| Report | Question ID | Question | Discussion | Answer | Year |
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20220049 | Solid Tumor Rules/Multiple Primaries--Lung: How many cases should be abstracted for a patient with 2022 wedge biopsy of right upper lobe acinar predominant lung adenocarcinoma and wedge biopsy of right lower lobe lepidic predominant adenocarcinoma if there is concern for diffuse spread throughout the lungs secondary to the lymphangitic carcinomatosis and possible diffuse pneumonic type of adenocarcinoma? See Discussion. |
Acinar predominant adenocarcinoma measures at least 12 mm and involves wedge biopsy margins, while the lepidic predominant adenocarcinoma measures 11 mm and does not involve the margins of that separate specimen. Pathologist also notes, “CT findings of diffuse coarse reticular nodular opacity, these findings may represent pneumonic type adenocarcinoma/diffuse pulmonary involvement or intrapulmonary metastasis. Both of these scenarios have the corresponding stages of pT4 (if thought to be ipsilateral) or M1a (if thought to also involve the contralateral lobe).” Patient declined any further treatment and transitioned to hospice before expiring less than 1 month after wedge biopsies. It is unclear if Rule M6 would apply to these two specimens with different subtypes since this scenario is not specifically addressed in the M rule definitions. |
Abstract two separate primaries when separate/non-contiguous tumors are two or more different subtypes/variants in Column 3 of Table 3 using Rule M6 in the Solid Tumor Rules (September 2021 Update). They represent two subtypes/variants of the same NOS histology. When coding histology, tissue from pathology takes precedence over imaging, including when stated as differential diagnoses based on the CT scan, as noted by the pathologist in this example. |
2022 |
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20210072 | Hormone Therapy--Breast: How are hormone therapy (HT) and other related data items coded when a patient had a previous breast primary and is still on HT when diagnosed with a new breast primary? See Discussion. |
In this scenario, we record that HT began for the second primary on the date of diagnosis, and the Systemic/Surgery Sequence ends up usually being coded 4 because the HT continues even if the specific agent may be changed. This does not seem to meet the definition of neoadjuvant therapy for the second primary so we approach the staging and grade coding as just clinical/pathological? For example, if the tumor size at surgery is a little larger than estimated on imaging, we would use the pathologic size for our staging. The tumor size and grade of the second primary are not being changed by the ongoing HT. Do we have the right approach? |
For this example: 1. Code HT as treatment on the date of diagnosis for the second primary. 2. Code Systemic/Surgery Sequence as 4. 3. Do not code neoadjuvant data items as neoadjuvant started/completed. The HT given would not qualify for neoadjuvant therapy since the intent of the HT was not neoadjuvant. The HT would affect the second primary, but it is still not neoadjuvant. 4. Code clinical and pathological tumor size accordingly, based on the imaging and the pathological findings. 5. Code Extent of Disease data items based on the pathological findings since pathological findings take priority over clinical and this is not neoadjuvant therapy. |
2021 |
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20210073 | Solid Tumor Rules (2018/2021)/Multiple Primaries--Corpus Uteri: How many primaries should be reported when a hysterectomy identifies primary endometrial carcinosarcoma (8980/3) and the endometrium has a background of endometrioid intraepithelial neoplasia (EIN) (8380/2)? A tumor size is provided for the carcinosarcoma, but not the background EIN. |
Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN). |
Abstract this case as a single primary and code histology as carcinosarcoma (8980/3). The carcinosarcoma is intermixed with the EIN making this a single primary coded to the invasive histology. EIN is a precursor of endometrial carcinoma in the WHO Classification of Female Genital Tumors, 5th edition. Carcinosarcoma of the uterus is described in the literature as an aggressive variant of endometrial carcinoma characterized by unusual histologic features including discrete malignant epithelial and mesenchymal components (carcinoma and sarcoma). |
2021 |
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20210071 | Solid Tumor Rules (2018/2021)/Histology--Breast: How is histology coded for a diagnosis of invasive mammary neuroendocrine tumor (NET), grade 2/3? See Discussion. |
