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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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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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20240023 | Solid Tumor Rules/Histology--Penis: Why is warty carcinoma listed in Other Sites, Table 23 (Penis and Scrotum Histologies) as 8051 when the ICD-O-3.2 and SINQ 20200003 indicate the correct histology is 8054 for this neoplasm? See Discussion. |
The ICD-O-3.2 indicates histology 8051 only applies to diagnoses of condylomatous carcinoma and warty carcinoma made prior to 2018. For penis cases diagnosed 2018 and later, these neoplasms should be coded as 8054. This is consistent with SINQ 20200003. However, a new Table was added to the Other Sites schema in the 2024 Solid Tumor Rules update. Table 23 lists “Verrucous carcinoma / carcinoma cuniculatum / Warty carcinoma” as histology 8051. While verrucous carcinoma is still listed under histology 8051 in the ICD-O-3.2, warty carcinoma is not. Does Table 23 need to be updated? Or is this an error in both the ICD-O-3.2 and SINQ 20200003? |
Assign histology code 8054/3 for warty carcinoma. Assign 8051/3 for verrucous carcinoma and carcinoma cuniulatum. The WHO Classification of Urinary and Male Genital Tumors, 5th edition (2022) revised the terminology for squamous cell carcinoma groupings from "non-HPV-related" to "HPV-independent" and from "HPV-related to "HPV-associated". Warty carcinoma is defined as a "morphologically distinct HPV-associated verruciform neoplasm that shares histological features with a giant condyloma but has definitive cytological atypia and a malignant infiltrative architecture." Verrucous carcinoma (including carcinoma cuniculatum) is defined as an HPV-independent squamous cell carcinoma, and is correctly coded to 8051/3. The 2024 Solid Tumor Rules, Table 23, Penis and Scrotum Histologies will be updated to reflect this revised terminology and coding. |
2024 |
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20240012 | Solid Tumor Rules/Histology--Other Sites: Should an additional Note be added to Other Sites Solid Tumor Rules, Rule H12, to indicate that if the diagnosis is an NOS histology in a polyp, continue on through the rules or should Other Sites Rule H13 be moved ahead of Rule H12 to capture this specific histology? See Discussion. |
The accuracy rate for SEER Workshop Case 04 (a duodenal invasive adenocarcinoma in an adenomatous polyp) was very low because Rule H13 was either being ignored or users were stopping at Rule H12 to code adenocarcinoma. If the presence of an NOS histology in a polyp is still clinically relevant for the Other Sites module, this information will be missed due to the order of the H Rules, or the lack of clarification in Rule H12. If a change is made to Rule H12 (Single Tumor: Invasive Only module), then changes must also be made to the Single Tumor: In Situ Only module and the Multiple Tumors Abstracted as a Single Primary module because both these modules include the same polyp coding H Rule. |
The rule order is the same as in the previous MP/H rules. Will keep as is for now. Assign codes adenocarcinoma in adenomatous polyp (8210), adenocarcinoma in villous adenoma (8261), and (adenocarcinoma in tubulovillous adenocarcinoma (8263) using Other Sites Solid Tumor Rule H12 or Rule H27 as these are specific invasive histology codes. Rule H13 applies to histology codes associated with polyps but associated with a histology term/code other than adenocarcinoma. |
2024 |
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20240038 | Solid Tumor Rules/Multiple Primaries--Brain and CNS: How many primaries are accessioned, and what M Rule applies to a 2023 diagnosis of pituitary macroadenoma followed by a 2024 diagnosis of pituitary neuroendocrine tumor (PitNET) when the patient did not undergo surgery, but did undergo hormone therapy with Cabergoline? See Discussion. |
Malignant Central Nervous System (CNS) Rule M5 instructs us to abstract a single primary (as malignant) when a single tumor is originally diagnosed as non-malignant, the “First course treatment was active surveillance (no tumor resection),” and the subsequent resection pathology is malignant. This patient’s first course of treatment was not active surveillance. While the patient did not have first course tumor resection, the tumor was treated with Cabergoline. Should Rule M5 apply because there was no tumor resection? If so, should Rule M5 clearly state no tumor resection is the criteria (not active surveillance)? SINQ 20230023 does indicate a PitNET diagnosis following a diagnosis of pituitary adenoma does not fall into standard rules, but in the previous SINQ the first course treatment was a partial resection. It is unclear whether other types of treatment could result in a new malignant PitNET, following a previously treated non-malignant pituitary tumor. |
Abstract a single primary as 8272/3 (pituitary adenoma/PitNET) using the Malignant CNS and Peripheral Nerves Solid Tumor Rules, Rule M2, a single tumor is always a single tumor. Change the histology of the 2023 diagnosis to 8272/3. This scenario does not meet the criteria in the current rules for M5 in that it requires a resection as part of the criteria. Since the patient did not undergo resection for either diagnosis, the 2024 diagnosis may indicate recurrence or progression. A diagnosis of pituitary adenoma only is still coded 8272/0 (this code is still valid). A diagnosis of pituitary adenoma/PitNET, PitNET, or pituitary neuroendorine tumor is coded 8272/3. Cabergoline is used to treat prolactinoma or high levels of prolactin but does not impact the PitNET. |
