| Report | Question ID | Question | Discussion | Answer | Year |
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20200007 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is simultaneously diagnosed with systemic mastocytosis and chronic myelomonocytic leukemia (CMML-0) on a single bone marrow biopsy? See Discussion. |
The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that, However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2. |
Abstract a single primary when the diagnosis is systemic mastocytosis with an associated clonal hematogoical non-mast cell lineage disease (SM-AHNMD) (9741/3). However, if the patient has a previous history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia, abstract the SM-AHNMD as a second primary as stated in the Heme DB. SINQ 20130172 represents a single primary as there is no mention of a prior history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia. |
2020 |
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20200064 | Primary site--Heme &Lymphoid Neoplasms: What is the primary site of two extraosseous plasmacytomas, with positive pathology of right orbit and left lung. The patient's bone marrow biopsy, flow, and peripheral blood smear were negative. Is this coded as 9732/3, multiple myeloma (Primary Site and Histology Rule PH2) with the primary site as C809 (PH27)? Or is the primary site C421 since code 9732 says primary site is always C421, though bone marrow came back as negative? |
Assign the primary site to C421 since that is the only allowable primary site for plasma cell myeloma, even though the bone marrow was negative. According to the revised criteria from the WHO Blue Book for Hematopoietic and Lymphoid Neoplasms (2017), the presence of multiple plasmacytomas is plasma cell myeloma (9732/3). |
2020 | |
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20200042 | Solid Tumor Rules (2018)/Histology--Brain and CNS: How is the histology coded when the diagnosis comment for a posterior fossa tumor resection states: Taken together, these findings are indicative of medulloblastoma with extensive nodularity? See Discussion. |
Example: Posterior fossa tumor resection final diagnosis was medulloblastoma, WHO Grade IV. The diagnosis comment notes the current tumor resection reveals large irregular reticulin-free nodules with streams of neoplastic cells in a fibrillary background in association with narrow reticulin-rich internodular strands of poorly differentiated neoplastic cells. Taken together, these findings are indicative of medulloblastoma with extensive nodularity. The diagnosis comment provided only one histology. Per the 2018 Solid Tumor Manual, Malignant CNS, Priority Order for Using Documentation to Identify Histology instructions, an addendum or comment has priority over the final diagnosis. Although indicative is not listed on any ambiguous terminology list, is this an ambiguous diagnosis that must be ignored? Or does the diagnosis comment in this case provide a single, specific diagnosis of medulloblastoma with extensive nodularity? |
Code as medulloblastoma, nodular (9471/3) based on the findings from both the comment and final diagnosis. |
2020 |
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20200061 | Solid Tumor Rules (2018)/Histology--Bladder: A patient has high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation followed by carcinosarcoma. Is this one or two primaries? See Discussion. |
12-19-19 Transurethral resection of bladder tumor pathology revealed high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine features; Pathology Overread: High-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation. Carcinoma invades muscularis propria pT2. Histology 8130 01/20/20 to 07/01/20, completed 6 cycles of gemcitabine/cisplatin. 07/30/20 Robotic radical cystoprostatectomy with bilateral pelvic lymph node dissection, open ileal conduit pathology revealed carcinosarcoma, invading perivesical fat, no lymphovascular invasion, negative margins. ypT3bN0M0 disease; Pathology Overread: Carcinosarcoma arising in association with high-grade papillary urothelial carcinoma. Histology 8980/3 or is there another histology that should be used? |
The carcinosarcoma is a separate tumor, abstract a new primary per M13. Code this primary to 8980/3. Based on the information provided, the patient was first diagnosed with papillary urothelial carcinoma and received neo-adjuvant treatment for that specific histologic type. Subsequent resection identified carcinosarcoma arising within the papillary neoplasm. Carcinosarcoma is rare in bladder primaries and is not included in Table 2; however, it is a subtype/variant of sarcoma. |
2020 |
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20200016 | Reportability/Histology--Vulva: Is Extramammary Paget neoplasm (intraepithelial glandular neoplasm) reportable? See Discussion. |
Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification. Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable. |
According to our subject matter expert, vulvar extramammary Paget neoplasm (intraepithelial glandular neoplasm) represents an in situ malignancy and should be reported. He states "The traditional terminology should be 'extramammary Paget disease' to describe an in situ adenocarcinoma arising from extramammary glands in vulvar mucosa. I am not so sure about "extramammary Paget NEOPLASM", which may include all three Pagetoid processes: the traditional Paget disease, the Pagetoid spreading of an anal adenocarcinoma and a Pagetoid spreading of an urothelial carcinoma from the urethra. Regardless, all these entities are considered at least in situ carcinomas." We recommend that you review clinical records and imaging for the clinical scenarios mentioned above. |
2020 |
