Report | Question ID | Question | Discussion | Answer | Year |
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20210078 | Solid Tumor Rules (2018/2021)/Multiple Primaries--Skin Cancer: How many primaries are assigned for sebaceous carcinomas using the Solid Tumor/Multiple Primaries/Histology Rules? Does this scenario represent eight separate primaries? See Discussion. |
Details 4/15/2018: Right abdominal wall mass excision: infiltrating sebaceous carcinoma. Noted to have a history of Muir-Torre/Lynch syndrome. 1/21/2019: Two left upper back mass excisions and two lower back (laterality not specified) mass excisions: infiltrating sebaceous carcinomas 8/7/2019: Excision of multiple sebaceous carcinomas from the right posterior back, left posterior thigh, left anterior abdominal wall, left anterior thigh, right scrotum, right lower abdominal fold, all positive for sebaceous carcinoma on pathology report 9/30/2020: Right gluteal mass, left gluteal mass, back (NOS) excisions: sebaceous carcinomas. 10/14/2020: Right back excision: sebaceous carcinoma. Op note: History of Lynch syndrome with multiple sebaceous carcinomas, recurrent back mass, site of prior mass resection. 10/18/2021: Right thigh excision: sebaceous carcinoma Proposed primaries using MP/H Other Sites Rules #1: 4/15/2018: C445-1 #2: 1/21/2019: C445-2, separate from #1 per M8, same as 1/21/19 C445-9 per M18 #3: 8/7/2019: C445-1, separate from #1 per M10, separate from #2 per M8 #4: 8/7/2019: C447-2, separate from #1 & #3 per M8, separate from #2 per M12 #5: 8/7/2019: C632, separate from #1 per M10, separate from #2-#4 per M11 #6: 9/30/2020: C445-2, separate from #1 & #3 per M8, separate from #2, #4 & #5 per M10 #7: 9/30/2020: C445-1, separate from #2, #4 & #6 per M8, separate from #1, #3 & #5 per M10; I do not think the back, NOS (C445-9) is a new primary per M18. #8: 10/18/2021: C447-1, separate from #2, #4 & #6 per M8, separate from #1, #3, #5 & #7 per M10 |
Assign the number of primaries following the Other Sites Solid Tumor Rules. Based on sites, laterality and or timing there are 8 primaries. This is similar to SINQ 20061112 that advised to follow the Multiple Primaries/Histology rules for sebaceous carcinoma. According to the WHO Classification of Skin Tumors, 5th edition, there is a 30-40% risk of local tumor recurrence, and 20-25% risk of distant metastasis. In only one instance did a physician refer this as a recurrence in the available notes. |
2021 |
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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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20210005 | Reportability/Histology--Ovary: Is a 2020 ovary case reportable with the positive malignant findings in adnexal cystic fluid and peritoneal washing? See Discussion. |
11/24/20 Adnexal mass, cyst fluid: Positive for malignant cells. Clusters of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. Groups of inhibin-negative, CK7-positive epithelial cells consistent with serous borderline tumor cells. Peritoneal washing: Positive for malignant cells. Small groups of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. A. Left ovarian mass: Adult granulosa cell tumor (AGCT) of ovary (see note). pTNM Stage: pT1c3 pNX - Serous borderline tumor (SBT) of ovary (see note). pTNM Stage: pT1a pNX. Fallopian tube; unremarkable. B. Right ovary: - Serous cystadenofibroma of ovary. Fallopian tube; unremarkable. C. Left pelvic wall nodule: Fibro-calcified nodule, consistent with necrotic appendix epiploica. D. Uterus (hysterectomy): Uterine leiomyomas. Endosalpingiosis of uterine serosa and paracervical tissue. Atrophic endometrium. Note: The left ovarian mass is involved by a combined adult granulosa cell tumor and a serous borderline tumor. The AGCT mainly involves the thick-walled cystic area while the SBT the thin-walled cyst/s. The 2 neoplastic elements do, however, demonstrate areas of intimate and close intermingling. From the current literature, it appears that, based on FOXL2 mutation, the AGCT component of combined AGCT and ovarian epithelial tumors is either a true neoplastic processes or an AGCT- like proliferation morphologically indistinguishable from AGCT. To further evaluate the nature of the AGCT component, a FOXL2 analysis is in progress and an addendum will follow. |
For cases diagnosed prior to 2021, report adult granulosa cell tumor of ovary only when stated to be malignant or when metastases are indicated, as by the positive peritoneal washings for this 2020 case. Beginning in 2021, report all cases of adult granulosa cell tumor of ovary based on ICD-O-3.2. |