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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20240054 | EOD 2018/Primary Tumor--Breast: We are having difficulty deciding when we can or cannot use physician-assigned TNM staging to code EOD data items if the medical record or hospital abstract documentation is unclear. As a central registry, we are unable to query physicians for clarification. Please advise what is a “discrepancy” in the EOD General Instructions to “Use the medical record documentation to assign EOD when there is a discrepancy between the T, N, M information and the documentation in the medical record.” See Discussion. |
We know that physician TNM staging is not always accurate, and we also know that doctors sometimes use information in assigning their TNM which may not be available to registrars. Is it a discrepancy when the documentation in the chart is unclear or not definitive, yet the physician assigns a TNM that seems to incorporate that documentation? Or is a discrepancy an obvious conflict between chart documentation and the doctor’s staging – such as a mis-assignment of TNM category that doesn’t at all match with clear and complete medical record documentation, or the physician’s use of criteria that should be excluded from the TNM assignment per AJCC guidelines? A real case example is a patient with breast carcinoma, imaging states 12 cm tumor with thickening of dermis, and thickening of morphologically suspicious internal mammary and level 1-2 axillary lymph nodes. Medical oncologist states locally advanced breast cancer with extensive changes involving skin thickening associated with the mass, at least stage IIIC based on imaging and exam findings, cT4 N3b. Only axillary nodes were sampled and found to be positive. Post-neoadjuvant therapy resection showed only focal DCIS. Per EOD guidelines, would the oncologist’s staging be a discrepancy with the chart documentation and therefore ignored, with EOD-Primary Tumor coded 200 for skin thickening, and EOD-Lymph Nodes 200 for involvement of axillary nodes only? Or would the doctor’s TNM be a clarification/confirmation of documentation terms that we otherwise would not code, with EOD-PT coded 400 for extensive skin involvement and EOD-LNs 600 for internal mammary + axillary nodes? |
Use all information available in the medical record. EOD is a combination of the most precise clinical and pathological documentation of the extent of disease as instructed in the EOD 2018 General Instructions, Extent of Disease section. EOD 2018 General Instructions, General Coding Instructions section advises to use the medical record documentation to assign EOD when there is a discrepancy between the T, N, M information and the documentation in the medical record. When there is doubt that the documentation in the medical record is complete, code the EOD corresponding to the physician staging. A discrepancy can exist within the medical record when the information in the chart is unclear, incomplete, or conflicting, for example, the TNM staging from pathology differs from the medical oncologist’s TNM staging. In the scenario provided, use the medical oncologist stage information that takes into account imaging and exam findings. Based on the stage cT4 N3b, assign EOD Primary Tumor: 400 Extensive skin involvement WITHOUT a stated diagnosis of inflammatory carcinoma WITH or WITHOUT dermal lymphatic filtration EOD Regional Nodes: 600 Internal mammary node(s), ipsilateral, clinically apparent (On imaging or clinical exam) WITH axillary (level I, II, or III) lymph node(s), ipsilateral including infraclavicular |
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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20240019 | Solid Tumor Rules/Histology--Head and Neck, Other Sites: Do human papilloma virus (HPV) histologies that occur with subtype/variants of squamous cell carcinoma (SCC) in various sites apply only to sites in Solid Tumor Rules, Head and Neck, Table 5 and Other Sites, Table 23? See Discussion. |
The 2024 Solid Tumor Rules, Table 5: Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids contain notes that say beginning 1/1/2022, keratinizing or non-keratinizing SCCs, HPV positive or HPV negative, are coded 8085 or 8086, respectively, for sites listed in the Head and Neck Solid Tumor Rules, Table 5 only. Table 5 introductory section also states for cases diagnosed 1/1/2023 forward: “When the diagnosis is a subtype/variant of squamous cell carcinoma and HPV status is also noted, code the subtype/variant.” This latter instruction is also included in Other Sites Table 23 (Penis and Scrotum Histologies) as a “Penis Coding Note.” Do these instructions ONLY apply to sites on those tables (and only to Penis or to Scrotum also in Table 23)? How should we code HPV-related keratinizing/non-keratinizing or other subtype/variant SCCs, for sites NOT on those tables, given the fact that only the more common histologies are listed in the Solid Tumor tables? For example, we recently reviewed a case with HPV-positive basaloid squamous cell carcinoma of the anus (C21.0). |
Code the specific histology as stated by the pathologist according to the site-specific instructions in the Solid Tumor Rules. When the histology provides a subtype/variant in addition to the HPV histology codes, code the subtype/variant as it is important to capture this histology as in the example provided. the instruction to code the subtype/variant over 8085 or 8086 applies to the following sites: oropharynx, cervix, vagina, vulva, anus, and penis. A note will be added indicating this in 2025. 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 (reference: Cancer PathCHART: Product Downloads and Timelines). Other coding resources will be updated to reflect these changes in 2025. |
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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