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20240029 | Solid Tumor Rules/Multiple Primaries--Head and Neck: Is a 11/2023 diagnosis of invasive squamous cell carcinoma (SCC) in lower gum (C031) a new primary and what rules apply for a patient with 09/2017 invasive SCC of lower gum (C031) and 05/2022 invasive SCC of lateral tongue (C023)? See Discussion. |
The 11/2023 lower gum tumor is a separate tumor occurring after a disease-free interval, so we know the Head and Neck Multiple Tumors Module applies. However, our staff is having difficulty applying the rules to this particular scenario with consistent results. Is the 11/2023 SCC a non-reportable recurrence per M12, since M4 is ignored due to patient’s prior 2017 C031 (lower gum) primary, and then M6 is ignored due to patient’s prior 05/2022 C023 primary? Or is the 11/2023 SCC a new primary per M4, since the last diagnosis was in a site differing at the third character (C03 vs C02)? If M4 does not apply due to patient's previous C03 primary, then does M6 apply since it has been more than 5 years since the previous C03 primary? |
Abstract three primaries for the scenario you describe.
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2024 |
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20240052 | Reportability/Histology--Heme & Lymphoid Neoplasms: Should a non-Langerhans cell histiocytosis involving the hypothalamus and pituitary gland be accessioned as a reportable, behavior /1, CNS neoplasm? See Discussion. |
Imaging identified a mass involving the hypothalamus and pituitary gland and excision of the mass proved “histiocytosis.” The case was extensively reviewed, and the physician notes this patient has a pituitary tumor that is a “non-Langerhans cell histiocytosis,” or a “non-LCH histiocytic neoplasm.” There is no histology for histiocytosis (NOS) or non-Langerhans cell histiocytosis. However, this does appear to be a non-malignant histiocytic neoplasm. If this were a Langerhans cell histiocytic neoplasm in the CNS it would be reportable. Should this non-Langerhans cell histiocytic neoplasm also be accessioned as a reportable CNS neoplasm? If so, how is the histology coded? |
Report this case as a pituitary tumor (8000/1) based on the information provided. This is the best choice as no specific histology code exists for this generic term “non-Langerhans cell histiocytosis” in ICD-O-3.2, WHO Classification of CNS Tumors, 5th ed., and WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th ed. Be sure to double-check the behavior code of the tumor. Histiocytosis can be benign, borderline, or malignant. There was no mention of the behavior so we defaulted to uncertain behavior for this case. |
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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20240022 | Solid Tumor Rules/Histology: When should the designation of “poorly differentiated” be used to further specify histology for carcinoma, NOS (8010) as undifferentiated carcinoma (8020)? See Discussion. |
The term “poorly differentiated carcinoma (NOS)” is listed as related to “undifferentiated carcinoma (NOS)” in the ICD-O 3.2. It is also listed in the Solid Tumor Rules for Urinary Table 2 (Urinary subtypes), Other Sites Table 16 (uterine corpus primaries) and Table 19 (vulvar primaries). Are these the only sites in which one should code “poorly differentiated carcinoma (NOS)” as 8020 (undifferentiated carcinoma)? How is histology coded if the only microscopic confirmation is from a metastatic site showing “poorly differentiated carcinoma” (NOS) or “invasive carcinoma, poorly differentiated” (NOS)? Example 1: Primary pancreatic cancer diagnosed on imaging and confirmed with liver mets core biopsy showing “poorly differentiated carcinoma.” Immunostaining pattern was non-specific. No further workup or treatment was planned. Other Sites - Table 11 (Pancreas Histologies) includes undifferentiated carcinoma (8020/3) as a valid histology; however, the synonyms/subtypes/variants do not mention poorly differentiated carcinoma. How should histology be coded for this case? Example 2: Hemicolectomy with cecal tumor final diagnosis of “invasive carcinoma, poorly differentiated” and synoptic summary listing “Histologic type: Invasive carcinoma. Histologic grade: G3 of 4: poorly differentiated.” Colorectal Table 1 (Specific Histologies and Subtypes/Variants) includes undifferentiated adenocarcinoma/carcinoma 8020 as a subtype of adenocarcinoma NOS. There is no mention of poorly differentiated in this context. How should histology be coded for this case? |
