Report | Question ID | Question | Discussion | Answer | Year |
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20210056 | 2018 Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be reported when a left breast simple mastectomy identifies focal Paget disease of the nipple and 12 axillary nodes positive for metastatic lobular carcinoma (no primary lobular breast tumor identified)? |
Abstract two primaries, one lobular carcinoma (8520/3) and another one Paget disease of the breast (8540/3) using the 2018 Breast Solid Tumor Rules, Rule M9: Abstract multiple primaries when the diagnosis is Paget disease with underlying tumor which is NOT duct. Example: Paget disease of the nipple with underlying lobular carcinoma are multiple primaries. Additionally, Table 2, Histology Combination Codes, Note 2 states: Lobular carcinoma and Paget are separate primaries (see Lobular carcinoma and any histology in Table 3 with exception of duct carcinoma/carcinoma NST/DCIS (and subtypes/variants) 8500 and Paget disease, in situ and invasive). While not identified in the pathology of the mastectomy, the lobular carcinoma is likely underlying as it was identified in the axillary lymph nodes. The 2021 SEER Manual states: If the only pathologic specimen is from a metastatic site, code the appropriate histology code and the malignant behavior code (/3). The primary site and its metastatic site(s) have the same histology. |
2021 | |
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20210055 | Tumor Size--Pathologic/EOD 2018: How is Tumor Size--Pathologic coded when Extent of Disease (EOD) Primary Tumor is 800 (No evidence of primary tumor) and there has been no surgery to the primary site? See Discussion. |
The SEER Manual states to assign Tumor Size--Pathological code 000 when EOD Primary Tumor is coded to 800 (No evidence of primary tumor) for any schema. However, the definition of Tumor Size--Pathologic states that it records the size of a solid primary tumor that has been resected. If the primary site has not been resected (does not meet the pathologic staging criteria), then it seems that Tumor Size Pathologic should be 999 when EOD Primary Tumor is coded as 800. |
Assign code 999 for Tumor Size--Pathologic when there is no surgery of the primary site. Code 999 includes "No excisional biopsy or tumor resection done." |
2021 |
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20210054 | Tumor Size--Clinical/EOD 2018--Prostate: How is Tumor Size--Clinical coded when there is an incidental finding of prostate cancer on prostatectomy for another reason? See Discussion. |
SEER*RSA states EOD Primary Tumor should be coded to 800 for an incidental finding of prostate cancer on prostatectomy for other reasons. The SEER Manual states to assign code 000 for Tumor Size--Clinical when EOD Primary Tumor is coded to 800; however, the definition for Tumor Size--Clinical indicates clinical classification is composed only of diagnostic workup prior to treatment. If there is no clinical workup for an incidental finding of prostate cancer, code 000 does not seem appropriate (does not meet criteria for clinical classification). Code 999 seems more appropriate for incidental findings during surgery for other reasons. The SEER Manual does not provide this exception in the current instruction. |
Assign code 000 for Tumor Size--Clinical when EOD Primary Tumor is coded 800 (No evidence of primary tumor). Code 000 indicates no tumor was found since there was no clinical workup to identify this incidentally found cancer. This is a special instruction for cases coded 800 in EOD Primary Tumor. Text fields can be used to record details. |
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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20210051 | Primary site/Biliary tract--Ampulla of Vater: What is the correct primary site code for intra-ampullary and periampullary adenocarcinoma, C241 (8144/3) or C249? See Discussion. |
Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation. Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present. Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type. There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber. |
Assign C241. Ampulla (C241) includes both periampullary and intra-ampullary. |
2021 |
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20210050 | EOD 2018/Extension--Testis: How is Extent of Disease (EOD) Primary Tumor coded if it appears limited to testis on scrotal ultrasound and is treated with neoadjuvant chemotherapy prior to the orchiectomy when there is no residual tumor (staged as ypT0 disease) and in cases where there is residual tumor? See Discussion. |
