| Report | Question ID | Question | Discussion | Answer | Year |
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20220036 | Solid Tumors Rules/Histology--Head and Neck: How is histology coded for head and neck primaries when a tumor is diagnosed as an invasive squamous cell carcinoma with multiple subtypes? See Discussion. |
Example Case 1: 2022 mobile tongue tumor biopsy shows squamous cell carcinoma, basaloid non-keratinizing type. Example Case 2: 2022 base of tongue mass biopsy shows squamous cell carcinoma, basaloid non-keratinizing type, p16 positive. Table 5, Note 2 (Head and Neck Equivalent Terms and Definitions) instructs us to code non-keratinizing squamous cell carcinoma which is p16 positive to 8085 (Squamous cell carcinoma HPV-positive), ignoring the non-keratinizing subtype. Does p16 or HPV positivity also take priority over multiple subtypes (basaloid non-keratinizing type)? |
Assign 8083/3, basaloid squamous cell carcinoma (BSCC), in both examples. It is more important to capture the variant than to code 8085 or 8086. WHO Classification of Head and Neck Tumors, 5th ed., states that BSCC is a distinctive form of SCC, characterized by prominent basaloid morphology, squamous differentiation, and aggressive behavior. Some primary sites capture p16 status as a Site Specific Data Item; you may record the p16 results when that is the case. |
2022 |
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20210016 | Solid Tumor Rules (2018, 2021)/Histology--Kidney: What is the correct histology code for a kidney primary described as clear cell papillary renal cell carcinoma"? Should we use H2 and code 8312/3 or H3 and code 8323/3? |
Assign 8323/3, clear cell papillary renal cell carcinoma using the 2018 Kidney Solid Tumor Rules, Rule H1, as this is a single histology, a variant of renal cell carcinoma NOS. |
2021 | |
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20210024 | Primary Site--Vulva: What is the primary site of patient with an excision of a left vulvar cystic mass showing focal mammary-type ductal carcinoma in situ (DCIS) on 11/06/2020? See Discussion. |
Final Pathologic Diagnosis: Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment) Size of DCIS: 0.7 CM. Margins: Negative. Comment Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised. 12/01/2020 post op visit with surgeon: Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion. PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management. |
Code the primary site to vulva. Use text fields to record the details. According to the WHO classification, several types of primary vulvar mammary-like carcinoma have been reported. It is rare and is thought to arise from specialized anogenital mammary-like glands within the vulva. It does not arise from ectopic breast tissue and is does not represent metastatic breast carcinoma. |
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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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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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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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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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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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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20210076 | Reportability/Brain and CNS: Is a 2021 case of ecchordosis physaliphora (lesion within the prepontine cistern) on brain MRI reportable? |
Ecchordosis physaliphora is not reportable. |
2021 |
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