Assign code 8020/3 when the histologic type specifically includes the term of poorly differentiated, dedifferentiated, undifferentiated, or anaplastic undifferentiated carcinoma along with carcinoma as terms vary depending on the primary site. When the term poorly differentiated is included in the grade section only of the pathology report or only mentions poorly differentiated carcinoma without further substantiation from a pathology report as in examples 1 and 2, do not use code 8020/3. The histology code 8020/3 and terms may be used for selected primary sites as included in the Solid Tumor Rules, WHO Classification of Tumors series (latest versions), and the Site/Morphology Validation List including Nasal cavity Nasopharynx Salivary glands Urinary sites Colon, rectosigmoid, rectum Esophagus Stomach Gallbladder and extrahepatic bile duct Pancreas Thyroid Ovary Uterine corpus Vagina Uterine cervix (also referred to as unclassifiable in WHO Classification of Female Genital Tumors, 5th ed.) For sites other than those listed, if the diagnosis is poorly differentiated carcinoma, code 8010/3 and poorly differentiated in the grade field. |
2024 |
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20240077 | 2024 SEER Manual/Primary Site--Retroperitoneum: What is the primary site code for a final diagnosis of endometrioid adenocarcinoma from a biopsy of a right retroperitoneal mass? See Discussion. |
An 80-year-old post-menopausal female (status post hysterectomy for benign reasons) presents with a retroperitoneal mass on imaging. The pre-operative imaging shows the cervix and uterus are absent. Patient undergoes a robotic left salpingo-oophorectomy with biopsy of the retroperitoneal mass. |
Code Primary Site to C480 (retroperitoneum). Endometrial tissue may "break away” from the uterus and implant throughout the pelvic and abdominal cavities. This can occur in patients who suffer from endometriosis. This tissue remains behind when surgical removal of the uterus is done. Common sites of implantation are colon, peritoneum, retroperitoneum, and bladder. These cells may become malignant. When the uterus is no longer present (patient had surgical removal), code the site where the carcinoma was identified. The site-morphology combination of C480 and 8380/3 was designated as an unlikely site-morphology combination by the Cancer PathCHART expert pathologist review, as this is a rare type of tumor. Assign a value of 1 in the Over-ride Site/Type [2030] data item in order to pass the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) edit. |
2024 |
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20240044 | First Course Treatment/Neoadjuvant Therapy--Esophagus: Should the Neoadjuvant Therapy data item be coded as 1 or 2 when the patient completes all but one cycle of the planned neoadjuvant therapy and the managing physician notes the patient completed the neoadjuvant therapy? See Discussion. |
The patient had neoadjuvant chemotherapy (Carboplatin and Paclitaxel) concurrent with radiation per the managing physician. The physician stated the patient completed the neoadjuvant therapy; however, it was also noted that patient completed five cycles of chemotherapy, but the sixth cycle was held due to neutropenia. The SEER Manual does not address how to code Neoadjuvant Therapy when the patient completed almost all the planned neoadjuvant therapy. It seems inappropriate to code Neoadjuvant Therapy as 2 (Started but not completed) simply because the patient did not have one cycle of chemotherapy but is otherwise felt to have completed neoadjuvant therapy per the managing physician. Does the managing physician’s statement of “completion” impact how this scenario is coded? |
Assign code 2, Neoadjuvant therapy started, but not completed OR unknown if completed, for the 2024 SEER Manual data item Neoadjuvant Therapy. Assign code 2 when neoadjuvant therapy was begun and the patient did not complete the full course of neoadjuvant therapy. See Coding Instruction #3 on page 230. The fact that the patient completed five cycles of the planned chemotherapy, but the sixth cycle was held due to neutropenia is important information and should be abstracted correctly and documented via text data items. |
