two primaries (8090/3 and 8140/3), but we are thinking it is one. |
Abstract a single primary and code as 8147/3 using Rule M18 and Rule H17 of the 2018 Other Sites Solid Tumor Rules. This is based on the findings of basal cell carcinoma of the prostate (8147/3) and adenocarcinoma (8140/3). We consulted with the Subject Matter Expert who advises that basal cell carcinoma and basal cell adenocarcinoma can be used interchangeably. This updates previous consultation regarding this histology. The Other Sites rules will be updated for 2022 and include this information in the prostate histology table. |
2021 |
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20210034 | Reportability/Histology--Endometrium: Is endometrial hyperplasia with atypia equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable? |
Endometrial hyperplasia with atypia is equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable for cases diagnosed 2021 and later. Our expert pathologist consultant confirmed this for us. |
2021 | |
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20210017 | Update to current manual/Mets at diagnosis fields--Lymphoma: Are distant metastases possible for a lymphoma with a primary site of lymph nodes? The instructions in the SEER manual tell us to assign code 8 in each of the Mets at Dx fields for a lymphoma originating in lymph nodes. |
This is a correction to the SEER manual. Lymphomas originating in lymph nodes (C77) could have distant metastases to any site except lymph nodes. The following corrections to the manual apply now and will appear in the next version of the manual. Remove C770-C779 from the instruction for assigning code 8 on the following pages. Page 135 Mets at Dx--Bone Page 137 Mets at Dx--Brain Page 139 Mets at Dx--Liver Page 141 Mets at Dx--Lung Page 145 Mets at Dx--Other Example Biopsy of axillary lymph node: Diffuse Large B-Cell lymphoma. Lymph nodes involved above and below the diaphragm, multiple nodules seen in lung, lesions in liver. Bone marrow biopsy positive for DLBLC. Per Hematopoietic manual, primary site would be C778 for multiple lymph node regions involved. Mets at Dx--Bone-0 Mets at Dx--Brain-0 Mets at Dx--Liver-1 Mets at Dx--Lung-1 Mets at Dx--Distant Lymph Nodes-8 Mets at Dx--Other-1 |
2021 | |
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20210058 | Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion. |
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored. 4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH. 5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia. 6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma. |
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3). The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern. In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis. We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns. |
2021 |
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20210025 | Primary site--Ovary: What information takes precedence for coding the primary site in cases with high grade serous carcinoma that are clinically called ovarian but on pathology, the pathologist calls the primary site fallopian tube and the gynecology oncology/managing phsyician continues to call the cases ovarian. Both the ovary and tube are involved. Sometimes also referred to as "tubo-ovarian." |
When the choice is between ovary, fallopian tube, or primary peritoneal, any indication of fallopian tube involvement indicates the primary tumor is a tubal primary. Fallopian tube primary carcinomas can be confirmed by reviewing the fallopian tube sections as described on the pathology report to document the presence of either serous tubal intraepithelial carcinoma (STIC) and/or tubal mucosal invasive serous carcinoma. If there is no information about the fallopian tubes, refer to the histology and look at the treatment plans for the patient. If all else fails, you may have to assign C579 as a last resort. Use text fields to document the details. For additional information, see the CAP GYN protocol, Table 1: Criteria for assignment of primary site in tubo-ovarian serous carcinomas. |
2021 | |
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20210016 | Solid Tumor Rules (2018, 2021)/Histology--Kidney: What is the correct histology code for a kidney primary described as clear cell papillary renal cell carcinoma"? Should we use H2 and code 8312/3 or H3 and code 8323/3? |
Assign 8323/3, clear cell papillary renal cell carcinoma using the 2018 Kidney Solid Tumor Rules, Rule H1, as this is a single histology, a variant of renal cell carcinoma NOS. |
2021 |