Report | Question ID | Question | Discussion | Answer | Year |
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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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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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20240032 | Update to Current Manual/Reportability--Biliary Tract: Is a diagnosis of high grade dysplasia of the gallbladder reportable? See Discussion. |
Patient was diagnosed March 2024 with high grade dysplasia of the gallbladder during excision for clinical history of acute cholecystitis and obstruction. Per the STR, Table 10 for Gallbladder and Extrahepatic Bile Duct Histologies shows Biliary intraepithelial neoplasia, high grade as code 8148/2. High grade glandular intraepithelial neoplasia of the biliary tract is also code 8148/2. Recent SINQ 20240021 (GI specific) indicates high grade dysplasia is reportable as high grade glandular intraepithelial neoplasia (8148/2) for stomach, small intestine, and esophagus. Does the same hold true for gallbladder? If so, then it appears there is a conflict between STR and Appendix E2. However, using the logic of SINQ 20240021 for this site would appear to contradict Appendix E2 which indicates high grade dysplasia in sites other than stomach, intestine, and esophageal sites is not reportable. If we can code high grade dysplasia of GI sites to 8148/2, should we accession high grade dysplasia of the gallbladder and other biliary sites in a similar manner? If so, then Appendix E needs to be modified. |
Report biliary intraepithelial neoplasia (dysplasia), high grade. As noted in SINQ 20240021 and the Other Sites Solid Tumor Rules, Rules H4/H26, the listed sites may not include all reportable neoplasms for 8148/2. We will update the Other Sites Solid Tumor Rules to reflect this code as well as make revisions in the next release of the SEER Manual. |
2024 |
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20240065 | Solid Tumor Rules/Histology--Ovary: What is the histology code for an ovarian primary with a pathology report final diagnosis of “Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant” diagnosed in 2012 (using the Multiple Primaries H rules) and one diagnosed in 2024 (using the Solid Tumor Rules)? See Discussion. |
2012 Total abdominal hysterectomy - bilateral salpingo-oophorectomy Primary Site – Ovary, Right Histology - Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant 2024 Total abdominal hysterectomy - bilateral salpingo-oophorectomy Primary Site – Ovary, Left Histology - Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant |
Abstract this case as a single primary. Code as 8044/3 (small cell carcinoma, hypercalcemic type) listed in the Other Sites Solid Tumor Rules, Table 13. Small cell carcinoma, large cell variant, is a subtype of small cell carcinoma, hypercalcemic type. This table does not include all possible histologies. WHO Classification of Female Genital Tumors, 5th edition, states: Small cell carcinoma of the ovary, hypercalcemic type, is rare, accounting for < 1% of ovarian tumors. Small cell carcinomas, hypercalcemic type, are usually large, with a mean size of 15 cm (range: 6–26 cm). Large cells are present (in varying numbers) in half of these tumors, which are designated “small cell carcinoma, large cell subtype” if the large cells are predominant (which is rare). |
2024 |
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20240037 | Solid Tumor Rules/Histology--Bladder: How is histology coded for a bladder tumor when the diagnosis is 95% large cell neuroendocrine carcinoma and 5% high grade urothelial carcinoma of no special type? See Discussion. |
In the 2024 Solid Tumor Rules update, the small cell neuroendocrine carcinoma row in Table 2 was changed. The NOS histology became neuroendocrine carcinoma, NOS (8246) and both large cell and small cell neuroendocrine carcinomas (8013 and 8041, respectively) became the subtype/variants. This change impacts Rule H4 but Rule H4 was not updated. Rule H4 still refers to small cell neuroendocrine carcinoma as being the NOS histology. In the prior STR versions, it was clear the tumor in question would be coded as 8045 per Rule H4 and Table 2. Considering Rule H4 was not updated according to the changes for Table 2, does histology 8045 still apply to this diagnosis? There is currently no way to arrive at a histology for this case. Does Rule H4, bullet 3 need to be updated to indicate, “subtype/variant of neuroendocrine carcinoma mixed with any other carcinoma (does not apply to sarcoma)”? |
