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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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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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20240066 | Histology--Heme & Lymphoid Neoplasms: How should histology be coded for a pathologic diagnosis of “Follicular lymphoma, diffuse pattern grade 3A of 3, equivalent to diffuse large B cell lymphoma (germinal center cell type)” when later referenced clinically as follicular lymphoma grade 3A? See Discussion. |
The WHO Classification of Hematopoietic Tumors (Blue Book), 5th edition states: “Rare cases of classic follicular lymphoma with cytological features of follicular lymphoma (FL) grade 3A can present with a prominent diffuse pattern. In the previous edition, such cases were defined as diffuse large B-cell lymphoma (DLBCL). Currently, it is uncertain whether such cases should be classified as FL or diffuse large B-cell lymphoma; and in such cases, individual treatment choices should be made in multidisciplinary conference settings taking into consideration clinical, laboratory, and imaging parameters. The presence of diffuse areas composed entirely or predominantly of large cells, however, warrants a diagnosis of diffuse large B-cell lymphoma.” Our concern is that the Hematopoietic (Heme) Manual and Database do not provide instruction for coding this scenario. We hesitate to interpret the terms “equivalent to” as ambiguous because one could argue it is unambiguous. Barring this argument, the M and H rules would indicate this is a diagnosis of diffuse large B-cell lymphoma. However, the physician does not seem to agree with the pathologist. |
Assign histology as DLBCL (9680/3) as supported by the WHO Classification of Hematolymphoid Tumors, 5th edition. It is consistent with how it would have been coded in the 4th edition. The Heme Manual and Database currently are based on the 4th edition. Physicians are using the 5th edition blue book, whereas the cancer registry field is not yet at this time. Regarding the Heme Manual and Database, this type of scenario is not covered because it is part of the 5th edition WHO Blue Book. The database cannot be updated until the 5th edition is approved for implementation (2026). |
2024 |
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20240053 | Reportability/Behavior--Kidney: Is a 2022 diagnosis of “clear cell renal cell papillary tumor” on nephrectomy reportable? See Discussion. |
We are aware that the WHO 4th edition for urinary tumors has changed the behavior of “clear cell papillary renal cell carcinoma” to /1 but registries are to continue collecting as /3. While the diagnosis in our case is stated as “tumor” it does seem like the pathologist may be using the new WHO terminology of “tumor” rather than “carcinoma,” so we are not sure if behavior is /3 or /1. |
Report clear cell renal cell papillary tumor (CCRCPT), formerly classified as clear cell renal cell papillary carcinoma, and assign code 8323/3 until this new term and code (8323/1) have been adopted by standard setters. The Kidney Solid Tumor Rules advise to code clear cell papillary renal cell carcinoma as 8323/3. WHO Classification of Tumors of the Urinary System and Male Genital Organs, 4th ed., has reclassified this histology as a /1. This change has not yet been implemented and it remains reportable. WHO Classification of Urinary and Male Genital Tumors, 5th ed., has since reclassified clear cell papillary renal cell carcinoma as CCRCPT (8323/1). The name change was made because there have been no reports of metastatic events for this indolent tumor. The term clear cell renal cell papillary carcinoma is no longer recommended. |
2024 |
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20240072 | Solid Tumor Rules/Histology--Oropharynx: How is histology coded for a 2024 squamous cell carcinoma of the tonsil when immunohistochemistry (IHC) stains are negative for p16, but in situ hybridization (ISH) testing is positive for human papilloma virus (HPV)? See Discussion. |
The Solid Tumor Rules state that for cases diagnosed in 2022 and forward, p16 testing CAN be used to assign histology code 8085 (squamous cell carcinoma, HPV positive). The rules also state that for cases diagnosed prior to 1/1/2022, code 8085 MUST be based on ISH testing and not p16. ISH testing is not specifically addressed for 2022+ cases, but are we correct in assuming it can still be used as the basis for 8085? Multiple CAnswer Forum posts and the AJCC 8th edition Head and Neck staging webinar indicate that the correct chapter/registry staging schema in this situation is determined ONLY by p16 results - not ISH testing, and therefore the Schema Discriminator 2 SSDI should be coded as 1 – p16 negative, regardless of ISH results. While we understand that histology codes should not be changed based on staging criteria, there is a SEER/NAACCR edit, “Schema Discriminator 2, Head and Neck, Histology (NAACCR)” tag number N6802, that will not allow coding 8085 if Schema Discriminator 2 is coded as 1 (p16 negative). The edit does seem to be correctly enforcing the AJCC guidelines for choosing the staging schema, based on the sources noted above. Do the Solid Tumor or Site-Specific Data Items (SSDI) guidelines need to be modified for this situation? |