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20200043 | Histology/Behavior--Bladder: Is the behavior of a bladder tumor with low-grade papillary urothelial carcinoma /2 or /3? See Discussion. |
Transurethral resection: Microscopic Diagnosis: Bladder, transurethral resection: Low-grade papillary urothelial carcinoma Gross Description: Received in formalin labeled with the patient's name and bladder tumor is a 3.0 x 2.0 1.0 cm aggregate of friable tan tissue biopsies. The specimen is submitted in toto, cassettes This is all the information there is on this path report. Extent of Disease (EOD) instructions state inferred description of noninvasive: No statement of invasion (microscopic description present) SEER 2018 Appendix C Bladder Coding Guidelines state code behavior 3 if the only surgery performed is a transurethral resection of the bladder (TURB) documenting that depth of invasion cannot be measured because there is no muscle in the specimen OR the pathology report does not mention whether the submucosa is free of tumor or has been invaded by tumor. |
For cases diagnosed 2021 or later Code the behavior as in situ (/2) when the diagnosis is low grade urothelial carcinoma and there is no information regarding invasion. The SEER Manual Appendix C Bladder Coding Guidelines revision reflects this change. No changes have been made to EOD at this time. The guidelines have been updated as follows. Low grade urothelial carcinoma with no other information: Code to /2. High grade urothelial carcinoma with no other information: Code to /3. For cases diagnosed prior to 2021 Code the behavior as malignant (/3) for a bladder tumor with low-grade papillary urothelial carcinoma. |
2020 |
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20200049 | Summary Stage 2018/EOD 2018--Lymphoma Orbital Adnexa: What is the correct Summary Stage 2018 (SS2018) for the site/histology Orbit, NOS (C696), 9699/3? In SEER*RSA, Extent of Disease (EOD) Primary Tumor references code 7 (Distant), whereas SS2018 assigns code 2 (Regional)? See Discussion. |
We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses |
To clear this edit of the derived Summary Stage (based on EOD) and the manually assigned Summary Stage (based on Summary Stage 2018), assign the manually assigned Summary Stage to 7. For this particular case, EOD Primary Tumor 700 (which is correct based on the information received) derives Distant; however, for Summary Stage 2018, this description is under Code 2 for Regional by direct extension. This is an error. For 2022, Summary Stage for Lymphoma Ocular Adnexa description under Code 2 (Regional by direct extension) will be moved to Distant. No changes will be done to EOD. |
2020 |
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20200067 | Summary Stage 2018/Extension--Colon: What is the Summary Stage for adenocarcinoma of cecum where the tumor extends into the proximal portion of attached vermiform appendix? See Discussion. |
2020 Diagnosis: Patient had a right hemicolectomy showing adenocarcinoma of cecum, tumor extends into proximal portion of attached vermiform appendix. Tumor invades through muscularis propria into pericolorectal tissues (NOS). Regional lymph nodes: 06/39. Primary Tumor EOD: Where does the appendix involvement come into coding or will this be based on the pericolorectal tissue (NOS) invasion? What is my Summary Stage? I know it is at least 3 due to regional ln involvement, but the appendix involvement is making me question 3 vs 4. |
Assign code 4, Regional by BOTH direct extension AND regional lymph node(s) involved. In this case, the Regional component for Summary Stage 2018 is based on Note 6, under Colon and Rectum where Regional is defined as: -Mesentery -Peritonealized pericolic/perirectal tissues invaded [Ascending Colon/Descending Colon/Hepatic Flexure/Splenic Flexure/Upper third of rectum: anterior and lateral surfaces; Cecum; Sigmoid Colon; Transverse Colon; Rectosigmoid; Rectum: middle third anterior surface] -Pericolic/Perirectal fat |
2020 |
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20200036 | Reportability--Skin: Is malignant proliferative trichilemmal tumor (PTT) reportable, and if so, do we apply the matrix rule and code it to 8103/3? A literature search reveals these do exist, but are extremely rare. |
Malignant PTT (8103/3) of the skin is not reportable. A neoplasm originating in the skin with histology coded to 8103 is not reportable. See 1.b.i. on page 7 in the 2018 SEER manual for a complete list, https://seer.cancer.gov/manuals/2018/SPCSM_2018_maindoc.pdf |
2020 | |
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20200059 | Reportability--Kidney: Is Bosniak 4 cystic lesion of right kidney reportable, and would the first CT date be the date of diagnosis? See Discussion. |
CT a/p read by radiologist shows: "Bosniak 4 cystic lesion of right kidney." Follow-up MRI a month later reads "right kidney cystic lesion with enhancing mural nodule concerning for cystic renal cell carcinoma (RCC)." Urologist consult used the same wording of "Bosniak 4 cystic lesion" and "concerning for renal cell carcinoma." Treatment discussed but due to patient health status recommended repeat imaging. Repeat CT few months later reads: "cystic right renal lesion with enhancing nodule similar to most recent prior and suspicious for cystic RCC." Though "suspicious for cystic RCC" per latest imaging is reportable, Bosniak 4 is "clearly malignancy, ~100% malignant" by definition, so is the case actually reportable with the first CT a/p date as date of diagnosis? |
2023 Bosniak 4 is defined as "clearly malignant cystic mass." The case is reportable as of the first date it is diagnosed as a Bosniak 4 lesion unless further workup (especially biopsy or resection) disproves the CT findings. https://radiopaedia.org/articles/bosniak-classification-system-of-renal-cystic-masses?lang=us |
2020 |
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