Report | Question ID | Question | Discussion | Answer | Year |
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20190042 | Solid Tumor Rules (2018)/Multiple Primaries--Breast: Is a breast resection showing invasive mucinous carcinoma in a single tumor with associated ductal carcinoma in situ and additional findings of a background of lobular carcinoma in situ single or multiple primaries and which M rule applies? See Discussion |
Example: Right breast core biopsy found ductal carcinoma in situ in the upper outer quadrant. Subsequent resection has a final diagnosis of invasive mucinous carcinoma, grade 1, measuring approximately 7 mm, with close margins. See staging summary. Gross description mentions only the primary tumor with associated marker clip from previous biopsy. Breast Cancer Staging Summary lists (testing and margins removed for brevity): Procedure type: Lumpectomy. Specimen laterality: Right. Tumor size: 7mm. Histologic type: Invasive mucinous carcinoma. Histologic grade (Nottingham histologic score): Grade 1, (score 5/9). Tumor focality: Single focus. Lymph-vascular invasion: Not identified. Treatment effect: No known therapy. Ductal carcinoma in situ (DCIS): Present. Architectural pattern: Cribriform. Nuclear grade: Grade 1. Necrosis: Not identified. Calcifications: Not identified. Estimated size/extent of DCIS: Spanning an area measuring 15mm. Pathologic stage: pT1b, pNx. (AJCC 8th ed). Distant metastasis: Not applicable. Additional findings: Background lobular carcinoma in situ (LCIS), flat epithelial atypia (FEA), and atypical ductal hyperplasia (ADH). |
Apply Breast Solid Tumor Rule M3, abstract a single tumor when there is a single tumor, as there is reference to the primary, single 7 mm tumor. Apply Rule H7 and code the invasive histology only, mucinous carcinoma, when both invasive and in situ components are present. The rules state: Do not use Table 2 Histology Combination Codes for tumors with both invasive and in situ behavior. |
2019 |
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20200072 | Solid Tumor Rules (2018)/Multiple Primaries--Breast: How many primaries are accessioned when there are multiple synchronous/non-contiguous tumors when one tumor is metaplastic carcinoma (with carcinoma No Special Type (NST) or lobular carcinoma) and another tumor is strictly carcinoma, NST? See Discussion. |
Is an M rule needed to address multiple tumors and Note 2 in Table 3? Does Note 2 in Table 3 apply when multiple tumors exist and one tumor contains only ductal carcinoma? The M Rules currently confirm that a metaplastic carcinoma (whether it is involved with ductal or lobular) and a separate ductal carcinoma are separate primaries because these histologies are on different rows in Table 3 (separate primaries per M14). There is no specific rule regarding metaplastic carcinomas in the Multiple Tumors (M Rules) module, so presumably, the presence of a separate ductal carcinoma is not lumped into Note 2 in Table 3 for metaplastic carcinoma. However, the note is confusing when there are multiple tumors involved because it appears to the registrars there are two options for coding the histology. To some registrars, the rules indicate it does not matter if the tumor is predominantly ductal carcinoma as long as some percentage of metaplastic carcinoma is present, code histology to metaplastic carcinoma. For other registrars, the presence of solely a ductal carcinoma in a second tumor is a separate primary from the separate metaplastic carcinoma. The M rules and Note 2 need to clarify this issue to promote consistency. This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
The term "mixed" implies a single tumor comprised of metaplastic carcinoma or variants of metaplastic and duct or lobular. The metaplastic histology is coded regardless of whether it comprises the majority (greater than 50% of the tumor). M13 is the only rule specific to metaplastic and is in the single tumor module. This implies a single tumor with both histologies. When there are multiple tumors, one with metaplastic or a subtype/variant of metaplastic and another with a histology listed on a different row, continue to the Multiple Tumors module. M13 applies and there are two primaries. We will add "single tumor" to the note in Table 2 in the next update. |
2020 |
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20180087 | Solid Tumor Rules (2018)/Multiple Primaries--Brain: How many primaries are there and what M Rule applies when two tumors identified in the brain are pathologically proven to be glioblastoma, IDH-wild type and anaplastic astrocytoma per the pathology report final diagnosis, but the diagnosis comment and tumor board indicates multifocal glioblastoma is favored? See Discussion. |