Table 3 (Breast Equivalent Terms and Definitions) lists “Neuroendocrine tumor, well-differentiated” of the breast as histology 8246/3. There is no entry for a grade 2 neuroendocrine tumor of the breast in Table 3. The pathologist did not indicate the neuroendocrine tumor was poorly differentiated (or it would otherwise be a small cell carcinoma). The pathologist noted “By current WHO criteria, this tumor is characteristic of a mammary neuroendocrine tumor, grade 2. These invasive tumors have similar prognostic and predictive features of invasive ductal carcinoma of the same grade and stage.” |
Assign code 8249/3, neuroendocrine tumor, grade 2 based on the pathologist statement of mammary neuroendocrine tumor grade 2. According to WHO Classification of Tumors of the Breast, 5th edition, neuroendocrine tumor (NET) is an invasive tumor characterized by low/intermediate grade. If the histology term is not listed in the Solid Tumor rules, the instructions state to also check ICD-O and updates. Per ICD-O, NET, grade 2 is coded 8249/3. Breast Table 3 will be updated for 2023. |
2021 |
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20210053 | Reportability/Heme & Lymphoid Neoplasms: Is ALK positive (ALK+) histiocytosis involving the bone marrow and kidney reportable? See Discussion. |
2021 Bone marrow biopsy showed erythroid hyperplasia, increased histiocytes with hemophagocytosis and Factor XIIIa positive histocytic cells. Moderate cytoplasmic staining for ALK 1, consistent with bone marrow involvement of ALK-positive histiocytosis. A subsequent kidney lesion biopsy was also found to have ALK-positive histiocytosis. The patient was then treated with clofarabine. Patient is 3 years old. 07/2020-Chart indicates patient presented in June with fevers and refusing to walk with pancytopenia, bone marrow biopsy showed no leukemia buthistiocytes. Impression: ALK positive histiocytosis involving BM and kidney. 10/2020 Bone marrow final diagnosis states right and left bone marrow aspirates and biopsies: No morphologic or immunohistochemical evidence of involvement by the patient's previously diagnosed ALK+ histiocytosis (see Comments) - Multiple histiocytic collections with prominent hemosiderin; favor reactive - background normocellular bone marrow with maturing trilineage hematopoiesis. The patient's prior bone marrow samples are reviewed (9/2020 and 7/2020). Similar to the September bone marrow sample, the current marrow shows numerous histiocyte collections with abundant associated hemosiderin deposition. These histiocytes have a stellate/dendritic appearance and lack the atypical features noted in the patient's marrow at diagnosis, favoring a reactive process. This impression is further supported by the lack of immunoreactivity for either Factor XIIIa or ALK1 among these cells. There is no convincing morphologic or immunohistochemical evidence of marrow involvement by the patient's previously diagnosed ALK+ histiocytosis within the sampled material. Of note, the marrow otherwise appears normocellular for the patient's age, indicative of ongoing marrow recovery post therapy. It is not clear whether this would be equivalent to Langerhans cell histiocytosis, disseminated (9751/3) as there is not a statement of Langerhans cell or whether this is just histiocytosis, NOS and not reportable. |
Do not report this case of histiocytosis. Based on the information provided, this case is not reportable. |
2021 |
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20210041 | Reportability/Behavior--Paraganglia: Is a 2021+ diagnosis of paraganglioma reportable if the grading of adrenal pheochromocytoma and paraganglioma (GAPP) score falls outside the stated requirements for malignancy? See Discussion. |
Patient was diagnosed with a retroperitoneal paraganglioma on April 2021 mass resection. Final diagnosis included the comment: Based on the modified grading of adrenal pheochromocytoma and paraganglioma (GAPP), the GAPP score is 1. Scores greater than or equal to 3 are malignant. We are aware that paraganglioma is classified as malignant for cases diagnosed in 2021+, however it is unclear how the pathologist's interpretation of the GAPP score may affect the behavior of this case. |
Report retroperitoneal paraganglioma based on ICD-O-3.2 histology/behavior that lists paraganglioma, NOS as 8680/3 for cases diagnosed 2021 and forward. While GAPP is a predictor of metastatic potential, it does not factor into behavior, thus reportability. |
2021 |
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20210026 | Multiple primaries--Heme & Lymphoid Neoplasms--Lymphoma: Is a case initially submitted as C772 with histology coded 9591/3 (lymphoma, NOS) with a second case submitted as C162 with histology coded 9699/3 (extranodal marginal zone lymphoma of mucosal-associated lymphoid tissue (MALT lymphoma) a single primary or multiple primaries? See Discussion. |
