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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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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 |
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20240011 | Solid Tumor Rules/Histology--Other Sites: Other Sites Table 2 (Mixed and Combination Codes) requires site designations; can sites be added? See Discussion. |
There are multiple possible entries (rows) for a tumor with a neuroendocrine component and non-neuroendocrine component, but these rows do not specify which primary sites are applicable. Row 1 (Combined small cell carcinoma, 8045) seems applicable to a prostate primary, but not to a GI primary since GI primaries are now generally referred to as MiNENs (mixed neuroendocrine non-neuroendocrine tumors), but Table 2 does not provide any instructions regarding how to determine the difference between 8045 and 8154 (or 8244). For SEER Workshop Case 03 (mixed prostate case), many users selected 8154 or 8244 as the mixed histology code per Table 2, but these histology codes are not listed as applicable in Table 3 (Prostate Histologies). Per the WHO Blue Books, these histologies are not listed as applicable to the prostate. How are registrars to determine the correct mixed code without site designations, especially if they don't have access to the WHO Blue Book or to a pathologist who may be able to clarify the codes? |
Sites may be added to certain combinations when indicated by ClinCORE review for Cancer PathCHART. Please note some sites were added in the 2024 update as a result of PathCHART review. A newly-formed Solid Tumor Editorial Board and its subgroups are currently working to evaluate the Solid Tumor Manual and make recommendations on ways to improve the structure and formatting of the manual and its content. Follow the rules and instructions in the Other Sites STRs when assigning combination histology codes. Histology Coding Rules Use the Histology Coding Rules when assigning combination codes. Coding Histology Information Use this section that includes the mixed histology (Table 2) and site-specific histology tables (Tables 3-23) for one or more histologies within a single tumor. Do not use this section in place of the Histology Coding Rules. While site-specific histology tables, based on current WHO Classification of Tumors books, have been added to Other Sites STRs, not all site groups have individual histology tables; coding may require the use of ICD-O and updates. The histology tables in Other Sites STRs include additional coding instructions and notes to assign the correct ICD-O code when appropriate. The tables are not meant to be all-inclusive; rather they are intended to address difficult coding situations to facilitate the assignment of the correct histology code. Table 2: Mixed and Combination Codes Instructions Once you have identified the histology terms and have been instructed to use Table 2 by the Histology Coding Rules, compare the terms in the diagnosis to the terms in Column 1. When the terms match, use the combination code listed in Column 2. Use adenocarcinoma mixed subtypes 8255 as a “last resort” code. |
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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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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20240055 | Update to the Current Manual/Tumor Size Summary—Neoadjuvant Treatment: Would you clarify instructions in the 2024 SEER Program Coding and Staging Manual (SPCSM) for Tumor Size Summary when a patient receives neoadjuvant treatment? There seems to be a conflict with the STORE Manual. See Discussion. |
Starting for cases diagnosed in 2024, the SPCSM manual no longer requires the data items for clinical and pathologic tumor size. Instead, it appears to align with the CoC data item of Tumor Size Summary. The two manuals contradict each other when it comes to coding tumor size summary for neoadjuvant chemotherapy (NAC) treated cancers. STORE states: "If neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999." 2024 SPCSM states "If neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999." It continues to state 12. Assign code 000 when…. (a) no residual tumor is found…(i) Neoadjuvant therapy has been administered and the resection shows no residual tumor & 14. Assign code 999 when...(d) Neoadjuvant therapy has been administered and resection was performed. Do not use a post-neoadjuvant size to code pathologic tumor size; however, you may use the clinical tumor size if available It seems that we will lose the value of the tumor size summary if we code 000 when NAC is administered and there is no residual disease. Example: Patient has a 90 mm triple positive breast tumor and is treated with neoadjuvant TCHP (docetaxel/carboplatin/ trastuzumab/pertuzumab). After completing neoadjuvant therapy, the patient has a mastectomy with no residual disease noted on the final pathology report. Using the 2024 SPCSM instructions, code 000 for Tumor Size Summary instead of 090 for the clinical tumor size of 90 mm tumor noted before NAC was administered. This has the potential to affect data analysis, research, and clinical trial accrual. |
When there is neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999. We will remove Coding Instruction 12.a.i in the next version of the manual. |
2024 |
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20240009 | Solid Tumor Rules/Histology --Brain and CNS: Why is high grade astrocytoma with piloid features (HGAP) not grouped together with the other astrocytoma histologies as a subtype/variant of astrocytoma? See Discussion. |
It appears there was some confusion about finding this new malignant HGAP tumor (2023+) code. If this is not a specific subtype/variant of astrocytoma, can clarification be added to the “New for 2023” entry for HGAP? |
HGAP is listed as a separate classification and is not a subtype of the diffuse gliomas. WHO Classification of Tumors of the Central Nervous System, 5th edition, has two categories dealing with non-pediatric astrocytic tumors: Adult-type diffuse gliomas Circumscribed astrocytic tumors HGAP falls into the second category as a result of updates to the 4th edition WHO classification in 2016 with advances in the role of molecular diagnostics with the 5th edition. All astrocytic tumors were previously grouped together whereas not all diffuse gliomas (astrocytic or not) are grouped together on the basis of growth pattern and behaviors, and shared IDH1 and IDH2 genetic status. The new classification separates astrocytomas that have a more circumscribed growth pattern, lack IDH gene alterations, and sometimes have BRAF mutations (i.e., pilocytic astrocytoma). The impact of molecular advances has driven classification changes as described in the 5th edition. Review of site/histologiy combinations for CNS neoplasms is currently being performed by Cancer PathCHART experts. It's possible they will recommend HGAP be moved to a subtype/variant of astrocytoma, NOS. |
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. |
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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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20240008 | Solid Tumor Rules/Histology--Brain and CNS: Should the term “diffuse” be added to Note 2 in the Non-Malignant Central Nervous System (CNS) Solid Tumor Rules, Table 6: Specific Histologies, NOS, and Subtypes/Variants, for the papillary glioneuronal tumor 9509/1? See Discussion. |
Should Note 2 state, "Beginning with cases diagnosed 1/1/2023 forward, diffuse leptomeningeal glioneuronal tumor is coded 9509/3? See the Malignant CNS rules." Currently the Note only states, "leptomeningeal glioneuronal tumor," but the histology that changed behavior is listed in both Table 6, Column 1 (Non-Malignant CNS) and Table 3 (Malignant CNS) as, "Diffuse leptomeningeal glioneuronal tumor." |
The correct term is diffuse leptomeningeal glioneuronal tumor listed as a synonym in Column 2. We will add the term diffuse in Note 2, Column 1 with the 2025 updates. In the meantime, you can add "diffuse" to your pdf version until the update is published. |
2024 |
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