The patient has one tumor each in the left parietal and left medial temporal lobe. The tumors were excised. The final diagnosis for the left parietal tumor is glioblastoma, IDH-wild type. he final diagnosis of the left medial temporal tumor is, at least anaplastic astrocytoma, WHO grade III; see comment. The comment states: There is a single focus of vascular hyperplasia, separate from neoplastic cells. No necrosis is identified. These findings on their own would warrant a diagnosis of anaplastic astrocytoma, WHO grade III. However, in the context of the patient's glioblastoma in the left parietal lobe, and imaging showing ring-enhancing lesions of the parietal and temporal lobes, this specimen is favored to be an un-sampled glioblastoma, WHO grade IV. The Solid Tumor Rules indicate we may no longer use terms like favor(s) to code the histology, leaving the final diagnosis as the priority source for coding histology per the Histology coding rules. The tumor board review confirmed that, despite the anaplastic astrocytoma on pathology, they felt strongly that this is a multifocal glioblastoma and not an anaplastic astrocytoma. Both the pathologist's comment and the tumor board's assessment indicate this patient does not have two primaries. However, the Solid Tumor Rules do not give priority to the tumor board's assessment over the pathology, and registrars are not to use ambiguous terms to code histology thus leaving the two histologies to consider. Per the Solid Tumor Rules, one tumor that is glioblastoma and one tumor that is anaplastic astrocytoma are multiple primaries per M11 (Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant). As a central registry, we cannot ask the pathologist or attending physician for clarification as suggested in Section 3 of the Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions. We can only follow the current Solid Tumor Rules. In doing so, we would have to ignore both the pathologist's and tumor board's assessment that this patient has multifocal glioblastoma. Is there any concern that this will lead to over-reporting? |
Abstract separate primaries based on the two histology codes as these are separate tumors on different rows in Table 3 of the 2018 Solid Tumor Rules for Malignant CNS, Rule M11. The priority order for using documentation to identify histology for Malignant CNS is to use pathology/tissue from the resection over the tumor board. |
2018 |
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20180066 | Solid Tumor Rules (2018)/Laterality--Brain and CNS: How is laterality coded for bilateral non-malignant central nervous system (CNS) or malignant CNS tumors now that laterality is no longer used to identify these tumors as multiple primaries? See Discussion. |
The Equivalent Terms and Definitions sections in the Solid Tumor Rules for these schemas identify which sites must have laterality coded, but there is no instruction for coding laterality when bilateral tumors are a single primary. The SEER Manual currently only indicates code 4 (bilateral) is seldom used (e.g., bilateral ovarian tumors, Wilms tumors, etc.) but does not indicate laterality code 4 should be used for CNS tumors. Is this note going to be updated or should a non-bilateral code be applied? Example: MRI demonstrates multiple left-sided dural-based meningiomas including a 4.4 cm left posterior fossa meningioma, a 0.8 cm left frontal-parietal meningioma and a right posterior frontal meningioma. The large left posterior fossa meningioma was resected and proved atypical meningioma. Should the laterality be 4 (bilateral) as the patient had both left and right-sided meningiomas confirmed to be a single primary? Or should the laterality be coded as 2 (left) since only the large left-sided meningioma was proven to be a borderline tumor (atypical meningioma, 9539/1) and the others were benign? |
Determine whether the CNS tumors are single or multiple primaries. Multiple cerebral meningiomas are a single primary according to the non-malignant CNS Solid Tumor Rules. Assign laterality using the 2018 SEER Manual for select invasive, benign, and borderline primary intracranial and CNS tumors using codes 1-9 for all sites listed in the Sites for Which Laterality Codes Must Be Recorded table. In the example, assign code 4, bilateral involvement at time of diagnosis, lateral origin unknown for a single primary. The solid tumor rules are not a one-stop-shop for all coding. Refer to the appropriate coding manual for laterality. We removed laterality for determining multiple primaries in meningiomas as they were being over-reported according to CBTRUS. |
