Report | Question ID | Question | Discussion | Answer | Year |
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20210031 | Reportability--Brain and CNS: Are lipomas of the spinal column reportable as a benign tumor of the central nervous system (CNS)? This is seen occassionally at our pediatric facility. |
Spinal cord tumors (including lipomas) are reportable when they arise in the spinal dura or nerve root. The tumor must be of the spinal cord itself or within the spinal cord dura. Spinal cord tumors are reportable when they arise in the intradural space. A reportable intradural tumor can be either intramedullary or extramedullary. Extramedullary intradural spinal tumors are reportable. A spinal tumor originating in the extradural space is not reportable. If it is outside the dura, it is not reportable because it would be outside the CNS. They are not reportable when they arise in the peripheral nerves. |
2021 | |
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20210001 | SEER*RSA/Required data items--Melanoma: The site-specific data item, Ulceration, states it is required by "All" in SEER*RSA but in the NAACCR Data Dictionary table it states is it required by SEER, Commission on Cancer (CoC), and Canadian Cancer Registry (CCCR), not the National Program of Cancer Registries (NPCR). Does the definition of "All" in SEER*RSA not include NPCR? Also, please explain the difference between Required by: "All" and "Required by CCCR/Canada, COC, NPCR, SEER" (all listed out). |
Use the NAACCR Data Dictionary Required Status Table or refer to standard setter requirements. Do not use SEER*RSA to determine which data items are required to be collected or transmitted. Though "All" in SEER*RSA generally refers to the standard setters including CoC, NPCR, CCCR, and SEER, some items in SEER*RSA need updating; this is planned for 2022. |
2021 | |
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20210028 | Histology/Biliary tract--Ampulla of Vater: What is the histology code for Intra ampullary papillary-tubular neoplasm in association with microinvasion? See discussion. |
Patient was diagnosed on 01/2020, and primary site on the pathology report is Ampulla of Vater (C241). Synoptic Report states histology as: Intra ampullary papillary-tubular neoplasm in association with microinvasion. I have reviewed the ICD-O-3 coding table and found histology Intraductal tubulopapillary neoplasm (C25_) code 8503/2. Based on the Matrix principle (Rule F on the ICD-O-3), I will change the behavior to 3 and code as 8503/3. If I look in ICDO-3, Tubulopapillary adenocarcinoma is coded 8263/3. |
Assign code 8163/3. Based on the microinvasion, the correct term for this neoplasm is pancreatobiliary-type carcinoma. Unfortunately, WHO did not provide all synonyms or related terms for some of the new ICD-O codes. Pathologists may continue using non-preferred terminology as well. |
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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20210040 | Solid Tumor Rules (2018, 2021)/Histology--Breast: How is histology coded for a diagnosis ofmixed mucinous carcinoma? See Discussion. |
Patient was diagnosed with invasive ductal carcinoma with mucinous features in February 2021, left breast biopsies at 2:00 (3 cm from nipple) and 3:00 (8 cm from nipple) positions. Intraductal component was absent in those specimens. Subsequent left breast total mastectomy in March 2021, provided a final diagnosis of multifocal mixed mucinous carcinoma, grade 1, 27 and 8 mm and ductal carcinoma in situ (DCIS), low to intermediate grade, with focal mucinous features. The Staging Summary lists Histologic Type as mixed mucinous carcinomafor both foci of invasive carcinoma. DCIS is also noted as present negative for extensive intraductal component. There does not appear to be a clear instruction or code for this histology. As a central registry, we are unable to follow-up with the pathologist regarding this diagnosis. Just to be clear, there are 2 foci of invasive mixed mucinous carcinoma in this case measuring 27 mm and 8 mm. The DCIS component measured 56 mm. |
If the DCIS is separate from the mucinous, apply the breast M rules and abstract TWO primaries per M14. Since all we know of the “mixed mucinous” is there is mucinous present, code 8480/3. |
2021 |
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20210049 | Histology/Heme & Lymphoid Neoplasms--Leukemia: Is this the correct histology for a case of acute myeloid leukemia (AML) with recurrent genetic abnormalities? If the only information was AML with recurrent genetic abnormalities,"what code would you use: AML, NOS (9861/3) or AML with recurrent genetic abnormalities (9896/3)? See Discussion. |
12/3/2020 Pathology: AML: Blasts 40% of nucleated cells. CD45 positive, CD34 negative, CD 117+, CD13 positive, CD33 positive in 59.6% and HLA-DR was dim and myeloperoxidase was dim. Cytogenetics normal karyotype. The next generation sequencing detected IDH 2p.(R172K)c515>A. Because this was AML NOS, we consulted with the physician. The physician stated the patient had AML with recurrent genetic abnormalities"and the basis for the diagnosis was the IDH-2 mutation identified on Next Generation Sequencing. We assigned 9896/3, based on the physician's interpretation of the pathology. This histology is being questioned. |
We found that the term AML with recurrent genetic abnormalities, NOS"was incorrectly included as an alternate name with code 9896/3. We followed back with our expert hematopathologist and he stated that this should have been coded to 9861/3 (AML, NOS), for AML with recurrent genetic abnormalities, NOS. This alternate name has been added to 9861/3. (Note: The same alternate name has been removed from 9896/3). IDH-2 is not listed as a genetic abnormality for any of the histologies listed in the database. It could be that this is a new genetic marker for one of the AML with recurrent genetic abnormalities that we are not aware of. Without further clarification on which histology the IDH-2 would indicate, you would have to default to 9861/3. There are several histologies that are grouped as AML with recurrent genetic abnormalities."All of these have specific genetics listed as part of the ICD-O-3 histology name. 9865: Acute myeloid leukemia with t(6;9)(p23;q34.1) DEK-NUP214 9866: Acute promyelocytic leukemia with PML-RARA 9869: Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM 9871: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 9877: Acute myeloid leukemia with mutated NPM1 (2021+) 9878: Acute myeloid leukemia with biallelic mutation of CEBPA (2021+) 9879: Acute myeloid leukemia with mutated RUNX1 (2021+) 9896: Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1 9897: Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3 9911: Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 9912: Acute myeloid leukemia with BCR-ABL1 (2021)+ Of note, for the above histologies, since these are diagnosed solely based on genetics, diagnostic confirmation will always be 3. This instruction will be added to the Hematopoietic database for the 2022 update. |
2021 |
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20210043 | Reportability--Fallopian Tube: Is a diagnosis of serous tubal intraepithelial neoplasm (neoplasia) (STIN) equivalent to serous tubal intraepithelial carcinoma (STIC)? Does the designation of high or low grade have any effect on potential reportability? See Discussion. |
Patient has left salpingo-oophorectomy showing fallopian tube with focal high grade serous intraepithelial neoplasm. In reviewing some journal articles, the term STIN is being used to describe both STIC and serous tubal intraepithelial lesion (STIL). We will likely continue to see this term used, so it would be nice to have some clarity. |
Serous tubal intraepithelial neoplasm (neoplasia) (STIN) is not equivalent to serous tubal intraepithelial carcinoma (STIC). Report STIN only when stated to be high grade. STIC is reportable. Do not report STIL. According to our expert pathologist consultant, STIL and STIN are broad descriptive terms that reflect proliferation of epithelial cells with varying degrees of atypia, with the most developed, STIC, reflecting convincing neoplastic change. |
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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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 |