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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20240076 | SEER Manual/Reportability--Vulva: Is vulvar intraepithelial neoplasia (VIN II) alone reportable? An example is a final diagnosis from a pathology report that states only 'VIN II' with no additional details/wording. |
Report VIN II. The 2024 SEER Manual lists this as a separate diagnosis in the Reportability section under Malignant Histologies 1.a.x. |
2024 | |
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20240036 | Update to Current Manual/Race: How is Race coded when stated as Hispanic and there is no other information? See Discussion. |
There appears to be discrepant information in the 2024 (and prior) SEER manual regarding race coding when the patient is described only as Hispanic/Latina. Page 78 tells us to Code as 01 (White) when: b. There is a statement that the patient is Hispanic or Latino(a) and no further information is available
However, in Appendix D, under "Other Race descriptions", there is a statement that "If no further information is available, code as 99 Unknown." The list includes "Hispanic." |
Assign code 01 (White) for Hispanic when there is no additional information. It is listed in the 2024 SEER Manual, Race Coding Instruction 6.b.i. and in Appendix D for code 01. We will remove Hispanic from the list in Appendix D under code 99 in the next version of the manual. |
2024 |
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20240041 | Reportability--Brain and CNS: Is an optic nerve meningioma reportable if stated to arise in the “intraorbital segment” of the optic nerve meninges? See Discussion. |
Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma. Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location. It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable. |
Report optic nerve sheath meningioma arising in the intraorbital segment. The optic nerve contains four segments, of which intraorbital is one. The WHO Classification of Eye Tumors, 4th edition, defines meningioma as a neoplasm originating from the meningothelial cells of the optic nerve leptomeninges. According to the Table 3 of the Non-malignant Solid Tumor Rules, all portions of the optic are reportable and meningiomas arising in the dura/meninges of an intracranial nerve are coded to cerebral meninges C700. |
2024 |
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20240074 | Solid Tumor Rules/Histology--Head & Neck: How is histology coded for nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type? See Discussion. |
Example: Patient had a 2023 nasopharyngeal mass biopsy showing “Nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type.” The Head and Neck Solid Tumor Rules (STRs) do not include an H Rule that instructs us how to code histology when there are two subtypes/variants present for a head and neck primary, nor does the STR define undifferentiated carcinoma as a subtype/variant for 8072. The WHO Classification of Head and Neck Tumors states non-keratinizing nasopharyngeal carcinoma (non-keratinizing squamous cell carcinoma (SCC) is the most common subtype for nasopharyngeal ca, but that non-keratinizing can be subdivided into undifferentiated and differentiated subtypes. Should histology be 8020 (undifferentiated carcinoma) or 8072 (non-keratinizing SCC)? |
Assign histology as 8072 for non-keratinizing SCC, undifferentiated subtype. WHO Classification of Head and Neck Tumors, 5th edition assigns 8072/3 to squamous cell carcinoma, non-keratinizing, NOS in the nasopharynx. As the tumor exhibits a variety of architectural patterns and appearances histologically, they can be further classified as undifferentiated or differentiated subtypes. These subtypes do not change the histology code. |
2024 |
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20240017 | EOD/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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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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20240027 | Solid Tumor Rules/Multiple Primaries--Brain and CNS: How many primaries are accessioned when a 2005 diagnosis of glioblastoma multiforme is followed by a 2024 diagnosis of astrocytoma, IDH-mutant, WHO grade 4? See Discussion. |
The patient underwent a gross total resection of the 2005 glioblastoma multiforme (9440/3). The patient was subsequently diagnosed with a 2024 diagnosis of astrocytoma, IDH-mutant, WHO grade 4 (9445/3). Should Rule M13 apply to the new 2024 diagnosis and a new primary be accessioned because astrocytoma, IDH-mutant, WHO grade 4 is listed on a different row than glioblastoma? It is unclear whether histology 9445 should be classified as being on a different row because it is also listed as a subtype/variant for glioblastoma in Table 3. Table 3 lists histology 9445 as both “Astrocytoma, IDH-mutant, WHO grade 4” and as “Glioblastoma IDH-mutant.” |
Abstract two primaries using the 2024 Malignant Central Nervous System (CNS) and Peripheral Nerves Solid Tumor Rules, Rule M13. Glioblastoma, IDH-wild-type (9440/3) and astrocytoma, IDH-mutant, grade 4 (9445/3) are on two separate rows in Table 3 of the Malignant CNS and Peripheral Nerves Solid Tumor Rules. WHO Classification of Central Nervous System, 5th edition, lists the subtypes of glioblastoma, IDH-wild-type as giant cell glioblastoma; gliosarcoma; and epithelioid glioblastoma. The term glioblastoma multiforme is not recommended for glioblastoma, IDH-wildtype in the 5th edition, and lists astrocytoma, IDH-mutant, grade 4 as a subtype of astrocytoma, IDH-mutant. |
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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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 |