2018 |
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20180098 | Solid Tumor Rules (2018)/Histology: Please provide further explanation for prioritizing biomarkers in the histology coding rules. See Discussion. |
The 2018 Solid Tumor (ST) Rules General Rules state: For those sites/histologies which have recognized biomarkers, the biomarkers frequently identify the histologic type. Currently there are clinical trials being conducted to determine whether these biomarkers can be used to identify multiple primaries. Follow the Multiple Primary Rules; do not code multiple primaries based on biomarkers. Additionally, Biomarkers is at the top of the priority order to identify histology in several sections (it appears to be excluded from only Colon, Melanoma and Other sections). In the sections that include this rule, there is not much additional information on using biomarkers. Can you please provide further explanation for prioritizing biomarkers in the histology coding rules? For example, will the ST manual be updated when we need to look for specific biomarkers in a diagnosis? |
Instructions for biomarkers will be added to other site rules when applicable. The use of biomarkers to determine a specific histologic type is not yet a standard of care in the majority of cases. |
2018 |
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20200087 | Solid Tumor Rules (2018)/Histology--Thyroid: What is the correct histology code for a micropapillary thyroid carcinoma for cases diagnosed 1/1/2021 and later? See Discussion. |
The 2021 ICD-O-3.2 Update includes papillary microcarcinoma (8341/3) as the preferred term for thyroid primaries (C739). However, there are multiple SINQ entries instructing registrars not to use code 8341/3 for diagnoses of micropapillary carcinoma of the thyroid (including SINQ 20071076, 20081127, 20110027, 20150023, and 20180008). SINQ 20150023 specifically indicates: Per the WHO Tumors of Endocrine Organs, for thyroid primaries/cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult (1 cm or less in diameter) and was found incidentally. WHO does not recognize the code 8341 and classifies papillary microcarcinoma of the thyroid as a variant of papillary thyroid carcinoma and codes histology to 8260. If the primary is thyroid and the pathology states papillary microcarcinoma or micropapillary carcinoma, code 8260 is correct. Does this clarification apply to cases diagnosed 2021 and later? If WHO feels the term micropapillary still does not refer to a specific histologic type for the thyroid, why is 8341/3 listed as a preferred term for this morphology/site combination? For cases 2021 and later, should a diagnosis of Incidental papillary thyroid microcarcinoma (3 mm) in left lower pole, be coded as 8341/3 per the ICD-O-3.2, or as 8260/3 per clarification in multiple SINQ entries? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
Continue to code micropapillary thyroid carcinoma to 8260/3 until instructed otherwise. This coding instruction is based on input from expert endocrine pathologists. This issue will be revisted based on the 4th Ed WHO Endocrine Tumors and updated if needed. |
2020 |
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20190063 | Solid Tumor Rules (2018)/Histology--Sarcoma: How is histology coded for a CIC gene rearrangement sarcoma? See Discussion. |
According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded? |
Code as undifferentiated round cell sarcoma (8803/3). The CIC rearrangement exists as a distinct molecular and clinical subset of small round cell tumors, and though similar, is felt to be a distinct entity from Ewing sarcoma. According to WHO Classification of Soft Tissues and Bone, 4th Edition, CID-DUX4 is a recurrent gene fusion associated with pediatric round cell undifferentiated soft tissue sarcoma (USTS). Although the genes involved in the fusion are different from those in Ewing sarcoma, the CIC-DUX4 protein has been shown to upregulate genes of the ETS family of genes thus providing a molecular link between Ewing sarcoma and round cell USTS. In contrast, there are strong arguments to suggest that Ewing-like sarcomas represent a separate and distinct entity. |
2019 |
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20200052 | Solid Tumor Rules (2018)/Histology--Prostate: How is the histology coded for a diagnosis of mixed prostatic adenocarcinoma (5%) and small cell neuroendocrine carcinoma (95%) from a transurethral resection of the prostate? See Discussion. |
