| Report | Question ID | Question | Discussion | Answer | Year |
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20230001 | Solid Tumor Rules/Multiple Primaries--Lung: How many primaries should be reported when two separate squamous cell carcinoma (SCC) tumors, one in the left upper lobe (LUL) and one in the right lower lobe (RLL), are diagnosed? The tumors are separated by an interval occurring right hilar lymph node biopsy proving metastatic pulmonary adenocarcinoma without a clear description of a corresponding interval occurring lung tumor. See Discussion. |
The patient was diagnosed with a biopsy-proven 12/2020 LUL SCC treated with radiation only, followed by a right hilar lymph node biopsy in 07/2022, that proved “metastatic pulmonary adenocarcinoma” per pathology and treated with radiation, followed by a biopsy-proven 12/2022 RLL SCC to be treated with immunotherapy only. The imaging never definitively identified a lung tumor that can be assumed to be a primary adenocarcinoma tumor. In 06/2022, a PET scan only described a “strongly PET positive Rt inferior hilar LN vs infrahilar pulmonary mass,” as well as the subsequently biopsy-proven SCC in the RLL (12/2022 SCC primary). The biopsy path indicates this was a right hilar lymph node metastasis and does not indicate this is an infrahilar pulmonary mass. No other PET positive pulmonary lesions were seen at the time. The oncologist’s assessment indicates the right hilar node was the only positive finding on the biopsy, and it was unclear if this right hilar node metastasis was from the left lung or if the primary was “not detectable.” The oncologist summarized this as a LUL lung lesion radiated for SCC, a right hilar lesion radiated for adenocarcinoma, and a RLL lung lesion on pathology found to be SCC. Should the interval occurring metastatic adenocarcinoma be accessioned as a separate lung, NOS primary based on the histology difference? While the Solid Tumor Rules do not apply to metastasis, the oncologist did treat these three malignancies separately and does not indicate the hilar lymph node metastasis was felt to be from either SCC primary. |
Abstract three primaries based on this scenario. 1 – 2020, SCC LUL lung 2 – 2022, Adenocarcinoma lung, described as metastatic pulmonary, based on biopsy of right hilar node (Rule M8) 3 – 2022, SCC RLL lung (Rule M11) |
2023 |
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20230021 | Histology--Soft Tissue: How is histology coded for malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement diagnosed on left shoulder excision? See Discussion. |
March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation. Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement? |
NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3). WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics. |
2023 |
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20230052 | Reportability/Primary Site--Brain and CNS: What is the primary site of a meningioma arising from the jugular bulb/petrous aspect of the temporal bone? See Discussion. |
Example July 2022, Brain CT describes a mass appearing to be centered on the petrous aspect of the temporal bone with intracranial and extracranial extension. July 2022, Brain MRI describes an extra-axial mass centered in the right jugular bulb with intracranial and intraosseous extension as well as extension within the internal jugular vein. September 2022, Resection operative report surgical findings are of a calcified mass filling middle ear, abutting stapes and appearing to enter the stapes obturator foramen, debulked. Final diagnosis is right middle ear meningioma, WHO grade I of III. Is this a reportable intraosseous meningioma of the temporal bone/skull base, or a non-reportable meningioma arising in a meningocele within the middle ear? |
Do not report cases of meningioma originating in the jugular bulb or petrous aspect of temporal bone or middle ear. These are not intracranial locations. This is a non-reportable meningioma arising in a meningocele within the middle ear. The jugular bulb is the confluence of the lateral venous sinuses situated in the jugular fossa. The precise location of this structure within the temporal bone is variable.The jugular bulb, petrous aspect of temporal bone, and middle ear are not intracranial locations, and therefore meningiomas arising in these areas are not reportable. |
2023 |
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20230007 | SEER Manual/Reportability--Appendix: Is low-grade appendiceal mucinous neoplasm (LAMN) with peritoneal spread followed by evidence of extraperitoneal metastatic disease reportable prior to 2022? See Discussion. |
In 2021, the patient was diagnosed with a non-reportable appendiceal LAMN. Resection showed a tumor diffusely involving the appendix and perforating the visceral peritoneum, as well as extensive intraperitoneal metastasis. In 2023, a lung wedge resection revealed metastatic mucinous neoplasm involving lung parenchyma and pleura, consistent with metastasis of the known appendiceal primary. It is understood that intraperitoneal spread of an appendiceal LAMN does not make it reportable because the peritoneal disease is also non-invasive. Does extraperitoneal metastasis of an appendiceal LAMN diagnosed prior to 2022 make it invasive disease and therefore reportable? |
LAMN diagnosed prior to 1/1/2022 is not reportable even when it spreads or metastasizes according to our expert pathologist consultant. Spread of this neoplasm does not indicate malignancy. For this case to be reportable, the diagnosis must indicate “carcinoma” or “adenocarcinoma.” Pre-2022, LAMN is not reportable even when treated with surgery and chemotherapy. LAMN is reportable starting with cases diagnosed in 2022. |
2023 |
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20230048 | Solid Tumor Rules/Histology--Uterine Corpus: How is histology coded for an epithelioid and myxoid leiomyosarcoma of the myometrium? See Discussion. |