Assign 8013/3 (combined large cell neuroendocrine carcinoma). There are two histologies present: large cell NEC and urothelial. Literature search found primary large cell NEC of the bladder is extremely rare with less than 20 reported cases. This case does not fall into the site-specific rules and given it's raity, a specific rule for this situation was not and will not be added to the Bladder rules. See #1, Example 2, in the general instructions for coding histology. |
2024 |
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20240074 | Solid Tumor Rules/Histology--Head & Neck: How is histology coded for nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type? See Discussion. |
Example: Patient had a 2023 nasopharyngeal mass biopsy showing “Nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type.” The Head and Neck Solid Tumor Rules (STRs) do not include an H Rule that instructs us how to code histology when there are two subtypes/variants present for a head and neck primary, nor does the STR define undifferentiated carcinoma as a subtype/variant for 8072. The WHO Classification of Head and Neck Tumors states non-keratinizing nasopharyngeal carcinoma (non-keratinizing squamous cell carcinoma (SCC) is the most common subtype for nasopharyngeal ca, but that non-keratinizing can be subdivided into undifferentiated and differentiated subtypes. Should histology be 8020 (undifferentiated carcinoma) or 8072 (non-keratinizing SCC)? |
Assign histology as 8072 for non-keratinizing SCC, undifferentiated subtype. WHO Classification of Head and Neck Tumors, 5th edition assigns 8072/3 to squamous cell carcinoma, non-keratinizing, NOS in the nasopharynx. As the tumor exhibits a variety of architectural patterns and appearances histologically, they can be further classified as undifferentiated or differentiated subtypes. These subtypes do not change the histology code. |
2024 |
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20240018 | Solid Tumor Rules/Histology--Head and Neck, Other Sites: Please provide clarification about effective dates for using p16 testing to assign HPV-related histology codes for various primary sites. See Discussion. |
1. The 2022 and 2023 SEER Program Coding Manuals state under Histologic Type ICD-O-3: Beginning with cases diagnosed 01/01/2022 forward, p16 test results can be used to code squamous cell carcinoma, human papilloma virus (HPV) positive (8085) and squamous cell carcinoma, HPV negative (8086). NAACCR 2023 Implementation Guidelines contain similar instructions on HPV histologies for cervix, vulva and vagina that are applicable back to 2022 (2021 for cervix). The current Other Sites Solid Tumor Rules state on the Histology tables for anus, cervix, vagina, vulva, and penis and scrotum: "p16 is a valid test to determine HPV status and can be used to code HPV associated and HPV independent histologies." Since Other Sites Solid Tumor Rules apply to cases diagnosed 2023+, can p16 results only be used from 2023 onward, to code HPV-related histologies for primaries that fall under the Other Sites module? Or per the 2022 SEER Manual statement and NAACCR 2023 Implementation Guidelines, could a p16-confirmed HPV histology code also apply to a 2022 Other Sites case and if so, is that only for cervix, vulva, and vagina? Further complicating the matter are the 2024 ICD-O-3.2 update documents indicating these codes are valid 1/1/2024+ for the “Other Sites” penis and scrotum. 2. Is using p16 testing for HPV-related histology codes ONLY allowed for sites in the Solid Tumor tables that contain the statements about p16 (Head & Neck Table 5, and the Other Sites tables noted above for anus, cervix, etc.)? Or could it apply to primary sites outside of those tables; for example, a 2022 pathology report from the ethmoid sinus C311 indicating an HPV-related histology based on p16 testing? The ICD-O-3 Annotated Histology lists include C310-C313 among the common site codes for 8085 and 8086. The Head and Neck Solid Tumor Rules “New for 2022” section and rule H1 Note 4 also mention that p16 can be used to code HPV histologies; these sections would seem to apply to all sites in that module, since only the more common histology codes are listed in the tables and if not, we are instructed to use ICD-O. |
