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20240077 | 2024 SEER Manual/Primary Site--Retroperitoneum: What is the primary site code for a final diagnosis of endometrioid adenocarcinoma from a biopsy of a right retroperitoneal mass? See Discussion. |
An 80-year-old post-menopausal female (status post hysterectomy for benign reasons) presents with a retroperitoneal mass on imaging. The pre-operative imaging shows the cervix and uterus are absent. Patient undergoes a robotic left salpingo-oophorectomy with biopsy of the retroperitoneal mass. |
Code Primary Site to C480 (retroperitoneum). Endometrial tissue may "break away” from the uterus and implant throughout the pelvic and abdominal cavities. This can occur in patients who suffer from endometriosis. This tissue remains behind when surgical removal of the uterus is done. Common sites of implantation are colon, peritoneum, retroperitoneum, and bladder. These cells may become malignant. When the uterus is no longer present (patient had surgical removal), code the site where the carcinoma was identified. The site-morphology combination of C480 and 8380/3 was designated as an unlikely site-morphology combination by the Cancer PathCHART expert pathologist review, as this is a rare type of tumor. Assign a value of 1 in the Over-ride Site/Type [2030] data item in order to pass the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) edit. |
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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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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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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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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20240064 | Primary Site/Histology--Ovary: We are encountering a primary site, histologic type, and behavior combination edit based on the Cancer PathCHART (CPC) tables. Using the CPC*Search tool, C569 and 8441/3 is a valid combination. The diagnosis date is 01/13/2024. Should an over-ride be applied with this combination? |
The CPC Validity Status of the site morphology combinations of C569/8441/3 and C569/8441/2 was revised from Valid to Unlikely with the latest release of the Version v24A Edits Metafile. As a result, this site and morphology combination will now require an over-ride flag to be set. Code as 8461/3 (high-grade serous carcinoma) or 8460/3 (low-grade serous carcinoma) if at all possible. Use 8441/3 (serous carcinoma, NOS) only if it cannot be distinguished as low grade or high grade. The codes for high-grade serous carcinoma and low-grade serous carcinoma are relatively new. High-grade serous carcinoma and low-grade serous carcinoma are very different tumors and pathologists should state whether it is high grade or low grade. Please make every attempt to use the newer codes. If unable to determine high gade versus low grade, assign 8441/3 and override the edit. The files on the CPC website are currently being updated, and CPC*Search will be updated to reflect the changes sometime this Fall. |
2024 | |
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20240026 | Update to Current Manual/Reportability--Pancreas: For cases diagnosed 2024+, is a diagnosis of pancreatic intraepithelial neoplasia II (PanIN II) reportable? If so, how should histology be coded? See Discussion. |
SEER Program Coding and Staging Manual: Reportability – Reportable Diagnosis List indicates pancreatic intraepithelial neoplasia (PanIN II) (C250-C259) is reportable. However, the ICD-O-3.2 lists “Glandular intraepithelial neoplasia, grade II” and “Glandular intraepithelial neoplasia, low grade” as histology code 8148 with behavior of /0 (benign). |
Do not report PanIN II. WHO Classification of Digestive Tumors, 5th edition, now categorizes PanIN into two categories, low grade (8148/0) and high grade (8148/2). PanIN grade I and PanIN grade II are categorized as PanIN low grade; PanIN grade III is categorized as PanIN high grade. We will update the Reportability section of the manual. |
2024 |
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20240079 | Reportability/Histology--Conjunctiva: Is low-grade conjunctival melanocytic intraepithelial lesion (LG-CMIL) with focal high-grade features of the conjunctiva (C690) reportable? If reportable, what histology should be assigned? |
Additional comments in this pathology report state "The entire case was sent in consultation to an ophthalmic pathologist. [Pathologist] assigns a conjunctival melanocytic intraepithelial neoplasia (C-MIN) score of 2-3 due to the upward pagetoid migration of small, dendritic melanocytes. A C-MIN score of 2-3 is between low-grade conjunctival melanocytic intraepithelial lesion (LG-CMIL; C-MIN 2) and high-grade conjunctival intraepithelial lesion (HG-CMIL; C-MIN 3). The older terminology for this lesion would be primary acquired melanosis (PAM) with mild to focally moderate atypia." This term does not appear in the SEER Program Coding and Staging Manual (SPCSM), Appendix E1 of the SPCSM, or Solid Tumor Rules (specifically rule H3) . |
Conjunctival melanocytic intraepithelial neoplasia (C-MIN) is reportable; therefore, low-grade conjunctival melanocytic intraepithelial lesion (LG-CMIL) with focal high-grade features of the conjunctiva (C690) is reportable, 8720/2. We will add this to a future edition of the SEER manual. |
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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20240065 | Solid Tumor Rules/Histology--Ovary: What is the histology code for an ovarian primary with a pathology report final diagnosis of “Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant” diagnosed in 2012 (using the Multiple Primaries H rules) and one diagnosed in 2024 (using the Solid Tumor Rules)? See Discussion. |
2012 Total abdominal hysterectomy - bilateral salpingo-oophorectomy Primary Site – Ovary, Right Histology - Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant 2024 Total abdominal hysterectomy - bilateral salpingo-oophorectomy Primary Site – Ovary, Left Histology - Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant |
Abstract this case as a single primary. Code as 8044/3 (small cell carcinoma, hypercalcemic type) listed in the Other Sites Solid Tumor Rules, Table 13. Small cell carcinoma, large cell variant, is a subtype of small cell carcinoma, hypercalcemic type. This table does not include all possible histologies. WHO Classification of Female Genital Tumors, 5th edition, states: Small cell carcinoma of the ovary, hypercalcemic type, is rare, accounting for < 1% of ovarian tumors. Small cell carcinomas, hypercalcemic type, are usually large, with a mean size of 15 cm (range: 6–26 cm). Large cells are present (in varying numbers) in half of these tumors, which are designated “small cell carcinoma, large cell subtype” if the large cells are predominant (which is rare). |
2024 |
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