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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20240007 | Histology--Brain and CNS: Provide clarification about the priority order of histology coding sources and an explanation of why the annotated histology lists are not the same as the WHO IARC ICD-O-3.2 Excel Table (adopted 1/1/2021). See Discussion. |
We have had multiple users unable to find the applicable histology in the ICD-O-3.2 (i.e., the site-specific table did not include the histology) because they were using the annotated histology list and could not find the complete list of related terms or synonyms for the histology code. For example, the ICD-O-3.2 lists Medulloblastoma, SHH-activated, NOS as a related term for 9471/3, but many users were unable to find this valid histology because they were using the annotated histology list, not the ICD-O-3.2. |
The NAACCR Annotated Histology List (AL) serves as an aid to registry software vendors for implementing annual histology changes. This file has been maintained by the Registry Plus team at CDC’s NPCR for several years and reflects modifications to ICD-O-3 implemented by North American cancer registries over time. Although this list is reviewed multiple times prior to posting, there is no guarantee of 100% accuracy. As such, the AL is not a substitute for referring to various standard-setter documents and implementation guidelines. In this instance, Medulloblastoma Desmoplastic SHH-activated and TP53-wildtype 9471 is across several resources: the Solid Tumor Rules, Malignant CNS and Peripheral Nerves module in Table 3, column 3 as a subtype/variant of Medulloblastoma NOS 9470; in the CNS WHO 5th Edition BB; and in the WHO IARC ICD-O-3.2 posted to ICD O 3 Coding Updates (naaccr.org). Although the exact related term of Medulloblastoma, SHH-activated, NOS is not listed, the NAACCR Implementation Guidelines for 2024 recommend checking the 2024 ICD-O-3 Update Table 1 or 2 to determine if the histology is listed. If the histology is not included in the update, then review ICD-O-3.2 and/or Hematopoietic and Lymphoid Database and/or Solid Tumor Rules (MP/H). The Cancer PathCHART initiative has been undertaken to address gaps such as this between standard setting resources. Having all the standard histology coding resources included in a single all-inclusive database enables alignment of morphology codes & terms included in the CPC*SMVL (Cancer PathCHART Site-Morphology Validation List), Solid Tumors Rules, ICD-O-3 Annual Updates, NAACCR Annotated Histology List as well as the WHO 5th edition Blue Books. Please see Cancer PathCHART - Tumor Site-Morphology Surveillance Standards Initiative for more information on the Cancer PathCHART initiative, and more specifically, see Transitioning the Annotated Histology List to Cancer PathCHART (naaccr.org). |
2024 |
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20240070 | Reportability/Histology: Does Cancer Pathology Coding Histology And Registration Terminology (Cancer PathCHART) determine if the histology is reportable or do we have to use the Excel ICD-O-3.2 spreadsheet? |
The CPC ICD-O-3 Site Morphology Validation Lists (SMVLs) designate all tumor site-morphology combinations that are either valid or impossible as determined for the sites reviewed by the Cancer PathCHART initiative. These lists provide information on the Validity Status of specific tumor site and morphology combinations, similar to the way the ICD-O-3 SEER Site/Histology Validation List used to. However, the CPC SMVLs do not include information on the reportability of specific tumor site and morphology combinations. For tumor reportability, you will continue to use the Excel ICD-O-3.2 spreadsheets posted to the NAACCR ICD-O-3 Coding Updates website: https://www.naaccr.org/icdo3/, and the most recent SEER Manual and federal, state, local, and other standard setters' reportability requirements. |
2024 | |
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20240061 | Heme & Lymphoid Neoplasms/Histology--Myelodysplastic Neoplasm: What is the histology code for myelodysplastic neoplasm with low blasts and SF3B1 mutation with primary site bone marrow (C421) diagnosed in 2023? |
Assign histology as 9982/3 (myelodysplastic neoplasm with low blasts and SF3B1 mutation). This is a new term for 9982/3. WHO Classification of Hematolymphoid Tumors, 5th edition, defines myelodysplastic neoplasm (MDS) with low blasts and SF3B1 mutation (MDS-SF3B1) as a myeloid neoplasm with cytopenia and dysplasia characterized by SF3B1 mutation and often ring sideroblasts. |
2024 |
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