Patient had a total abdominal hysterectomy-bilateral salpingo-oophorectomy performed in January 2023 with final diagnosis of myxoid and epithelioid leiomyosarcoma. Diagnosis comment states: The tumor is 15 cm per report. It grows in nests and poorly formed interanastomosing trabeculae and cords that are separated by abundant myxoid background. The cells have an epithelioid morphology with eosinophilic cytoplasm, large nuclei, and very prominent nucleoli. The mitotic activity is overall low ranging from 1 to 3/10 HPFs. Immunohistochemical stains performed at the outside hospital showed diffuse positivity for SMA, desmin, caldesmon, and PR. They are negative for CD10, claudin-4, calretinin, HBM45, MART1 (rare weakly positive cells), PANCK, and SOX10. This immunohistochemical profile supports a smooth muscle derivation of this neoplasm. As this tumor is extensively myxoid, diagnostic criteria differ from the spindle cell leiomyosarcoma. Per Solid Tumor Rules Other Sites, Table 16: Uterine Corpus Histologies, Epithelioid Leiomyosarcoma (8891/3) and Myxoid Leiomyosarcoma (8896/3) are both subtypes of Sarcoma, NOS (8800/3). Per Rule H21, use a combination code when there are multiple specific histologies AND the combination is listed in Table 2 OR there are coding instructions for the combination in the applicable histology Tables 3-21 OR you receive a combination code from Ask A SEER Registrar. Since there is no combination listed in Table 2 and there is no instruction for the combination in Table 16, how should the histology be coded for this tumor? |
Assign code 8891/3 (epithelioid leiomyosarcoma) as cells were described as have an epithelioid morphology; whereas, myxoid was used as a descriptive term and not a specific histologic type. |
2023 |
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20230058 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be accessioned for a patient with known history of right breast carcinoma in 2018 followed by 2022 biopsy proven right and left breast invasive ductal carcinoma if the physician states this is a right breast primary with widespread metastasis including the left breast? See Discussion. |
The patient was initially diagnosed with invasive mammary carcinoma of the right breast in 2018, treated with lumpectomy, sentinel node biopsy, radiation, and hormones. Hormones were discontinued early due to dysfunctional uterine bleeding. |
This is a single primary according to the Solid Tumor Rules.
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2023 |
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20230064 | Primary Site--Cervix Uteri: When no other information is available regarding the origin of the tumor, can an overlapping cervical adenocarcinoma (C538, 8140/3) be coded to the endocervix (C530) based on the histology? See Discussion. |
Adenocarcinoma is a glandular tumor and the endocervix is generally the origin of glandular tissue for the cervix. However, if the only available information is pathology proving a single tumor overlapping the endocervix and exocervix, can we code the site to C530 instead of C538? Applying the current primary site coding instructions, primary site would be coded as C538 because there is no specific statement of the tumor origin; the primary site coding instructions state the tumor is coded to an overlapping site in the absence of a specific statement of origin and there is no existing SINQ confirming the site can be assumed to be the endocervix based on the histology. |
Code Primary Site as Overlapping lesion of cervix uteri (C538). The 2023 SEER Program Coding and Staging Manual Primary Site Coding Instructions for Solid Tumors #4 says to code the last digit of the primary site code to ‘8’ when a single tumor overlaps an adjacent subsite(s) of an organ and the point of origin cannot be determined. This is also supported by the ICD-O-3, 3rd edition, note in the Topography section that states: In categories C00 to C809, neoplasms should be assigned to the subcategory that includes the point of origin of the tumor. A tumor that overlaps the boundaries of two or more subcategories and whose point of origin cannot be determined should be classified to subcategory ‘8.” |
2023 |
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20230056 | Reportability/Histology--Heme and Lymphoid Neoplasms: What is the histology code for nodular lymphocyte predominant B cell lymphoma that is never called Hodgkin lymphoma? Is it acceptable to record the histology code for nodular lymphocyte predominant Hodgkin lymphoma, (9659/3)? See Discussion. |
Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.” |
According to the 5th edition WHO Blue Book for Hematopoietic Neoplasms, Beta Version, (not released yet), nodular lymphocyte predominant B-cell lymphoma is an alternate name for 9659/3. We will update the Heme database once the 5th edition is released in print. |
2023 |
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20230030 | Primary site: Is there a physician priority list for coding primary site? For example, the surgeon states during a pancreatectomy that the primary is in body while the pathologist states in their synopitc report that primary is neck; neither is in agreement, or neither is available for confirmation. |
As a general rule, the surgeon is usually in a better position to determine the site of origin compared to the pathologist. The surgeon sees the tumor in its anatomic location, while the pathologist is often using information given to him/her by the surgeon and looking at a specimen removed from the anatomic landmarks. However, when a pathologist is looking at an entire organ, such as the pancreas, he/she may be able to pinpoint the site of origin within that organ. In the case of pancreas body vs. neck, the neck is a thin section of the pancreas located between the head and the body. It may be a matter of opinion whether a tumor is located in the "body" vs. the "neck." In the situation you describe, we would give preference to the surgeon and assign the code for body of pancreas, C251. |
2023 | |
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20230069 | First Course Treatment/Immunotherapy--Colon: Is infliximab cancer directed treatment? See Discussion. |
While SEER*Rx does indicate infliximab should be coded as biological response modifier (BRM)/Immunotherapy, the manufacturer website for this medication indicates it is given for: Crohn’s disease, ulcerative colitis, rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and plaque psoriasis. In addition, SEER*Rx does not indicate which primary sites this treatment may be given for. If it is indeed cancer directed treatment, can the typical primary sites be added for clarity? Case example: Patient is diagnosed with colorectal cancer and also has an existing diagnosis of Crohn’s disease; received surgery and FOLFOX6, as well as infliximab. There was no statement of what disease the infliximab was given to treat. |
infliximab is not cancer-directed treatment. This drug was last updated by the FDA 2/22/2023 with additional information on its approval to treat non-malignant neoplasms. To date, the FDA has not approved it for use in colon cancer. This drug was intially developed to treat colon cancer; however, found to be ineffective treating cancer. |
2023 |
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