Per 2024 Cancer PathCHART expert pathologist review, morphology codes 8085/3 and/or 8086/3 are valid and applicable to head and neck, oropharynx, cervix, vagina, vulva, fallopian tube, anus, and penis scrotum (reference: Cancer PathCHART: Product Downloads and Timelines). The Cancer PathCHART SMVL will be updated for C632, Scrotum, with the next release of the NAACCR Edits Metafile, currently scheduled for May 2024. Assign histology codes 8085 and 8086 for the sites listed in the Solid Tumor Rules histology tables. The codes 8085 and 8086 are applicable for a small group of sites according to the year they became valid for implementation as follows. Head and Neck Oropharynx, Base of Tongue, Tonsils, Adenoids (2022+) Other Sites Cervix (2021+) Anus (2023+) Vagina (2023+) Vulva (2023+) Penis (2024+) Scrotum (2024+) While ICD-O-3.2 and Cancer PathCHART list additional sites such as Accessory Sinuses, they have not yet been implemented in the U.S. |
2024 |
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20240013 | Solid Tumor Rules/Histology--Testis: Can a definition for "teratoma with somatic-type malignancy" (9084) be added to the Other Sites Solid Tumor Rules? See Discussion. |
We included this histology in SEER Workshop Case 12 and the histology coding accuracy was less than 40%. From emails we received, it is clear that registrars are unaware that the "somatic type malignancy" can vary but code 9084 applies when the diagnosis is teratoma WITH any non-germ cell tumor component. It may be helpful to add a definition for "teratoma with somatic-type malignancy" (9084) to the Solid Tumor Manual. |
We will add the same definition for teratoma with malignant transformation found in the ovary table: 9084/3 Teratoma with malignant transformation when a malignant (/3) histology arises in a benign teratoma. Teratoma with malignant transformation and teratoma with somatic-type malignancy are synonoyms. The term teratoma with somatic-type malignancy is outdated and no longer recommended. |
2024 |
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20240002 | First Course Treatment--Heme & Lymphoid Neoplasms: How should treatment data items be coded for a diagnosis of myelodysplastic syndrome (MDS) and symptomatic anemia treated with Reblozyl (Luspatercept)? See Discussion. |
Example: Patient has a 04/2023 diagnosis of symptomatic anemia not responsive to Retacrit. Further testing includes diagnostic bone marrow biopsy 10/2023 proving MDS with low blasts and SF3B1 mutation, treated with Relozyl (Luspatercept). There is no SEER*Rx listing for Reblozyl or Luspatercept. Per web search, Luspatercept, sold under the brand name Reblozyl, is a medication used for the treatment of anemia in beta thalassemia and myelodysplastic syndromes. Is this non-cancer directed treatment since it is given to address the anemia rather than the MDS? If cancer-directed treatment, how should it be coded? |
Do not code Reblozyl (luspatercept) as treatment. Luspatercept is an ancillary drug approved to treat anemia associated with MDS but not the malignancy. |
2024 |
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20240071 | Heme and Lymphoid Neoplasms/Multiple Primaries--Myeloproliferative Neoplasms: Are essential thrombocytosis (ET) in 1998 and primary myelofibrosis in 2022 the same primary or is the 2022 diagnosis a new primary? See Discussion.
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Patient was diagnosed with essential thrombocytosis 9962/1 or 3 in 1998 (depending if ET was reportable in 1998), treated with Hydrea. 11-17-2022 Blood smear: CALR + myeloproliferative neoplasm, Most Consistent with Primary Myelofibrosis 9961/3 (Noted CALR and ASXL1 mutations). The following abstractor note from 9661/3 is confusing: A diagnosis of "post essential thrombocythemia myelofibrosis" is a progression of essential thrombocythemia and would be the same primary. |
Abstract two separate primaries, ET (9962/3) and primary myelofibrosis (9961/3) using the current Hematopoietic and Lymphoid Neoplasms (Heme) Manual and Database (DB), Rule M15, use the Heme DB Multiple Primaries Calculator. Also refer to the example in Rule M15. In 1998, though the ET was not reportable (9962/1), the patient was treated with chemotherapy as a malignant neoplasm (9962/3). The Calculator instructs us to code separate primaries for these two histologies. ET may evolve into a secondary myelofibrosis, also known as post-essential thrombocythemia-myelofibrosis (post-ET MF). The diagnosis must be stated as post-ET MF; this would be a single primary. |
2024 |