Report | Question ID | Question | Discussion | Answer | Year |
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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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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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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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20240006 | Primary Site/Histology--Heme & Lymphoid Neoplasms: What are the correct primary site and histology for patient diagnosed with an oropharyngeal soft tissue mass revealing plasma cell neoplasm with 5-10% of marrow cellularity in 2022? See Discussion. |
Patient underwent excision of an oropharyngeal soft tissue mass revealing plasma cell neoplasm with extensive amyloid deposition. During work-up, bone marrow biopsy also revealed involvement by plasma cell neoplasm, with 5-10% of marrow cellularity. No amyloid seen in bone marrow. Patient was referred for radiation of the oropharyngeal mass. Per medical oncology qualifying best for the diagnosis of solitary extramedullary plasmacytoma with minimal marrow involvement. Decision made for observation by medical oncology in view of “minimal” bone marrow involvement. Question: Is rule M11 correct, and I abstract this case as a plasma cell myeloma, 9732/3, C421? |
Code as an oropharyngeal primary site and histology as solitary plasmacytoma (9734/3) based on consultation with our hematological expert. The WHO Classification of Hematopoietic and Lymphoid Tissues defines multiple myeloma as "bone marrow plasma cell percentage >60%." There are several other factors, but the bone marrow involvement is the key point for your case. The pathologist also states that the bone marrow is consistent with "plasma cell neoplasm," which by itself is not the same as multiple myeloma. This case has 5-10% involvement by plasma cell neoplasm. This does not meet the bone marrow qualifications for multiple myeloma and is consistent with the pathologist's statement that there is minimal bone marrow involvement. We will be updating the Hematopoietic and Lymphoid Neoplasms Database and Manual to clarify this (2025 updates). |
2024 |
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20240005 | SEER Manual/Mets at Diagnosis--Lung: Would calvarium lesions invading the brain be both brain and bone metastasis or only bone metastasis? See Discussion. |
Lung cancer, 2022 12/1/2022 PET/CT showed destructive hypermetabolic bone lesions in right frontal and left posterior calvarium. Left posterior calvarium lesion involves portions of left parietal and temporal bones w/invasion of mastoid air cells. 1/4/2023 MRI Brain showed large destructive mass involving left posterior temporal calvarium that extends into left mastoid region and may invade left distal transverse sinus. 2/8/2023 Radiation Oncology follow-up note: MD states there are extensive calvarium metastasis with the left parietal lesion invading the brain causing edema and MS-like changes. 2/13/23 Radiation Oncology Final Letter- Patient was treated with 1 EBRT fraction aimed at brain/skull before enrolling in hospice. |
Abstract as bone metastasis for the first two examples. Abstract as both bone and brain metastasis for the third and fourth examples in the respective Mets at Diagnosis fields based on the description provided. |
2024 |
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20240004 | Reportability/Histology--Skin: Is a malignant spindle cell neoplasm consistent with atypical fibroxanthoma reportable for cases diagnosed 1/1/2023 and later, after thorough immunohistochemical work-up? See Discussion. |
Appendix E1 in both the 2023 and 2024 SEER Program Coding and Staging Manual (SPCSM) lists these malignant spindle cell neoplasms, consistent with atypical fibroxanthoma, as reportable when other tumors have been ruled out with immunohistochemistry. This contradicts both SINQ 20190102 and the Solid Tumor Rules (STRs) general instructions indicating ambiguous terminology (e.g., “consistent with”) cannot be used to code the more specific histology when there is a NOS (malignant spindle cell neoplasm, 8004/3) and a more specific (malignant atypical fibroxanthoma, 8830/3) histology. These tumors are typically diagnosed and treated in dermatology offices, so further chart review or confirmation by a physician is not possible for central registries. As non-melanoma skin primaries are included in the Other Sites schema, and this schema was updated for cases diagnosed 2023 and later, which instruction applies to 2023+ diagnoses? Should these continue to be collected per Appendix E1 despite the conflict with the STR Manual and SINQ? If these are reportable, should the SINQ and STR Manual be updated to reflect this? Or should these be non-reportable per the STR Manual and SINQ? |
