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20240041 | Reportability--Brain and CNS: Is an optic nerve meningioma reportable if stated to arise in the “intraorbital segment” of the optic nerve meninges? See Discussion. |
Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma. Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location. It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable. |
Report optic nerve sheath meningioma arising in the intraorbital segment. The optic nerve contains four segments, of which intraorbital is one. The WHO Classification of Eye Tumors, 4th edition, defines meningioma as a neoplasm originating from the meningothelial cells of the optic nerve leptomeninges. According to the Table 3 of the Non-malignant Solid Tumor Rules, all portions of the optic are reportable and meningiomas arising in the dura/meninges of an intracranial nerve are coded to cerebral meninges C700. |
2024 |
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20240059 | Update to the Current Manual/SEER*RSA--SEER Site-specific Factor 1: The 2024 SEER Manual and SEER*RSA say that Codes 10-51 are hierarchical; use the highest code that applies (10 is highest, 51 is lowest). Should the guidance say that 51 is highest, 10 is lowest since code 10 is a HPV negative p16 test? |
Assign the highest code with 51 as the highest and 10 the lowest when there is more than one test performed with differing results. The SEER Manual will be updated in the 2025 release. SEER*RSA will be updated in a future release, version 3.3 in 2026, as version 3.2 has been updated for 2025. |
2024 | |
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20240060 | Solid Tumor Rules/Histology--Lung: What is the histology code for a lung case with a diagnosis only stated as "high-grade neuroendocrine tumor" in 2022? As the disease was advanced, limited workup was done, and no more specific diagnosis was provided. See Discussion. |
SINQ #20170064 states this should be coded as neuroendocrine carcinoma for rectum, but that may not apply for a 2018+ lung case. The Solid Tumor Manual lists "neuroendocrine tumor, grade 3" as 8249 in the Lung module, Table 3, but our pathology report does not specify grade 3 and we are unsure if that would be equivalent to "high grade" in this case. We were unable to find this exact term in the Solid Tumor Manual or the ICD-O-3.2 update documents. |
Assign 8249/3 for high-grade neuroendocrine tumor of the lung. WHO Classification of Thoracic Tumors, 5th edition, defines two subtypes of neuroendocrine tumor of the lung, typical carcinoids (8240/3), and atypical carcinoids. WHO assigns typical carcinoid/neuroendocrine tumor grade 1 as 8240/3 and atypical carcinoid/neuroendocrine tumor grade 2 as 8249/3. They are regarded as low-grade and intermediate-grade, respectively. The preferred term for 8249/3 in ICD-O-3.2 is neuroendocrine tumor grade 2, with neuroendocrine tumor grade 3 as a related term. The Lung Solid Tumor Rules assign atypical carcinoid as 8249/3. |
2024 |
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20240043 | Reportability/Histology--Digestive Sites: Is a diagnosis of “tubulovillous adenoma with high grade dysplasia” in the duodenum equivalent to a diagnosis of “tubulovillous adenoma, high grade” and, therefore, non-reportable, or is this a reportable non-colorectal high grade dysplasia? See Discussion. |
The 2022 ICD-O-3.2 Implementation Guidelines indicate “Tubulovillous adenoma, high grade” is 8263/2 and is not SEER reportable. However, the 2024 SEER Manual and clarification from recent SINQs (20240021 and 20240025) confirm high grade dysplasia in the esophagus, stomach, and small intestine is reportable (8148/2). Which reportability reference applies to a diagnosis of a tubulovillous adenoma with high grade dysplasia in non-colorectal sites? |
A diagnosis of “tubulovillous adenoma with high grade dysplasia” in the duodenum is not equivalent to a diagnosis of “tubulovillous adenoma, high grade.” Tubulovillous adenoma, high grade (8263/2) is not reportable as of 2022. High grade dysplasia (glandular intraepithelial neoplasia, grade III) is reportable in the esophagus, stomach, and small intestine (8148/2). |
2024 |
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20240015 | Solid Tumor Rules/Histology--Breast: Is ductal carcinoma in situ (DCIS), solid type coded as 8500/2 or 8230/2? See Discussion. |
In the NAACCR Coding Pitfalls 2023 webinar, the example of DCIS, solid type is given. The webinar advised us to code 8230/2 (ductal carcinoma in situ, solid type). When going through the beginning of the solid tumor rules in the Changes from 2007 MPH Rules section it states "DCIS/Carcinoma NST in situ has a major classification change. Subtypes/variant, architecture, pattern, and features ARE NOT CODED. The majority of in situ tumors will be coded to DCIS 8500/2." In the equivalent or equal terms section it lists "Type, subtype, variant" can be used interchangeably. Since the example has it listed as as ductal carcinoma in situ, solid "type," would we code 8500/2 or 8230/2? |
