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20210015 | Solid Tumor Rules (2007/2021)/Multiple Primaries--Anus: Have the disease free interval criteria been met for the following case scenario. A patient was diagnosed with anal intraepithelial neoplasia (AIN) III in 7/2018 that was treated with local tumor destruction, followed by Pap smears and biopsies that prove AIN I or AIN II through 2020, before being diagnosed with a reportable AIN II or AIN III in 2021. See Discussion. |
Since AIN I is not reportable and AIN II is not reportable until 2021, we are not sure if we can say the patient was disease free because there was no intervening reportable tumor (AIN III), or was never disease free because there was evidence of related disease (lower grade dysplasia). |
The 2021 AIN III is not a new primary. According to our GI pathology expert, findings of AIN I and/or AIN II following a diagnosis of AIN III indicates the patient was never NED and indicates persistent disease. . |
2021 |
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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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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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20210070 | Histology/Reportability--Digestive System: Is “neuroendocrine neoplasm” reportable? See Discussion. |
We are confused by SINQs 20180097, 20150001, and 20140051. The latter two indicate that “well-differentiated neuroendocrine neoplasms” of the duodenum and appendix are reportable because they’re synonymous with neuroendocrine tumor (NET). Yet 20180097 states “primary hepatic neuroendocrine neoplasm” is NOT reportable unless there is documentation that it’s being used as a synonym for Primary Hepatic Neuroendocrine Tumor (PHNET). In addition, we see in the 2021 ICDO-3.2 update that only “poorly differentiated neuroendocrine neoplasm” is listed with behavior code /3 and noted to be reportable for 2021+ on the companion annotated histology list. Does reportability of neuroendocrine neoplasms depend on primary site, differentiation terminology within the histology name, or something else? Our casefinding staff is hoping for a general reportability guideline to follow when they come across “neuroendocrine neoplasms” NOS. For example, we have a 2020 pathology report for a core biopsy of a soft tissue pelvic mass with final diagnosis of low grade neuroendocrine neoplasm; there is no further clarification as to whether it is felt to be primary or metastatic, and we have no other associated records for this patient in our central registry. |
Reportability of neuroendocrine neoplasms depends on primary site, terminology, and differentiation. "Neuroendocrine neoplasm" is an umbrella term for a variety of neuroendocrine tumors and carcinomas. Neuroendocrine neoplasm, not otherwise specified (NEN, NOS) is not reportable as in your example unless it is being used as a synonym for neuroendocrine tumor (NET), as with digestive system tumors. According to WHO Classification of Digestive System Tumors, 5th ed., NENs of the appendix and liver are epithelial neoplasms with neuroendocrine differentiation, including well-differentiated tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The guidance in SINQ 20180097, 20150001, and 20140051 is still valid. |
2021 |
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20210034 | Reportability/Histology--Endometrium: Is endometrial hyperplasia with atypia equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable? |
Endometrial hyperplasia with atypia is equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable for cases diagnosed 2021 and later. Our expert pathologist consultant confirmed this for us. |
2021 | |
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20210006 | Behavior/Summary Stage 2018--Colon: What is the correct behavior and Summary Stage for a case of intramucosal adenocarcinoma arising in tubular adenoma? AJCC states this is Tis, though SEER Summary Stagie states this is Localized (code 1). The histology is 8140/2 (adenocarcinoma in situ), but the SEER Summary Stage is Locallized. |
Intramucosal carcinoma of the colon is assigned behavior code of /3. Intramucosal is not the same as in situ in terms of behavior. Behavior and staging are separate concepts, although there is some overlap. Use the instructions for coding behavior to code this field. Do not use stage to determine behavior in this case. For purposes of Summary Stage, intramucosal carcinoma is a localized lesion; however, for purposes of AJCC staging, assign Tis for the stage. |
2021 | |
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20210021 | EOD 2018/Regional Nodes--Breast: Should Extent of Disease (EOD) Regional Nodes be coded as 150 (Clinical assessment only; Positive needle core biopsy/fine needle aspirate [FNA]) when the patient has a biopsy-proven, clinically apparent, movable ipsilateral axillary lymph node, but no evidence of involvement at surgery after neoadjuvant therapy? See Discussion. |
