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20210014 | Solid Tumor Rules (2018, 2021)/Multiple Primaries--Lung: How many primaries should be reported for a 4/2019 diagnosis of left upper lobe (LUL) adenosquamous carcinoma (left lingula mass biopsy: adenosquamous carcinoma; LUL lung biopsy: pulmonary adenocarcinoma, stated to be a collision tumor and single primary per the Tumor Board), treated with radiation followed by an enlarging LUL mass in 7/2020 found to be squamous cell carcinoma? See Discussion. |
The physician stated the prior LUL adenosquamous carcinoma was PD-L1 negative and the LUL squamous cell carcinoma is PD-L1 positive and is calling it a new primary. 5/22-7/3/19 6000x30 IMRT Photons LUL lung Chemo refused Not a Surg candidate 10/01/2019 CT Chest: IMP: In comparison to CT chest 03/06/2019 and PET/CT 03/21/2019, left lingular mass has mildly decreased in size. Left apical anterior and posterior lung lesions more anterior lesion appears slightly increased in size, the other slight decreased in size, with adjacent areas of atelectasis and scarring. 06/23/2020 CT Chest: MP: In comparison to CT chest 10/1/2019, left lingular mass has increased in size concerning for increasing tumor with adjacent thicker focal pleural thickening involving the chest wall, concerning for possible chest wall invasion. Left apical anterior and posterior lung lesions appears more solid in appearance, representing known adeno CA, given that the appearance has changed, is concerning for residual tumor. 07/06/2020 PET: Hypermetabolic lingular mass and peripheral nodularity has increased in size and FDG avidity on the prior PET/CT. Left apical nodular opacity is difficult to separate from fairly uniform mild left apical pleural hypermetabolism which may be treatment related and/or neoplastic. |
Abstract two primaries: 8560 and 8140 using rule M6. One of the original tumors with adenosquamous now shows only residual SCC following XRT. PD-L-1 is not used to determine multiple primaries. Assuming three tumors (the post-XRT SCC is not a new tumor but residual from one of the adenosquamous tumors) there are two primaries: 8560 and 8140 per M6. For collision tumors, each histology identified in the tumor is used to determine multiple primaries. |
2021 |
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20210007 | First Course Treatment/Reason for No Surgery of Primary Site: How should we be coding Reason For No Surgery of Primary Site for cases where surgery was planned but ultimately cancelled due to progression? See Discussion. |
There is a discrepancy in the SEER and STORE manual definition of code 2 for Reason for No Surgery of Primary Site. STORE includes progression of tumor prior to planned surgery as part of the definition for code 2, but the SEER Manual does not. The progression statement is included in the SEER Manual (2018 and 2021) for Reason for No Radiation, but not for Reason for No Surgery. |
Assign code 2 for cases where surgery was planned but ultimately cancelled due to progression in the data item Reason For No Surgery of Primary Site. Code 2 description contains examples and is not exhaustive of reasons for no surgery. We will add the example for consistency in the next version of the SEER manual. |
2021 |
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20210002 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed with therapy-related myelodysplastic syndrome (t-MDS) (9920/3) in 2015 followed by a 2020 diagnosis of myelodysplastic syndrome, NOS (MDS, NOS) (9989/3)? See Discussion. |
Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified. MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS. The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary. |
Abstract separate primaries using Rule M15 of the Hematopoietic and Lymphoid Neoplasms (Heme) Manual. The Heme Database states: Excluded from this category are progression of myeloproliferative neoplasms (MPNs) and evolution of primary MDS or primary MDS/MPN to acute myeloid leukemia (AML); in each of these latter cases evolution to AML is part of the natural history of the primary disease and it may be impossible to distinguish natural progression from therapy-related changes. There is no indication of transformation. |
2021 |
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20210056 | 2018 Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be reported when a left breast simple mastectomy identifies focal Paget disease of the nipple and 12 axillary nodes positive for metastatic lobular carcinoma (no primary lobular breast tumor identified)? |