Assign 8230/2 (ductal carcinoma in situ, solid type/intraductal carcinoma, solid type) using Breast Solid Tumor Rules Table 3 as instructed in Rule H2 for in situ tumors. The carcinoma, NST row lists this histology in the subtype/variant column 3. Coding histology for in situ breast tumor differs from invasive. While the majority of in situ breast primaries will be coded to DCIS 8500/2, there are others that are listed in Table 3 that should be coded according to the specific histology. Some codes have the word subtype or type as part of their histologic term so these can be coded based on the histologic term as listed in the table. We suggest you routinely review the histology tables to see if a term is listed. |
2024 |
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20240050 | Solid Tumor Rules/Multiple Primaries--Vulva: Why is there no M Rule in the Other Sites Multiple Primary Rules related to extramammary Paget disease of the vulva? See Discussion. |
The only Other Sites H Rule related to extramammary Paget disease is included in the Multiple Tumors Abstracted as a Single Primary module. Rule H28 instructs one to code the histology of the underlying tumor when there is extramammary Paget disease and an underlying tumor of the anus, perianal region, or vulva. Therefore, a vulvar extramammary Paget disease with underlying adenocarcinoma is coded as adenocarcinoma (8140/3), and not extramammary Paget disease (8542/3). However, there is no M Rule confirming extramammary Paget disease and the underlying adenocarcinoma are a single primary (i.e., multiple tumors abstracted as a single primary) making it difficult for one to use the Multiple Tumors Abstracted as a Single Primary H rules module. We recognize this is a longstanding histology coding rule, but how are registrars supposed to arrive at Rule H28 when the M Rules must be applied first and do not instruct one to accession a single primary? Moreover, if this is to be a single primary (per rule M2), why is there no H Rule in the Single Tumor module? |
In sites other than breast (see Breast Solid Tumor Rules M8/M9), Paget disease with underlying invasive disease is a single primary and the underlying histology is coded. Primary Paget disease of the vulva is uncommon, and we cannot create rules for all possible situations in the Other Sites module. A GYN specific module is in development, and we will look into adding a Paget-related rule. It will differ because Paget in breast is a different situation while Paget in the vulva is always adenocarcinoma. Paget disease of the vulva is an in-situ adenocarcinoma of vulvar skin with or without an underlying adenocarcinoma (WHO Classification of Female Genital Tumors, 5th ed.). When there is a statement of “underlying” adenocarcinoma, it is a single primary as with Breast Solid Tumor Rule M8. |
2024 |
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20230027 | Solid Tumor Rules/Multiple Primaries--Peripheral Nerves: How many primaries should be abstracted, and which M Rule applies, when a malignant peripheral nerve sheath tumor (MPNST) in the right arm (C471) is followed greater than one year later by a separate malignant peripheral nerve sheath tumor of the thoracic chest wall (C473)? See Discussion. |
Since the peripheral nerves are included in the Malignant CNS schema of the Solid Tumor Rules, neither the differences in subsite nor timing indicate these are separate primaries (Rule M10 indicates a single primary). However, these are separate MPNSTs in different sites and the tumors are not stated to be metastasis. Additionally, these are treated as separate primaries by the managing physician. While the malignant CNS tumors do not take timing into account, is this correct for these peripheral nerve tumors that are often treated similarly to soft tissue tumors? Should Rule M8 be updated to include tumors in different peripheral nerve subsites? |
Abstract a single primary using Solid Tumor Rules, Malignant CNS and Peripheral Nerves, Rule M10 based on the information provided. Rule M10 applies as both non-contiguous tumors are of the same histology; i.e., on the same row in Table 3. As MPNST can arise in many sites, look for information about the precise location and tissue type in which the tumor arose. For example, if the tumors are stated to arise in soft tissue, follow the Multiple Primary Rules for Other Sites. Both WHO Classification of Central Nervous System Tumors and WHO Classification of Soft Tissue and Bone Tumors state that MPNST is a malignant spindle cell tumor often arising from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1). Future updates will move C470-C479 from CNS to other sites module. |
2023 |
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20230043 | Solid Tumor Rules/Histology--Lung: What is the histology code for a lung tumor diagnosed as “Minimally invasive adenocarcinoma, mixed mucinous and non-mucinous, grade 1, lepidic-predominant”? See Discussion. |