The following cases were submitted to the central registry as separate primaries. First case submitted as C772 with histology coded 9591/3 (Lymphoma, NOS). Second case submitted as C162 with histology coded 9699/3 (MALT Lymphoma). Sequence 01 - 5/2016, Excisional biopsy pancreatic tail lymph node: suspicious for malignant B-cell lymphoma. No treatment recommended or administered. Sequence 02 - 2/2019, Stomach biopsy: MALT Lymphoma. Unknown if treatment was recommended or administered. Biopsy was only at this facility. Using the Hematopoietic and Lymphoid Neoplasm Multiple Primaries/Histology rules, Rule M7 makes this a single primary. Note 4 instructs to change the histology of the initial abstract to the more specific histology (9699/3). If this is done, they would be multiple primaries per the exception within Rule M2. Should the histology on sequence 01 be changed to the MALT lymphoma and the cases would be multiple primaries or is this a single primary? |
Abstract two primaries and assign Primary 1: C772, 9699/3 Primary 2: C162, 9699/3 Per Rule M7, you would change the first case to histology 9699/3 based on Note 4 under Rule M7, Note 4: Change the histology code on the original abstract to the more specific histology when the original diagnosis is in your registry database. Use previous editions of ICD-O (i.e., ICD-O-1, ICD-O-2) or the Hematopoietic Database to assign the code applicable to the year of diagnosis for the more specific histology. Per Rule M2 this would be the same primary based on both being the same histology; however, there is an exception for MALT lymphomas (9699/3), which states: Abstract multiple primaries when a nodal MALT (C770-779, 9699/3) occurs before or after an extranodal MALT (all other sites, 9699/3). |
2021 |
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20210007 | First Course Treatment/Reason for No Surgery of Primary Site: How should we be coding Reason For No Surgery of Primary Site for cases where surgery was planned but ultimately cancelled due to progression? See Discussion. |
There is a discrepancy in the SEER and STORE manual definition of code 2 for Reason for No Surgery of Primary Site. STORE includes progression of tumor prior to planned surgery as part of the definition for code 2, but the SEER Manual does not. The progression statement is included in the SEER Manual (2018 and 2021) for Reason for No Radiation, but not for Reason for No Surgery. |
Assign code 2 for cases where surgery was planned but ultimately cancelled due to progression in the data item Reason For No Surgery of Primary Site. Code 2 description contains examples and is not exhaustive of reasons for no surgery. We will add the example for consistency in the next version of the SEER manual. |
2021 |
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20210063 | Solid Tumor Rules (2018/2021)/Multiple primaries--Ovary, Fallopian Tubes: How many primaries should be reported and for which primary site(s) when pathologist identifies bilateral ovarian high-grade serous carcinoma with involvement of the left fallopian tube (also showing serous tubal intraepithelial carcinoma (STIC))? See Discussion. |
Patient is diagnosed July 2021 with high-grade serous carcinoma on ascites cytology. Tumor debulking total abdominal hysterectomy/bilateral salpingo-oophorectomy in August shows high-grade serous carcinoma involving the right ovary (capsule intact, right fallopian tube is negative), left ovary (capsule ruptured), and fallopian tube. Pathologist has chosen tumor site to be bilateral ovaries in the staging summary, with the left fallopian tube listed as “other tissue/organ involvement” along with uterus, peritoneum, and omentum. Additional findings in staging summary includes serous tubal intraepithelial carcinoma (STIC). Our interpretation of SINQ 20210025 is that any case with both ovarian and tubal involvement would be coded as a fallopian tube primary if STIC is present, even when the pathologist is clearly calling the case ovarian. If this is correct, then the previous SINQ 20120093 may need to be updated with a date restriction reference since it would be in disagreement with this instruction. If our interpretation is incorrect, then the STIC would be an additional primary per MP/H Rule M11. |
Bilateral ovarian tumors are a single primary per M7. Abstract the STIC as a second primary. SINQ 20210025 is intented to address situations with confliciting information about the primary site. The answers remain unchanged in 2012009 and 20210025. |
2021 |
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20210027 | Reportability--Heme and Lympoid Neoplasms--Polycythemia vera: Is secondary polycythemia vera reportable? See Discussion. |
A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea. According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia. |
Abstract as a new primary for polycythemia vera, 9950/3. JAK2 is commonly used to assess suspected polycythemia vera and in this case, the mutation is positive for V617F. Based on the JAK2 results, this looks like a true polycythemia vera and not a secondary polycythemia. |
2021 |
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