Following the existing Solid Tumor Rules Histology Rules, it would seem this is a single primary with histology 8045 (Combined small cell carcinoma) because there is no indication there are multiple prostate tumors and Table 2 states combined adenocarcinoma and small cell carcinoma is Combined small cell carcinoma (8045). Conversely, while not an exact match to this case, SINQ 20190083 implies small cell carcinoma and adenocarcinoma of the prostate are separate primaries. In that SINQ case, the patient was simultaneously diagnosed with metastatic small cell carcinoma of the prostate on a liver biopsy and prostate adenocarcinoma on a prostate biopsy. There is no indication that patient had separate tumors in the prostate, however the SINQ instructs to code as separate primaries. Would the previous SINQ logic apply to synchronous diagnoses in the prostate as well? Or does code 8045 apply to this situation? |
Assign histology code 8045 for combined small cell carcinoma as this represents one tumor with mixed histologies using the 2018 Other Sites Solid Tumor Rules, Rule H16. |
2020 |
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20200081 | Solid Tumor Rules (2018)/Histology--Pancreas: How is the histology coded, and what H Rule applies, for a 2021 diagnosis when the pathological diagnosis is neuroendocrine tumor (NET) G1 or NET G2, but clinically, the tumor is stated to be insulinoma? See Discussion. |
Insulinoma, NOS is reportable for cases diagnosed 2021 and later. However, the diagnosis of insulinoma is most frequently made with clinical correlation of the patient's clinical syndrome and serum hormone levels. Despite a pathological diagnosis of NET, this will clinically be stated as insulinoma based on the functional type of tumor. At the largest facility in our area, all pathology reports with a diagnosis of insulinoma over the last year only provide a pathological Final Diagnosis of NET (either G1 or G2), but elsewhere specify, Functional Type: Pancreatic neuroendocrine tumor, functional. Correlation with Clinical Syndrome and Elevated Serum Levels of Hormone Product: Insulin-producing (Insulinoma). For 2021 and later, it seems this should be accessioned as insulinoma (8151/3), but one cannot arrive at that histology using the current Other Sites (MP/H) H Rules. Following the existing rules, one would code the histology to NET, G1 or NET, G2 (8240 or 8249) per Rule H6. There are technically two specific histologies to consider: NET (either 8240 or 8249) and insulinoma, NOS (8151). Following the H Rules, Rule H6 instructs one to code the histology with the numerically higher ICD-O-3 code (8240 or 8249). Coding this histology to NET (8240 or 8249) does not seem to reflect the most accurate classification of this tumor, but applying the current rules, this is the only histology that can be coded. There is no current guideline in the Other Sites schema or the ICD-O-3.2 Implementation Guidelines instructing us to ignore the pathological diagnosis of a NET for these tumors (even though insulinomas are NETs). The only SINQ that currently exists (SINQ 20150019) states the histology can be coded as either a NET or an insulinoma in these cases. How are registrars to consistently code histology for these tumors without a rule clarification? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
Code the tissue/pathology histology over the clinical diagnosis. Because of implementation timelines, a comprehensive revision to Other Sites rules will not be available 2022. A limited revision is planned and histology tables will be added for select sites. The General Instructions will also be revised for Other Sites. |
2020 |
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20190039 | Solid Tumor Rules (2018)/Histology--Lung: What is the histology code of invasive moderately differentiated adenocarcinoma, predominantly papillary subtype, with minor acinar and lepidic subtypes? See Discussion. |
11/01/2018, lung, left upper lobe, wedge resection: Invasive moderately differentiated adenocarcinoma, predominantly papillary subtype, with minor acinar and lepidic subtypes. Would this be 8260/3 since the acinar and lepidic subtypes are described as minor or would this be 8255/3 because there is papillary plus two other subtypes/variants described as subtypes? |
Code as adenocarcinoma, papillary predominant (8260/3) according to the Lung Solid Tumor Rules, Coding Multiple Histologies, which says to code the specific histology. The most specific histology may be described as component, majority/majority of, or predominantly, where predominantly describes the greater amount of tumor. |
2019 |