| Report | Question ID | Question | Discussion | Answer | Year |
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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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20190005 | Primary Site--Bladder: Does instruction #4 in the Urinary Sites Solid Tumor Rules Instructions for Coding Primary Site apply to a mix of in situ and invasive urothelial tumors? Instruction #4: Code Urinary System NOS C689 when there are multiple non-contiguous tumors in multiple organs within the urinary system. See Discussion. |
Example: Patient has multiple biopsies with final diagnosis of in situ papillary urothelial carcinoma in the prostatic urethra and invasive papillary urothelial carcinoma in the bladder. How should primary site be coded in this type of mixed in situ and invasive situation? |
Code Urinary System NOS C689 for this case since there are two separate urinary sites involved. Apply instruction #4 when there is a mix of in situ and invasive urothelial tumors. |
2019 |
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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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20190026 | Solid Tumor Rules (2018)/Multiple primaries--Bladder: Does Rule M11 in the 04/2019 Solid Tumor Rules Urinary update apply to synchronous/simultaneous tumors only or to multiple tumors with any timing? See Discussion. |
Rule M11 states: Abstract a single primary when there are urothelial carcinomas in multiple urinary organs, but neither the Rule nor the Notes describe the timing of these multiple urinary organ carcinomas. Timing requirements for other rules are clearly stated. Does Rule M11 have a timing requirement or is it intended to apply to all urothelial carcinoma tumors regardless of timing (and not already qualifying for application of a previous M rule)? |
The revised Urinary Solid Tumor Rules 2018 Rule M11, updated April 2019, removed the requirement of synchronous. This applies to urothelial carcinoma (8120) and its corresponding subtypes, regardless of behavior, that occur in more than one urinary site in a patient's lifetime. See change log for the April 2019 update to urinary rules.This is the same M/PH rule for multiple sites. Timing does not factor in to this rule. |
2019 |
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20190021 | Sequence Number Central--Brain and CNS: How is Sequence Number--Central coded for current/recent benign brain/CNS tumors when the patient has a history of an additional non-malignant CNS tumor diagnosed prior to 2004 (when these tumors became reportable to SEER)? See Discussion. |
We are confused by the SEER Program Coding and Staging Manual 2018 instruction that states: This sequence number counts all tumors that were reportable in the year they were diagnosed even if the tumors occurred before the registry existed or before the registry participated in the SEER Program. Does this rule apply to benign and borderline CNS tumors? Does this mean that any non-malignant CNS tumor diagnosed prior to 2004 should NOT be included in the sequencing (in the 60s range) if we were collecting non-malignant CNS per our State Registry reporting requirements prior to 2004? Example: Patient has a March 2017 diagnosis of right sided vestibular schwannoma (C724-1, 9560/0) and a prior history of left sided acoustic neuroma (c724-2, 9560/0) diagnosed in 1991. How should sequence be coded for each primary in our file? |
For your example, code the Sequence Number--Central as 61 for the 1991 diagnosis if this was a state registry requirement in 1991 and code 62 for the 2017 diagnosis. |
2019 |
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20190098 | Solid Tumor Rules (2018)/Multiple primaries--Breast: How many primaries are there and how is histology coded for a breast primary showing encapsulated papillary carcinoma and Paget disease of the nipple? See Discussion. |
Patient has a 1.7 cm encapsulated papillary carcinoma staged as pTis located 2 cm from the nipple and Paget disease of the nipple on mastectomy pathology. There is no indication in Table 3: Specific Histologies, NOS/NST, and Subtypes/Variants that encapsulated papillary carcinoma is a subtype of ductal carcinoma. Rule M8 notes that if the histology of the underlying tumor is any histology OTHER THAN duct or subtypes of duct, one should continue through the rules. But if M9 applies to this case, then incidence reporting will be increased in comparison to prior years. |
Abstract multiple primaries when there is Paget disease (8540/3) and an underlying tumor that is not duct, in this case, encapsulated papillary carcinoma (8504/2) using Rule M9 of the 2018 Breast Solid Tumor Rules. |
2019 |
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20190088 | Surgery of Primary Site/Surgical Procedure of Other Site--Breast: When bilateral nipple/skin sparing mastectomies are performed for a single primary confined to one breast, we should code 30 for surgery and 0 for Surgery of Other Site or follow the CAnswer Forum and code 1 in Surgery of Other Site? See Discussion. |