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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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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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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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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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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20210029 | Multiple primaries--Heme and Lymphoid Neoplasms: Is a patient with peripheral blood initially showing chronic myelogenous leukemia (CML), lymph node biopsy showing granulocytic sarcoma (9930/3), and bone marrow biopsy showing acute myeloid leukemia (AML) one or two primaries? See Discussion. |
1. 12/11/2020 Peripheral blood revealing what was thought to be chronic myelogenous leukemia BCR/ABL1 positive (9875/3). Patient was started on Hydrea while waiting for further tests on 12/12/2020. 2. 12/14/2020 Lymph node biopsy showed granulocytic sarcoma (9930/3), but flow cytometry states it is similar to that seen in the patient's peripheral blood and is consistent with nodal involvement by myeloblasts. 3. 12/15/2020 Bone marrow biopsy reads acute myeloid leukemia (9861/3), likely arising from BCR/ABL1 positive chronic myeloid leukemia. There is a note on this pathology from medical oncologist that says: This will dramatically change the course of his treatment, likely with a TKI. 4. 12/17/2020 Sprycel started. Patient was weaned off Hydrea. According to Rule M3, abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. It lists 9930/3 when simultaneously (or after) with 9861/3. Technically, it was two days before, but I feel like I should and could count that as simultaneously because of Note 1 that says: These sarcomas are solid manifestations of the associated leukemia. For example, when acute myeloid leukemia and myeloid sarcoma are diagnosed simultaneously, the myeloid sarcoma is the result of myeloid cells migrating from the bone marrow or blood into tissue. It is part of the disease process for the acute leukemia. Also, the providers never mention granulocytic sarcoma Based on that, I think that #2 & #3 above are the same primary, which would be acute myeloid leukemia (9861/3). Per the hematopoietic database, 9875/3 transforms to 9861/3. Therefore, Rule M8 is confusing with the "only one" biopsy. Does this rule apply because the 9875/3 was from peripheral blood only? But peripheral blood is coded in Diagnostic Confirmation as histology. Rule M9 reads: The two diagnoses are likely the result of an ongoing diagnostic work-up. The later diagnosis is usually based on all of the test results and correlated with any clinical information. Because that is truly what I think is happening here though that rule states there is no available documentation. If you do not have any documentation, how would you know you are dealing with a chronic and an acute diagnosis? M10 does not apply. According to Rule M11, abstract as multiple primaries when both a chronic and an acute neoplasm are diagnosed simultaneously or within 21 days and there is documentation of two biopsies. The chronic myelogenous leukemia only had peripheral blood and not a bone marrow, lymph node or tissue, but that is counted as positive histology in diagnostic confirmation, but I don't know if that is kept as a separate field/thought. I would not code a peripheral blood smear as with a surgical code or a surgical diagnostic and staging procedure code, so maybe that is what I should be thinking about and therefore would probably say Rule M8 and one primary. |
This is one primary based on Rule M3. Abstract as a single primary site for the granulocytic sarcoma and AML since they are both evaluating the blood/bone marrow, which are counted as one site. To count them twice would result in over counting primaries. For Rule M9: This would not apply to your situation since you do have information on both the CML and the AML. We had to write in this rule for cases where you do not always have the information available. In terms of the peripheral blood versus actually biopsy: In this case, do not count the peripheral blood as a separate site. Rule M8 does fit your case, coding this as the AML and having this as one primary. |
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 |