Unless there is a biopsy that proves in situ tumor (EOD code 000, Tis) or extratesticular invasion into the scrotum, penis, or further contiguous extension (EOD code 700, T4), EOD Primary Tumor must be coded based on the PATHOLOGICAL assessment (orchiectomy). There are no other CLINICAL codes because the AJCC indicates imaging is not used for local T-categorization, and the EOD derives the AJCC TNM staging. If the case can not be coded to either EOD Primary Tumor codes 000 or 700 clinically, the only clinical code that seems to apply is 999 (Unknown). We are seeing more cases treated with neoadjuvant chemotherapy prior to orchiectomy, especially in patients with distant metastatic disease. The EOD Manual indicates that clinical evidence takes priority over pathological evidencewhen neoadjuvant treatment is given, unless the extent of disease following neoadjuvant treatment is greater than pre-treatment clinical findings. If the clinical and pathological information are the same, code the extension based on the clinical information. Do these general rules also apply to testis even though we cannot code CLINICAL findings for these tumors? If so, will EOD Primary Tumor be coded to 999 (Unknown) for any testis primary that is not in situ or invasive into the scrotum, etc., that is treated with neoadjuvant therapy? Or should the post-neoadjuvant PATHOLOGICAL assessment be coded for these tumors because the CLINICAL assessment would otherwise be unknown? How is the EOD Primary Tumor coded for the following two cases? 1. Left testicular mixed germ cell tumor, biopsy-proven metastasis to a supraclavicular lymph node. The left testis contained a small mass on scrotal ultrasound. The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved no residual primary tumor (ypT0). Is EOD Primary Tumor 999 because it is clinically unknown (even though it was clinically limited) or 800 (No evidence of primary tumor) because there was no pathological evidence of tumor following neoadjuvant treatment? 2. Right testicular mixed germ cell tumor with biopsy-proven inguinal lymph node metastasis. There was a palpable mass in right testis on physical exam (not described as fixed or involving scrotum). The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved a residual 2 cm tumor limited to the testis without lymphovascular invasion (LVI). Is EOD Primary Tumor 999 because it is clinicallyunknown or 200 (PATHOLOGICAL assessment only - Limited to testis WITHOUT LVI)? |
Assign code 999 to EOD Primary Tumor for testis when neoadjuvant therapy is given and clinical assignment is unknown and the extent of the primary tumor is not fully assessed due to post neoadjuvant treatment effect as with the two case scenarios. Both clinical examination and histologic (pathologic) confirmation are required by AJCC for clinical assessment and was not met in these scenarios. While EOD Primary Tumor is based on pathologic assessment, the EOD general instructions are to code the clinical information if that is the farthest extension when the patient received neoadjuvant systemic therapy unless the post-neoadjuvant surgery shows more extensive disease. As there is neoadjuvant treatment effect and there is no clinical assessment, the primary tumor cannot be fully assessed. |
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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20210048 | Reportability--Anal Canal: Is a 2021 diagnosis of moderate squamous dysplasia (AIN II) of the anal canal reportable? See Discussion. |
We are aware that squamous intraepithelial neoplasia, grade II (e.g., AIN II), 8077/2 is reportable for 2021. However, because this is also called rather than high grade squamous dysplasia (8077/2), we are unsure about reportability. There is no known histology and behavior code for moderate squamous dysplasia, the classifications available are only low grade (8077/0) or high grade (8077/2). |
If possible, clarify with the pathologist/physician what is meant by "moderate squamous dysplasia (AIN II)." If no further information can be obtained, report this case based on the diagnosis of "AIN II." Squamous intraepithelial neoplasia, grade II is listed in ICD-O-3.2 as 8077/2 making it reportable for cases diagnosed in 2021. AIN is a type of squamous intraepithelial neoplasia. |
2021 |