Report malignant spindle cell neoplasms consistent with atypical fibroxanthoma as directed by Appendix E.1 of the 2023 and 2024 versions of the SEER Manual using 8830/3 (fibroxanthoma, malignant). We will update the answer in SINQ 20190102. While the Other Sites Solid Tumor Rules address coding an NOS and specific histology sub-type/variant, this situation is not specifically addressed. We will also review the rules. |
2024 |
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20240003 | Solid Tumor Rules/Histology--Head & Neck: How is histology coded for laryngeal intraepithelial neoplasia II-III (LIN II or LIN III)? See Discussion. |
Laryngeal intraepithelial neoplasia II-III is not included in the ICD-O-3.2 and, while the SEER Program Coding and Staging Manual (SPCSM) confirms this is reportable, neither the SPCSM nor the Solid Tumor Rules Manual provide the specific histology to use for LIN II or LIN III. Should this be coded as 8077/2 since this is most like a high grade squamous dysplasia? |
Assign histology code, 8077/2 (squamous intraepithelial neoplasia, high grade) for LIN III and for LIN II. ICD-O-3.2 lists squamous intraepithelial neoplasia, grade II and grade III as 8077/2 indicating it is reportable. ICD-O-3.2 does not list every site-specific type of intraepithelial neoplasia. Check the SEER manual for reportable and non-reportable examples. |
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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20230080 | Solid Tumor Rules/Histology--Brain and CNS: What is the histology code for low grade glioma? See Discussion. |
Patient has a 3 cm tumor in the temporal lobe of the brain. This was noted on MRI 12/2022. The radiologist states this is a low-grade glioma and recommends following with routine scans. No pathology or resection performed or planned. Patient has been followed with imaging every six months with stable disease. Low grade glioma is not currently listed in ICD-O-3.2 or the current Solid Tumor Rules. What histology should be assigned to the case? |
Assign 9380/1 for low grade glioma diagnosed 1/1/2018 forward and for low grade glioma diagnosed prior to 1/1/2018 assign code 8000/1 on the advice of our expert neuropathologists. The site/type combination of C71 _ and 9380/1 will flag histology/site/behavior edits which should be overridden. Low grade glioma is an umbrella term or non-specific diagnosis, primarily seen on radiologic reports such as CT scans and MRIs. Often, the patient is actively followed with scans and surgical intervention delayed or not recommended. WHO Classification of Central Nervous System Tumors, 5th edition, does not recognize this term and indicates that tissue diagnosis (including genetic testing) is needed to provide a specific diagnosis. Since biopsy of these “neoplasms” is not routinely done, a definitive diagnosis is not available. Literature searches yielded conflicting information with some stating low grade gliomas are malignant with an indolent clinical course while others felt they were benign. Until such time as WHO proposes a code for this neoplasm, our expert neuropathologists recommend coding glioma, NOS with borderline behavior 9380/1. |
2023 |
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20230079 | Solid Tumor Rules/Histology--Cutaneous Melanoma: How is histology coded for a 2023 diagnosis of “early lentiginous melanoma in situ” of the skin? See Discussion. |
Previous SINQ 20091100 has a similar scenario and the instruction was to code as lentigo maligna (8742/2); however, it does not appear to be applicable to cases diagnosed after 2020. The WHO Blue Book does not list melanoma, lentiginous type or lentiginous melanoma in situ as an alternate term for lentigo maligna and neither do the STR or the ICD-O-3.2. |
Assign code 8742/2 (lentigo maligna) for “early lentiginous melanoma in situ.” ICD-O-3.2 lists the preferred term for 8742/2 as lentigo maligna (C44._). |
2023 |