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20230066 | Solid Tumor Rules/Histology--Lung: Table 3 in Lung Solid Tumor Rules, 2023 Update, lists neuroendocrine carcinoma, NOS 8246 as a specific subtype/variant for small cell carcinoma 8041/3. Should the table be updated? See Discussion. |
Small cell carcinoma is a specific type of neuroendocrine carcinoma for the lung. However, Table 3 lists neuroendocrine carcinoma, NOS as the more specific subtype/variant in Column 3. Using Lung Solid Tumor Rules, Rule H6, a diagnosis of poorly differentiated neuroendocrine carcinoma (small cell carcinoma)” would be coded as 8246, instead of 8041, because there are two histologies under consideration (an NOS and a subtype/variant in Table 3), and the rule tells us to code the subtype/variant. However, small cell carcinoma is more specific than the NOS diagnosis (neuroendocrine carcinoma, NOS). Should Table 3 be updated to reflect which histology is the NOS and which is the more specific? |
The Solid Tumor Rules for Lung have been updated for 2024. The row for Small cell carcinoma 8041/3 has been deleted and new separate rows have been added for Neuroendocrine carcinoma (NEC) 8246 and Neuroendocrine tumor, NOS (NET) 8240. This change is based on the WHO Classification of Thoracic Tumors, 5th edition, and current concepts. In addition, Table 3 now reflects that Small cell carcinoma/small cell neuroendocrine carcinoma 8041 (located in Column 3) is a subtype/variant of neuroendocrine carcinoma, NEC 8246 (Column 1). As a result, application of Rule H6 to a diagnosis of poorly differentiated neuroendocrine carcinoma (small cell carcinoma)” would be coded as 8041, instead of 8246. Please note: the 2024 updates may be used for cases diagnosed prior to 1/1/2024 unless otherwise noted in the rules. |
2023 |
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20230072 | Solid Tumor Rules/Multiple Primaries--Bladder: How many primaries and what M Rule applies to a diagnosis of non-invasive urothelial carcinoma of the bladder in 1996, followed by multifocal non-invasive papillary urothelial carcinoma involving bladder, prostatic urethra, and left ureter in 2022? See Discussion. |
An argument could be made to apply Rule M10 (timing rule which may result in reporting the case as an additional primary) because the 2022 primary included multiple non-invasive urothelial carcinoma tumors in both the bladder and other urinary sites (coded to site C689, not C679) following a long disease-free interval. While Rule M10 excludes multiple bladder tumors, does that also apply when new, multifocal urothelial tumors arise in both bladder and other urinary sites? Does the presence of any subsequent bladder tumor rule out the use of M10 and one must use M11 that indicates reporting this disease process is a single primary? |
Abstract as a new primary per rule M10, as the subsequent tumors are not limited to the bladder. Code the primary site to C689, per Instructions for Coding Primary Site, #4: "Code Urinary System NOS C689 when there are multiple non-contiguous tumors in multiple organs within the urinary system", and following Note: "The physician subject matter experts (SME) discussed the issue of coding primary site for multifocal/multicentric urinary tract carcinoma. Although the SMEs understood and acknowledged the importance of coding a specific primary site, there is no literature or criteria for determining the organ of origin for multiple tumors involving multiple urinary sites". |
2023 |
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20230080 | Solid Tumor Rules/Histology--Brain and CNS: What is the histology code for low grade glioma? See Discussion. |
Patient has a 3 cm tumor in the temporal lobe of the brain. This was noted on MRI 12/2022. The radiologist states this is a low-grade glioma and recommends following with routine scans. No pathology or resection performed or planned. Patient has been followed with imaging every six months with stable disease. Low grade glioma is not currently listed in ICD-O-3.2 or the current Solid Tumor Rules. What histology should be assigned to the case? |
Assign 9380/1 for low grade glioma diagnosed 1/1/2018 forward and for low grade glioma diagnosed prior to 1/1/2018 assign code 8000/1 on the advice of our expert neuropathologists. The site/type combination of C71 _ and 9380/1 will flag histology/site/behavior edits which should be overridden. Low grade glioma is an umbrella term or non-specific diagnosis, primarily seen on radiologic reports such as CT scans and MRIs. Often, the patient is actively followed with scans and surgical intervention delayed or not recommended. WHO Classification of Central Nervous System Tumors, 5th edition, does not recognize this term and indicates that tissue diagnosis (including genetic testing) is needed to provide a specific diagnosis. Since biopsy of these “neoplasms” is not routinely done, a definitive diagnosis is not available. Literature searches yielded conflicting information with some stating low grade gliomas are malignant with an indolent clinical course while others felt they were benign. Until such time as WHO proposes a code for this neoplasm, our expert neuropathologists recommend coding glioma, NOS with borderline behavior 9380/1. |
2023 |
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20230008 | SEER Manual/Surgery of Primary Site 2023--Breast: What instructions should be followed when the 2023 SEER Manual Appendix C 2023 Breast Surgery Codes advise to code 1 in Surgical Procedure of Other Site for a simple bilateral mastectomy but the 2023 STORE Manual does not. See Discussion. |
The 2023 SEER Manual, Appendix C 2023 Breast Surgery Codes, note reads: SEER Note: Assign code A760 for a more extensive bilateral mastectomy. Assign code 0 in Surgical Procedure of Other Site (NAACCR #1294). For a simple bilateral mastectomy, assign code A410 with code 1 in Surgical Procedure of Other Site (NAACCR #1294). In the 2023 STORE Manual, these notes are not mentioned and we are instructed not to code surgery to other site. Other education related to 2023 breast coding provided by NAACCR states to not code surgery to other site. |