Registrars are confused because the STORE manual dropped "involved" from the description of contralateral breast removal in the Appendix B surgical codes. In April, 2019, CAnswer Forum instructed registrars to code both the surgery with uninvolved breast to the proper code, plus code Surgery of Other Site to 1. In October, they stepped back and instructed registrars not to code Surgery of Other Site to 1 if a code for uninvolved breast removal is included in the breast surgery code. However, they insist that if the surgery code is 30, subcutaneous mastectomy, and the uninvolved contralateral breast is also removed, then continue to code Surgery of Other Site to 1. This contradicts the specific instructions for Surgery of Other Sites. |
For single primaries only, code removal of involved contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item # 1294), this is, code 1, according to the 2018 SEER Manual: Assign code 1 When the involved contralateral breast is removed for a single primary breast cancer This would also apply when Surgery of the Primary Site code is 30 (subcutaneous mastectomy) for breast. If uninvolved, assign code 0 to Surgical Procedure of Other Site SEER registries should follow the instructions according to the SEER Manual. |
2019 |
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20190040 | Reportability--Heme & Lymphoid Neoplasms: Is peripheral blood with a diagnosis of monoclonal B-cell lymphocytosis (MBL) with chronic lymphocytic leukemia (CLL) phenotype reportable for any year? See Discussion. |
SINQ 20180050 and 20130041 appear to have conflicting answers regarding the reportability of MBL with CLL (immuno)phenotype. While the question content of SINQ 20180050 does not reference the CLL phenotype, it is included in the Discussion as part of the oncologist's assessment. The answer does not address the clinical diagnosis of MBL with CLL-phenotype and simply states that monoclonal B-cell lymphocytosis is not reportable. SINQ 20130041 does include the CLL phenotype information in the primary question and it is expanded on in the discussion as present in peripheral blood. Based on that information, the answer is that it should be reportable and coded as CLL (9823/3). |
The description in the question is for 9823/1 per WHO blue book 2016. This description and code are not reportable. We will review the other SINQ questions and revise if necessary. |
2019 |
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20190097 | Solid Tumor Rules (2018)/Multiple primaries--Lung: How many primaries are there and what M rules apply for multiple lung histologies in the left lower lobe (LLL) and right upper lobe (RUL) of the lungs? See Discussion. |
There is one tumor in the left lung that is acinar adenocarcinoma, 8551/3, and two tumors in the right lung, one of which is 8551/3 and a separate one that is mucinous adenocarcinoma 8253/3. 3/21/18- left robotic video assisted thoracoscopy with left lower lobe lobectomy: 2.5 cm adenocarcinoma, acinar predominant, margins negative 11/3/18- right upper lobe lobectomy: invasive mucinous adenocarcinoma, 1.7 cm, invasive adenocarcinoma, acinar predominant, 0.6 cm, margins negative If you start by comparing the 8551/3 left lung tumor to the 8253/3 right lung tumor, M6 applies and these would be separate primaries (seq 01 and seq 02). How would we handle the third tumor, 8551/3, in the right lung? Seq 01: 3/21/18- left lung primary 8551/3 Seq 02: 11/3/18- right lung primary 8253/3 Is the right lung tumor 8551/3 a third primary, and if so, which M rule applies? I cannot find a rule that seems to fit completely. Rule M6 may apply if you were comparing the right 8551/3 tumor to the seq 02 8253/3 tumor. But how would you know to use the seq 02 histology code 8253/3 or seq 01 histology code 8551/3 for the comparison? I think M9 was designed for situations where you have multiple tumors involving both lungs but they didn't biopsy all of them. Is that correct? If so, then we would be able to bypass M9. Would M11 apply since we already took care of two of the tumors with rule M6? If M11 doesn't apply, it seems like you would get to M14. |
Abstract two primaries applying Rules M6 and M9 s follows. First, assign a histology for each tumor. --LLL adenocarcinoma, acinar predominant 8551/3 --RUL invasive mucinous adenocarcinoma 8253/3 --RUL invasive adenocarcinoma, acinar predominant 8551/3 For the RUL, this is two primaries according to Rule M6, to subtypes in Column 3 of the histology table. For the LLL and RUL, this represents the same primary as these are the same histology according to Rule M9. |
2019 |
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20190041 | Reportability/Primary Site--Gastrointestinal (GI) Tract: Is a gastrointestinal stromal tumor (GIST) with a single nodule in the small intestine (C17_) and a nodule in the stomach (C16_) reportable per the 2018 SEER Coding Manual reporting instructions for GIST due to the multiple foci or do the multiple foci need to be in the same organ to be reportable? See Discussion. |
Example: Small intestine wedge resection with GIST, 1.8 cm in mid small intestine, single nodule. Stomach nodule biopsy: GIST, 0.3 cm. Pathology report comment section indicates the gastric GIST is not staged due to the small size and incidental nature. |
Report the GIST in the small intestine. The 2018 SEER Manual says to report GIST when there are multiple foci and to code the primary site to the site where the malignancy originated. Use text fields to record the details, including the stomach nodule. |
2019 |
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