Abstract two primaries, one lobular carcinoma (8520/3) and another one Paget disease of the breast (8540/3) using the 2018 Breast Solid Tumor Rules, Rule M9: Abstract multiple primaries when the diagnosis is Paget disease with underlying tumor which is NOT duct. Example: Paget disease of the nipple with underlying lobular carcinoma are multiple primaries. Additionally, Table 2, Histology Combination Codes, Note 2 states: Lobular carcinoma and Paget are separate primaries (see Lobular carcinoma and any histology in Table 3 with exception of duct carcinoma/carcinoma NST/DCIS (and subtypes/variants) 8500 and Paget disease, in situ and invasive). While not identified in the pathology of the mastectomy, the lobular carcinoma is likely underlying as it was identified in the axillary lymph nodes. The 2021 SEER Manual states: If the only pathologic specimen is from a metastatic site, code the appropriate histology code and the malignant behavior code (/3). The primary site and its metastatic site(s) have the same histology. |
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20210049 | Histology/Heme & Lymphoid Neoplasms--Leukemia: Is this the correct histology for a case of acute myeloid leukemia (AML) with recurrent genetic abnormalities? If the only information was AML with recurrent genetic abnormalities,"what code would you use: AML, NOS (9861/3) or AML with recurrent genetic abnormalities (9896/3)? See Discussion. |
12/3/2020 Pathology: AML: Blasts 40% of nucleated cells. CD45 positive, CD34 negative, CD 117+, CD13 positive, CD33 positive in 59.6% and HLA-DR was dim and myeloperoxidase was dim. Cytogenetics normal karyotype. The next generation sequencing detected IDH 2p.(R172K)c515>A. Because this was AML NOS, we consulted with the physician. The physician stated the patient had AML with recurrent genetic abnormalities"and the basis for the diagnosis was the IDH-2 mutation identified on Next Generation Sequencing. We assigned 9896/3, based on the physician's interpretation of the pathology. This histology is being questioned. |
We found that the term AML with recurrent genetic abnormalities, NOS"was incorrectly included as an alternate name with code 9896/3. We followed back with our expert hematopathologist and he stated that this should have been coded to 9861/3 (AML, NOS), for AML with recurrent genetic abnormalities, NOS. This alternate name has been added to 9861/3. (Note: The same alternate name has been removed from 9896/3). IDH-2 is not listed as a genetic abnormality for any of the histologies listed in the database. It could be that this is a new genetic marker for one of the AML with recurrent genetic abnormalities that we are not aware of. Without further clarification on which histology the IDH-2 would indicate, you would have to default to 9861/3. There are several histologies that are grouped as AML with recurrent genetic abnormalities."All of these have specific genetics listed as part of the ICD-O-3 histology name. 9865: Acute myeloid leukemia with t(6;9)(p23;q34.1) DEK-NUP214 9866: Acute promyelocytic leukemia with PML-RARA 9869: Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM 9871: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 9877: Acute myeloid leukemia with mutated NPM1 (2021+) 9878: Acute myeloid leukemia with biallelic mutation of CEBPA (2021+) 9879: Acute myeloid leukemia with mutated RUNX1 (2021+) 9896: Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1 9897: Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3 9911: Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 9912: Acute myeloid leukemia with BCR-ABL1 (2021)+ Of note, for the above histologies, since these are diagnosed solely based on genetics, diagnostic confirmation will always be 3. This instruction will be added to the Hematopoietic database for the 2022 update. |
2021 |
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20210012 | Solid Tumor Rules (2018, 2021/Multiple Primaries/)--Lung: How many primaries should be reported and what M rule applies when a diagnosis of presumed adenocarcinoma in situ (AIS) of the left lung follows a known diagnosis of progressive multifocal malignant adenocarcinoma in the right lung? See Discussion. |
Patient was initially diagnosed with a right lower lobe (RLL) lung adenocarcinoma in 2014 followed by subsequent right upper lobe (RUL) lung adenocarcinoma in 2016 (single primary). Both were treated with radiation and the nodules were seen as stable on surveillance. There was subsequent growth in the RUL nodule in 2019 and RLL nodule in 2020 as well as a new right middle lobe (RML) nodule in 2020. All left sided nodules were noted to be stable and/or ground glass opacities. There was no documented diagnosis of malignancy in the left lung until June 2020 when the physician noted that if there was a response in the left lung to systemic treatment, then this was probably multifocal AIS. However, only one tumor in the left lung responded to treatment. While it seems somewhat unlikely that only a single AIS in the contralateral lung should be metastasis from the right lung malignancy, it is difficult to apply the multiple tumors rules to this case. |