Assign code 1 in Surgical Procedure of Other Site (NAACCR #1294) when a simple bilateral mastectomy is performed for a single tumor involving both breasts. This statement was inadvertently omitted from the STORE manual and will be added back in: For single primaries only, code removal of contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item #1294) or Surgical Procedure/Other Site at This Facility (NAACCR Item #674). The information presented by NAACCR was intended to be consistent with what is in the SEER manual. It may have been misuderstood. |
2023 |
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20230013 | Reportability/Histology--Skin: Is dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous overgrowth, DFSP with fibrosarcomatous component Grade 2, or DFSP with focal myxoid features (2022) reportable for 2021-2022 diagnoses? |
Yes. DFSP with fibrosarcomatous overgrowth and DFSP with fibrosarcomatous component Grade 2 are synonymous with fibrosarcomatous DFSP (8832/3). Our expert pathologist also advises that DFSP with focal myxoid features is the same as DFSP, myxoid (8832/3). |
2023 | |
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20230038 | Histology/Heme & Lymphoid Neoplasms--Mycosis Fungoides: What is the histology code for lymphomatoid papulosis that transforms initially to mycosis fungoides (MF)/cutaneous T-cell lymphoma (CTCL) and subsequently to CTCL with large cell transformation, and is it a new primary? See Discussion. |
Disease History 2018 - Lymphomatoid papulosis (non-reportable) 2020 - Transform to CTCL (and called Mycosis Fungoides specifically) (CTCL/MF same primary) 2021 - Transform to CTCL with large cell transformation |
Abstract a single primary and assign code 9700/3 for MF. According to our subject matter expert, this is all MF. When MF progresses, there can be large cd30 positive T cells. This is not the same as anaplastic large cell lymphoma. |
2023 |
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20230039 | Histology/Hematopoietic and Lymphoid Neoplasms--AML: What is the histology code for Acute Myelogenous Leukemia (AML) with monocytic differentiation, 9891/3: acute monoblastic and monocytic leukemia or 9867/3: Acute myelomonocytic leukemia? |
Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist. Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned. Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3. |
2023 | |
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20230045 | Reportability/Histology--Thyroid: Is a diagnosis of “angioinvasive oncocytic thyroid neoplasm with features worrisome for a poorly differentiated oncocytic carcinoma” reportable if the diagnosis comment states, additional immunostains were performed which demonstrate the carcinoma cells are positive for thyroglobulin and negative for calcitonin? See Discussion. |
Patient had a right thyroid lobectomy on 12/2022, with initial diagnosis of “thyroid carcinoma pending expert consultation for definitive classification.” The slide review documented in the addendum shows a final diagnosis of “Angioinvasive oncocytic thyroid neoplasm, see comment.” The subsequent comment states, “I would classify this lesion as an angioinvasive oncocytic thyroid neoplasm with features worrisome for a poorly differentiated oncocytic carcinoma.” The comment goes on to state, “Additional immunostains were performed which demonstrate the carcinoma cells are positive for thyroglobulin and negative for calcitonin. The diagnosis remains unchanged.” |
Do not report angioinvasive oncocytic thyroid neoplasm with features worrisome for a poorly differentiated oncocytic carcinoma based on the final, unchanged diagnosis. Worrisome is not a reportable ambiguous terminology. |
2023 |
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20230046 | Reportability/Histology--Tongue: Is high grade squamous dysplasia of the tongue reportable; and is it the same as carcinoma in situ (CIS), code 8077/2? |
High grade squamous dysplasia of the tongue is reportable as of 2021 and later as 8077/2. |
2023 | |
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20230011 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries are accessioned when a 2023 liver biopsy diagnosed metastatic small cell carcinoma (SmCC) of the prostate following a 2018 radical prostatectomy treated diagnosis of prostatic adenocarcinoma? See Discussion. |
SINQs 20190083, 20180088, and 20130221 all indicate diagnoses of prostate adenocarcinoma, followed by a diagnosis of metastatic small cell carcinoma of the prostate are separate primaries because these are distinctly different histologies. Does this logic still apply for 2023 and later since Rule M4 was added to the Other Sites M Rules? Rule M4 states, “Abstract multiple primaries when the patient has a subsequent small cell carcinoma of the prostate more than 1 year following a diagnosis of acinar adenocarcinoma and/or subtype/variant of acinar adenocarcinoma of prostate.” This patient has a 2018 diagnosis of prostate adenocarcinoma treated with radical prostatectomy, followed by a 2023 diagnosis of metastatic small cell carcinoma of the prostate diagnosed on a liver metastasis core biopsy. Rule M4 does not indicate whether it applies to subsequent biopsy confirmed metastatic tumor only. When a diagnosis of small cell carcinoma follows a diagnosis of prostatic adenocarcinoma, it is almost always confirmed in metastatic sites rather than in the primary site. Does the logic in the referenced SINQs above still apply for Rule M4? |
Accession two primaries, adenocarcinoma (8140/3) of the prostate and SmCC (8041/3) of the prostate using Rule M4 of the current Other Sites Solid Tumor Rules. The guidance in the aforementioned SINQ entries still applies with the additional criteria of being diagnosed more than one year following the diagnosis of acinar adenocarcinoma, or subtype, of the prostate as stated in Rule M4 of the updated 2023 rules. Small cell carcinomas of the prostate are often diagnosed on follow-up TURP/biopsies; however, if a patient had a previous radical prostatectomy, the small cell carcinoma would be identified in a metstatic site and would still be a new prostate primary. This includes biopsy confirmed metastatic tumors only. It remains important to capture the two distinct histology types. |
2023 |
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