Report | Question ID | Question | Discussion | Answer | Year |
---|---|---|---|---|---|
|
20230070 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be accessioned for a diagnosis of invasive carcinoma of the left breast (8500/3) in 2020 followed by a 2023 diagnosis of dedifferentiated carcinoma in the left breast (8020/3)? See Discussion. |
The WHO Blue Books do not include dedifferentiated carcinoma as a valid histology for the breast. However, there is known to be progression of ductal carcinoma that is essentially dedifferentiation of an estrogen receptor, progesterone receptor, and HER2 breast carcinoma to a triple negative "dedifferentiated" carcinoma which it appears this patient has. Whether we should accession this as a separate 8020/3 primary per M14 is unclear and the Solid Tumor Manual does not address this scenario. |
Abstract a single primary using Breast Solid Tumor Rules, Rule M18, as none of the previous rules apply. Undifferentiated carcinoma is a malignant epithelial tumour lacking overt evidence of a specific line of differentiation. Dedifferentiated carcinoma is composed of an undifferentiated carcinoma and a differentiated component. Dedifferentiated carcinoma (8020/3) as a morphology is associated with cancer of the endometrium and ovary rather than the breast. Breast cancer shows a broad spectrum of morphology with extensive variation in histological type and grade, related to the complexity of carcinogenesis. This includes initial genetic changes in the cell of origin, subsequent genetic and epigenetic alterations, and reprogramming that occur at various stages of development along with interaction of other factors that influence the process of differentiation. This scenario likely represents the process of phenotypic change of a carcinoma at a later stage, better known as transdifferentiation. |
2023 |
|
20230036 | Reportability/Histology--Vulva: Is angiomyxoma (8841/1), such as aggressive angiomyxoma of vulva diagnosed in 2022, reportable? |
Do not report superficial angiomyxoma (8841/0) or aggressive angiomyxoma (8841/0). WHO Classification of Female Genital Tumors, 5th edition, defines deep (aggressive) angiomyoma as a benign, infiltrative, myxoid spindle cell neoplasm that occurs in deep soft tissue of the pelviperineal region. |
2023 | |
|
20230018 | SEER Manual/First Course Treatment--Chemotherapy: Does the First Course of Treatment end when subcategories change for treatments such as hormone therapy or immunotherapy or is that instruction specific to chemotherapy? See Discussion. |
Treatment for estrogen receptor positive (ER+) breast cancer started with tamoxifen (non-steroidal estrogen subcategory) and switched to letrozole (non-steroidal aromatase inhibitor subcategory). Patient being treated with immunotherapy, Avastin (cytostatic agent-antiangiogenesis agent subcategory), and then changed to Atezolizumab (monoclonal antibody subcategory). Is Atezolizumab a new course of therapy because it is a different subcategory? |
Answer updated April 2025 A change in the subcategory for a hormone drug does not indicate the end of First Course of Treatment because different hormone therapies generally achieve the same result. For example, some forms of breast cancer are estrogen-dependent and the various subcategories of hormone drugs used to treat them, such as gonadotropin-releasing factor agonists, aromatase inhibitors and estrogen antagonists, all achieve the same result - to block estradiol effects in these tumors. Similarly, a change in immunotherapy is not a new course of treatment. However, if a change to hormone therapy or immunotherapy is due to a change in the patient's ER, PR, or Her2 status, this could signify a new course of treatment. The instruction in the SEER Manual is specific to chemotherapy. Chemotherapy is the only systemic treatment for which a change in the subcategory of a drug indicates the end of First Course of Treatment, due to the fact that different chemical agents damage cancer cells in different ways and at different phases in the cell cycle. |
2023 |
|
20230039 | Histology/Hematopoietic and Lymphoid Neoplasms--AML: What is the histology code for Acute Myelogenous Leukemia (AML) with monocytic differentiation, 9891/3: acute monoblastic and monocytic leukemia or 9867/3: Acute myelomonocytic leukemia? |
Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist. Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned. Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3. |
2023 | |
|
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 |
|
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 | |
|
20240078 | Reportability/Histology--Lung: Are adenocarcinoma spectrum lesions on lung imaging reportable when no further information is available? See Discussion. |
For example, a chest computed tomography showed multiple subsolid and ground glass pulmonary nodules measuring up to 6 mm; findings favored to reflect adenocarcinoma spectrum lesions. A literature search seems to indicate that adenocarcinoma spectrum lesions include atypical adenomatous hyperplasia through invasive adenocarcinomas. |
Do not report this case of "adenocarcinoma spectrum lesions" based on the information provided in the absence of a more specific diagnosis. Do not report until/unless a definitive diagnosis of malignancy is made. "Adenocarcinoma spectrum lesion" covers a continuum of lung neoplasms from preinvasive lesions (atypical adenomatous hyperplasia and adenocarcinoma in situ) to invasive lesions (minimally invasive adenocarcinoma and invasive adenocarcinoma). Should additional information become available, report the case and assign the histology code if a more specific histology is confirmed later. Use text fields to record the details.