Assign histology as squamous cell carcinoma, HPV positive (8085) for tonsil, NOS (C099) based on the positive HPV test. Codes 8085 and 8086 are valid for a select group of sites. The histology terms and codes that are valid for head and neck sites are included in the Head and Neck Solid Tumor Rules, Table 5 (oropharynx). HPV detection tests that are used to identify HPV include DNA polymerase chain reaction (PCR), p16 (IHC), or DNA/RNA in situ hybridization. Assign the appropriate method of detection in the SEER data item, SEER Site-Specific Factor 1. Schema Discriminator 2 captures additional information needed to generate AJCC ID and Schema ID for some anatomic sites as stated in the SSDI Manual. For oropharyngeal cancer, a schema discriminator is used to discriminate between oropharyngeal tumors that are p16 positive, p16 negative, or p16 status unknown in order to assign the appropriate schema ID. Only the HPV p16 test can be used to assign Schema Discriminator 2. If another HPV test is performed, code 9. Override the edit for Schema Discriminator 2 when p16 is negative. Coding updates will be implemented in 2025. |
2024 |
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20240064 | Primary Site/Histology--Ovary: We are encountering a primary site, histologic type, and behavior combination edit based on the Cancer PathCHART (CPC) tables. Using the CPC*Search tool, C569 and 8441/3 is a valid combination. The diagnosis date is 01/13/2024. Should an over-ride be applied with this combination? |
The CPC Validity Status of the site morphology combinations of C569/8441/3 and C569/8441/2 was revised from Valid to Unlikely with the latest release of the Version v24A Edits Metafile. As a result, this site and morphology combination will now require an over-ride flag to be set. Code as 8461/3 (high-grade serous carcinoma) or 8460/3 (low-grade serous carcinoma) if at all possible. Use 8441/3 (serous carcinoma, NOS) only if it cannot be distinguished as low grade or high grade. The codes for high-grade serous carcinoma and low-grade serous carcinoma are relatively new. High-grade serous carcinoma and low-grade serous carcinoma are very different tumors and pathologists should state whether it is high grade or low grade. Please make every attempt to use the newer codes. If unable to determine high gade versus low grade, assign 8441/3 and override the edit. The files on the CPC website are currently being updated, and CPC*Search will be updated to reflect the changes sometime this Fall. |
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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20240063 | Solid Tumor Rules/Multiple Primaries--Bladder: How many primaries and what M Rule applies for a diagnosis of noninvasive micropapillary urothelial carcinoma (8131/2) in 2019, followed by a diagnosis of noninvasive papillary urothelial carcinoma (8130/2) in 2024? |
Abstract two primaries using Urinary Solid Tumor Rules, Rule M12. The histologies include non-invasive papillary urothelial carcinoma (8130/2) and non-invasive micropapillary urothelial carcinoma (8131/3). The two histology codes are listed as subtypes of Papillary urothelial (transitional cell) carcinoma in column 3 of Table 2. WHO Classification of Urinary and Male Genital Tumors, 5th edition classifies micropapillary urothelial carcinoma as an aggressive subtype of urothelial carcinoma with carcinoma in situ present in more than half of all micropapillary carcinomas. Rule 7 Note 3 of the Urinary Solid Tumor Rules states that there are no /2 subtypes for urothelial carcinoma with the exception of papillary urothelial carcinoma and applies to multiple occurrences of /2 urothelial carcinoma of the bladder. Rule 8 applies to 8131/3 and 8120/3. |
2024 | |
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20240034 | SEER Manual/Reportability--Skin: Is keratoacanthoma (8071/3) of the skin reportable? This code is also for squamous cell carcinoma (SCC), keratinizing. In the 2024 SEER manual, 8071/3 falls under the not reportable section of skin (outside of specific sites). |
Do not report keratoacanthoma of the skin (8071/3). The preferred term for keratoacanthoma is squamous cell carcinoma (SCC), keratinizing, NOS. According to the 2024 SEER Manual, Reportability section, SCC of skin (8050-8084) is not reportable. |
2024 | |
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20240028 | 2024 SEER Manual/Primary Site--Breast: Is Primary Site coded as C504 or C501 based on the Solid Tumor Rules and the SEER Manual Breast Coding Guidelines? The pathology report reads "Right Breast 10:00 1 cm from the nipple." Codes C502-C505 take priority over code C501. The description for C501 in the Solid Tumor Rules has "Area extending 1 cm around areolar complex." |
Assign Primary Site code C504 based on the location in the upper outer quadrant of the right breast, 10 o’clock, as opposed to code C501, around the areolar complex. The 2024 SEER Manual Breast Coding Guidelines advise that C502 - C505 are generally preferred over C501 when there is no other way to determine the subsite. |
2024 |
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