The resection pathology report final diagnosis indicates this is both mixed mucinous and non-mucinous with a lepidic predominant component. The pathologist notes this is “Lepidic: 75%. Acinar: 25%.” The percentage of the mucinous component is not documented. Rule H1, Note 1, states “When mucinous carcinoma is mixed with another histology, such as adenocarcinoma and mucinous carcinoma, code mucinous ONLY when mucinous is documented to be greater than 50% of the tumor.” While mixed invasive mucinous and non-mucinous carcinoma is included in Table 2 (Combination/Mixed Histology Codes) without a required percentage, it is unclear whether one should move past Rule H7 and use Rule H8 to code this combination histology code. Rule H7 would instruct one to code the histology to lepidic adenocarcinoma (adenocarcinoma, lepidic predominant) based on the percentage of the lepidic component in the tumor. However, this does not address the mixed mucinous and non-mucinous diagnosis. Which H Rule and histology apply to this case? |
Assign histology code 8254/3 (mixed invasive mucinous and non-mucinous adenocarcinoma) to this lung tumor using Lung Solid Tumor Rules, Rule H4. This is a new code/term approved by IARC/WHO for ICD-O. Rule H4 instructs one to code the histology when only one histology is present. In this case, the pathologist indicates the tumor is mixed mucinous and non-mucinous histologies. The non-mucinous carcinoma that is seen in this mixed histology may be identified as: Adenocarcinoma in situ, minimally invasive adenocarcinoma, or lepidic predominant adenocarcinoma. In this case it is lepidic predominant adenocarcinoma. Lepidic is a recognized histology in lung. It is not unusual for the pathologist to indicate mixed non-muncinous and mucinous adenocarcinoma AND also list the non-mucinous subytpe. It is important to capture both mucinous and non-mucinous histologies which drives treatment, etc. |
2023 |
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20230076 | Solid Tumor Rules/Histology--Prostate: How is histology coded and what rule applies to a diagnosis of “prostatic adenocarcinoma with neuroendocrine differentiation” with reference to the Comment: Immunohistochemical findings are consistent with amphicrine carcinoma for a patient with no prior androgen-deprivation therapy. See Discussion. |
The case in question represents an adenocarcinoma with neuroendocrine differentiation that arises in the absence of androgen-deprivation therapy. A 2023 journal article states, “We show that amphicrine prostate cancer is a unique entity and differs in clinical and molecular features from high-grade neuroendocrine carcinomas of the prostate. Our study highlights the need to recognize AMPC as a unique molecularly defined subgroup of prostate cancer.” Should we be coding this with histology 8140 (Adenocarcinoma, NOS) because we have no specific code for an amphicrine carcinoma? Should we code this as 8045 (Mixed small cell carcinoma) because this is possibly the only way to capture both the adenocarcinoma and neuroendocrine components in a patient without previous treatment? Our concern about using histology code 8574 (Adenocarcinoma with neuroendocrine differentiation) is that, while a valid histology code, this might confound the data if researchers are trying to separate the truly treatment-related tumors from other histologies captured under 8574. |
Assign 8140/3 (adenocarcinoma, NOS). WHO has not yet recognized the variant amphicrine prostate carcinoma and have not proposed an ICD-O code for this neoplasm. Document information in a related text field. |
2023 |
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20230030 | Primary site: Is there a physician priority list for coding primary site? For example, the surgeon states during a pancreatectomy that the primary is in body while the pathologist states in their synopitc report that primary is neck; neither is in agreement, or neither is available for confirmation. |
As a general rule, the surgeon is usually in a better position to determine the site of origin compared to the pathologist. The surgeon sees the tumor in its anatomic location, while the pathologist is often using information given to him/her by the surgeon and looking at a specimen removed from the anatomic landmarks. However, when a pathologist is looking at an entire organ, such as the pancreas, he/she may be able to pinpoint the site of origin within that organ. In the case of pancreas body vs. neck, the neck is a thin section of the pancreas located between the head and the body. It may be a matter of opinion whether a tumor is located in the "body" vs. the "neck." In the situation you describe, we would give preference to the surgeon and assign the code for body of pancreas, C251. |
2023 |
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