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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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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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20240056 | Reportability/Histology--Heme & Lymphoid Neoplasms: How should this unusual 2023 pathology-only case be reported and coded for leukemia cutis? See Discussion. |
10/25/2023: Patient presents to dermatology office with a questionable drug eruption having 3 weeks of papular eruptions of Trunk (Left Chest). Punch biopsies were taken that came back as immature hemopoietic infiltrate with monocytic differentiation. Comment: Myelodysplastic syndrome and leukemia cutis are possibilities. Addendum Report: Additional stains were prepared. ERG is strongly positive. CD1a and S100 do not stain the atypical cells.The controls stain appropriately. CD123 perform with appropriate control is also negative. The pattern is that of so-called "leukemia cutis" which could be seen in the clinical setting of myelodysplasia, chronic myelomonocytic leukemia (CMML) or precursor to acute myelomonocytic leukemia (AMML). Recommend work up. The only available information at present is a diagnosis of leukemia cutis, and that there was no prior history of a hematological malignancy in this patient. |
Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available. Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis. |
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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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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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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20240035 | Solid Tumor Rules--Urinary: The example used in Rule M15 of the Urinary Solid Tumor Rules refers to the same row in Table 3. Should the example say Table 2 since Table 3 is non-reportable urinary tumors. See Discussion. |
Rule M15 Abstract a single primary when synchronous, separate/non-contiguous tumors are on the same row in Table 2 in the Equivalent Terms and Definitions. Note: The same row means the tumors are • The same histology (same four-digit ICD-O code) OR • One is the preferred term (column 1) and the other is a synonym for the preferred term (column 2) OR • A NOS (column 1/column 2) and the other is a subtype/variant of that NOS (column 3) OR • A NOS histology in column 3 with an indented subtype/variant Example: TURBT shows invasive papillary urothelial carcinoma 8130/3 and CIS/in situ urothelial carcinoma 8120/2. Abstract a single primary. Papillary urothelial carcinoma and urothelial carcinoma are on the same row in Table 3. |
The example used in Rule M15 of the Urinary Solid Tumor Rules should refer to Table 2. We will update this in the next revision of the Rules. |
2024 |
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20240017 | EOD 2018/Prostate Pathologic Extension--Prostate: Is a pathology report from a prostate biopsy/transurethral resection of the prostate that states "with intraductal spread" extraprostatic/extracapsular extension or localized? |
Code as a localized, intracapsular tumor as ductal carcinoma in situ does not invade. Intraductal spread is describing the neoplasm spreading through the acinar/ductal cells in the prostate specimen. It is an in-situ type of spread and not invasive but almost always presents with an invasive tumor. |
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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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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