The Breast EOD Regional Nodes notes contain new clarification regarding the clinical assessment vs. pathological assessment codes, but the new Note 2 does not specifically indicate an exception for neoadjuvant therapy. However, if the pre-treatment lymph node core biopsy proved cN1 disease, and the post-treatment resection proved ypN0 disease, should the clinical assessment code (code 150) have priority over any pathological assessment code (including 200) since the involved lymph node was only clinically positive and not pathologically positive? Should an exception be added to Note 2 to address cases where neoadjuvant therapy is given, but the clinical assessment is greater than the pathological assessment? |
The clinical assessment code takes priority over the pathological assessment code in this case because the clinical assessment was worse than the pathologic assessment. Although there was a pathological assessment, the clinical assessment is greater. According to the general coding guidelines for neoadjuvant therapy, code the worst information, which in this case is the clinical assessment. The 2018 EOD General Instructions for EOD Regional Nodes, instruction #4, addresses neoadjuvant therapy as follows. Neoadjuvant (preoperative) therapy: If the patient receives neoadjuvant (preoperative) systemic therapy (chemotherapy, immunotherapy) or radiation therapy, code the clinical information if that is the most extensive lymph node involvement documented. A new note is being included for the 2022 updates. Exception: If patient has neoadjuvant therapy, and the clinical assessment is greater than the pathological assessment, the clinical assessment code takes priority. |
2021 |
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20210004 | Solid Tumor Rules (2018)/Histology--Colon: What is the histology for a 2020 pathology report final diagnosis showing invasive adenocarcinoma, poorly differentiated with signet ring cell features and signet-ring cell carcinoma in the synoptic report? See Discussion. |
Since the synoptic report and final diagnosis are equal in priority, and the Solid Tumor Rules tell us to code the more specific histology, would this be coded to signet ring cell adenocarcinoma, 8490/3, even though the pathologist used features in the final diagnosis? There is no histology adenocarcinoma with signet ring cell features on the CAP Protocol, so the pathologist may check off the next closest histology " signet ring cell carcinoma " which would not be truly representative of the actual histology. Final Diagnosis: Proximal colon, segmental resection: Invasive adenocarcinoma, poorly differentiated, with signet ring cell features. Synoptic Report A: Colon and Rectum - Resection Specimen Procedure: Right hemicolectomy, Tumor Site: Right (ascending) colon, Histologic Type: Signet-ring cell carcinoma, Histologic Grade: G3: Poorly differentiated. |
Code histology to 8490/3 per H6. The December 2020 Solid Tumor Update includes addition of the following instructions to the "Priority Order for Using Documentation to Code Histology" section. Which document to use when there is conflicting information between the final diagnosis, synoptic report, or CAP protocol: When there are discrepancies between the final diagnosis and synoptic report, use the document that provides the more specific histology. This will likely be found in the synoptic report. The CAP Protocol should be used only when a final diagnosis or synoptic report are not available. Definitions for CAP Protocol, final diagnosis, and synoptic report can be found in the Definitions section. |
2021 |
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20210017 | Update to current manual/Mets at diagnosis fields--Lymphoma: Are distant metastases possible for a lymphoma with a primary site of lymph nodes? The instructions in the SEER manual tell us to assign code 8 in each of the Mets at Dx fields for a lymphoma originating in lymph nodes. |
This is a correction to the SEER manual. Lymphomas originating in lymph nodes (C77) could have distant metastases to any site except lymph nodes. The following corrections to the manual apply now and will appear in the next version of the manual. Remove C770-C779 from the instruction for assigning code 8 on the following pages. Page 135 Mets at Dx--Bone Page 137 Mets at Dx--Brain Page 139 Mets at Dx--Liver Page 141 Mets at Dx--Lung Page 145 Mets at Dx--Other Example Biopsy of axillary lymph node: Diffuse Large B-Cell lymphoma. Lymph nodes involved above and below the diaphragm, multiple nodules seen in lung, lesions in liver. Bone marrow biopsy positive for DLBLC. Per Hematopoietic manual, primary site would be C778 for multiple lymph node regions involved. Mets at Dx--Bone-0 Mets at Dx--Brain-0 Mets at Dx--Liver-1 Mets at Dx--Lung-1 Mets at Dx--Distant Lymph Nodes-8 Mets at Dx--Other-1 |
2021 | |
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20210058 | Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion. |
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored. 4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH. 5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia. 6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma. |
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3). The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern. In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis. We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns. |
2021 |
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