|
2024 |
|
20240049 | First Course Treatment/Neoadjuvant Therapy--Breast: When are pre-operative therapies given as part of a clinical trial coded as neoadjuvant treatment versus limited systemic exposure in the Neoadjuvant Therapy data item? See Discussion. |
The SEER Manual seems to give somewhat conflicting instructions for clinical trial therapies under the Neoadjuvant Therapy data item. One section states that limited systemic therapy may occur in clinical trials to impact the biology of a cancer, but is not a full course of neoadjuvant therapy with the intent to impact extent of surgical resection or other outcomes (organ preservation, function or quality of life); do not code as neoadjuvant therapy for the purposes of this data item. Then another section states for purposes of this data item, the criteria for neoadjuvant therapy include that treatment must follow recommended guidelines for the type and duration of treatment for that particular cancer site and/or histology, and that neoadjuvant therapy may be given as part of a clinical trial. For example, a patient was diagnosed with invasive ductal carcinoma of the breast, 6 cm in size; treatment planning conference recommended neoadjuvant chemotherapy. The patient elected to participate in a clinical trial and was assigned to a group given the antibody drug conjugate datopotamab deruxtecan (Dato-DXd) plus durvalumab for 12 weeks. There was no physician documentation of intent or expected outcomes, nor yC staging or statement of clinical response. Post-therapy imaging showed no residual mass, and post-therapy mastectomy path report showed only residual ductal carcinoma in situ, stating "Treatment Effect (after neoadjuvant): Residual Cancer Burden - pCR, In the breast - complete response." The medical oncologist stated post-therapy stage was ypTis ypN0 cM0. The trial drugs this patient were given do not appear to be approved or standard neoadjuvant/pre-operative drugs in SEER*Rx or NCCN guidelines for this type of cancer; however, the duration of treatment was fairly substantial, and although we don't have clear documentation from physicians as recommended in the SEER manual (which is usually not stated, in our experience), it seems like they may be considering it as neoadjuvant therapy. How should the Neoadjuvant Therapy data item be coded for cases like this? What is the best way to differentiate between clinical trial therapies that are "limited systemic exposure" (code 3) versus true neoadjuvant therapy (code 1)? |
When pre-operative therapies are given as part of a clinical trial, code as neoadjuvant treatment in the Neoadjuvant Therapy data item when the intent is neoadjuvant and/or when surgical resection follows the clinical trial therapies. In the example, neoadjuvant chemotherapy was recommended in the treatment planning and the patient had the planned resection after neoadjuvant treatment. The treatment effect outcome is based on imaging that reported no mass and as documented by the physician, pathologist in this case as complete response to the neoadjuvant therapy based on the resection. Use code 3 (limited systemic exposure) when treatment does not meet the definition of neoadjuvant therapy in the data item, Neoadjuvant Therapy. Limited exposure occurs when the patient receives some therapy prior to surgical resection, but the treatment is not enough to qualify for a full course of neoadjuvant therapy with the intent to impact extent of surgical resection or other outcomes. While this type of treatment may given as part of a clinical trial, it mostly refers to short term treatments such as hormone therapy. When neoadjuvant therapy is given prior to surgical resection that is planned (intended) or performed to improve outcomes, use Code 1 or 2. Because a clinical trial is a type of research study that tests new methods of screening, prevention, diagnosis, or treatment of a disease, the treatment regimens likely will not be incorporated in recommended guidelines until all phases of the trial are completed and approved by the U.S. Food and Drug Administration. ClinicalTrials.gov is available to learn more about clinical studies around the world. |
2024 |
|
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 |
|
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 |