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20210047 | Summary Stage 2018/EOD 2018--Colon: Does the 2018 SEER Summary Staging Manual, Digestive System Sites, Distinguishing In Situ and Localized Tumors for the Digestive System, #1. b., Exception, include in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.)? This seems to be in conflict with Extent of Disease (EOD) 2018. See Discussion. |
We are preparing to send our hospitals a reminder that the behavior changes from 2 to 3 at the bottom of the basement membrane, and the T category changes from Tis to T1 at the bottom of the mucosa for colon and rectum carcinomas. We are confused by the wording of the Exception. Distinguishing In Situ and Localized Tumors for the Digestive System 1.b. If the tumor has penetrated the basement membrane to invade the lamina propria, in which case it is localized and assigned Summary Stage 1 (localized) and for invasion of the lamina propria Exception: Code 0 (behavior code 2) includes cancer cells confined within the glandular basement membrane (intraepithelial); includes in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.) The text following (intraepithelial) is unclear. The question is: Does the text include in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.) mean the following: Code 0 (behavior code 2) includes in situ plus intramucosal carcinoma. In situ plus intramucosal carcinoma is involvement of the lamina propria, which may involve (but not penetrate through) the muscularis mucosae. Penetration through the muscularis mucosa is behavior 3. If that is what the text above means, then it seems that the 2018 SEER Summary Stage Manual is saying colorectal tumors reported as: adenocarcinoma in situ, at least intramucosal adenocarcinoma in situ, high grade dysplasia/intramucosal adenocarcinoma in situ, focally intramucosal at the margin are to be coded behavior 2 and SEER Summary stage In situ (0) like the intraepithelial carcinoma tumors. However, it conflicts with the EOD Data for Colon and Rectum, Note 2, and SINQ 20210006. The text for both EOD Data for Colon and Rectum and SINQ 20210006 is clear. According to them, the above bulleted adenocarcarcinoma examples are coded SEER Summary Stage localized (1) and behavior 3. SINQ 20210006 states that: For purposes of Summary Stage, intramucosal carcinoma is a localized lesion So, intramucosal carcinoma is coded SEER Summary Stage 1 (localized) and (behavior code 3). According to the text for EOD Primary Tumor, Colon and Rectum, Note 2 below, intramucosal, NOS involvement is invasive. Note 2: Code 050 (behavior code 3) includes the following: Intramucosal, NOS Lamina propria Mucosa, NOS Confined to, but not through the muscularis mucosa Thank you for your help clarifying the 2018 SEER Summary Manual Exception text above. |
For purposes of Summary Stage, intramucosal, NOS is a localized lesion. Intramucosal carcinoma is coded SEER Summary Stage 1 (localized) and (behavior code 3). The involvement of the following are assigned localized in Summary Stage and assigned a behavior code of 3. Intramucosal, NOS Lamina propria Mucosa, NOS Confined to, but not through the muscularis mucosa The Exception you cite may need to be reworded. We will review for the next version of the Summary Stage manual. |
2021 |
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20210046 | Reportability--Skin: Is dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation synonymous with dermatofibrosarcoma protuberans, fibrosarcomatous, and therefore reportable for diagnosis year 2021 and forward? See Discussion. |
Patient has a 2021 skin excision showing an atypical spindle cell neoplasm, most consistent with dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation. Per the ICD-O-3.2 Coding Table, DFSP, NOS has a behavior code of /1, and DFSP, fibrosarcomatous has a behavior code of /3. There is no code listed for DFSP with fibrosarcomatous transformation. Transformation is not included as a term that can/cannot be used for the Other Sites Schema, but this type of DFSP is often described as DFSP with fibrosarcomatous transformation. How do we code DFSP when transformation is used to describe fibrosarcomatous? |
Report DFSP with fibrosarcomatous transformation as it is synonymous with fibrosarcomatous DFSP (8832/3). According to the WHO Classification of Skin Tumors, 4th edition, fibrosarcomatous DFSP is a variant of DFSP and that fibrosarcomatous transformation is seen in approximately 10% of DFSP cases. It is characterized by an often abrupt transition of DFSP. |
2021 |