Abstract a single primary using 2018 Lung Solid Tumor Rule M9. The 2014 and 2016 R lung tumors were pathologically confirmed; it is not stated if they were resected. Follow up after XRT noted stable disease but no indication of NED. Subsequent right lung tumor is also the same primary. The issue is the assumed left lung adenocarcinoma in situ. It is not clear how long the left lung nodules were present, but they appeared to be stable as well and only diagnosed as a malignancy based on treatment response. At this time M9 applies and the left lung AIS is not a separate primary. We have discussed at length with lung pathology experts the issue of determining multiple primaries. Identifying and diagnosing lung tumors has become easier with new technology and the result is patients are being diagnosed with multiple lung tumors. Some lung experts feel we are under-reporting lung primaries but all noted the many issues with creating rules for consistency. |
2021 |
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20210043 | Reportability/Histology--Fallopian Tube: Is a diagnosis of serous tubal intraepithelial neoplasm (neoplasia) (STIN) equivalent to serous tubal intraepithelial carcinoma (STIC)? Does the designation of high or low grade have any effect on potential reportability? See Discussion. |
Patient has left salpingo-oophorectomy showing fallopian tube with focal high grade serous intraepithelial neoplasm. In reviewing some journal articles, the term STIN is being used to describe both STIC and serous tubal intraepithelial lesion (STIL). We will likely continue to see this term used, so it would be nice to have some clarity. |
Serous tubal intraepithelial neoplasm (neoplasia) (STIN) is not equivalent to serous tubal intraepithelial carcinoma (STIC). Report STIN only when stated to be high grade. STIC is reportable. Do not report STIL. According to our expert pathologist consultant, STIL and STIN are broad descriptive terms that reflect proliferation of epithelial cells with varying degrees of atypia, with the most developed, STIC, reflecting convincing neoplastic change. |
2021 |
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20210003 | Solid Tumor Rules (2018)/Primary Site--Head & Neck: The instructions for Table 9 of the Head and Neck Solid Tumor Rules instruct registrars to code the primary site to C479 (Autonomic nervous system) for paragangliomas that arise in the head and neck region, but the ICD-O-3.2 provides a site-associated code for most of these tumors (C754, Carotid body and C755, Paraganglion). Which primary site is correct? See Discussion. |
While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755. Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755. |
Always code primary site to the site of origin. Look for information about where the neoplasm originated. Primary site should always be coded to reflect the site of origin according to the medical opinion on the case. Always code the primary site based on where the tumor arose / site of origin. Site of origin may be indicated by terms such as "tumor arose from," "tumor originated in," or similar statements. Refer to ICD-O-3.2 and ICD-O-3 for topographty codes that are associated with specific histologies whenthe medical documentation does not specify the primary site. |
2021 |
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20210031 | Reportability--Brain and CNS: Are lipomas of the spinal column reportable as a benign tumor of the central nervous system (CNS)? This is seen occassionally at our pediatric facility. |
Spinal cord tumors (including lipomas) are reportable when they arise in the spinal dura or nerve root. The tumor must be of the spinal cord itself or within the spinal cord dura. Spinal cord tumors are reportable when they arise in the intradural space. A reportable intradural tumor can be either intramedullary or extramedullary. Extramedullary intradural spinal tumors are reportable. A spinal tumor originating in the extradural space is not reportable. If it is outside the dura, it is not reportable because it would be outside the CNS. They are not reportable when they arise in the peripheral nerves. |
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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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