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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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20200069 | Solid Tumor Rules (2018)/Histology--Breast: What histology code is used for an in situ encapsulated papillary carcinoma with an invasive carcinoma, NST? See Discussion. |
In Table 3 (Specific Histologies, NOS/ NST, and Subtypes/Variants), the entry for papillary carcinoma, NOS includes a change in column 3 of the 2018 Breast Solid Tumor Rules that conflicts with the H Rules. It is not accounted for in the change log. No explanation is offered as to why this change was made. This is a major change because encapsulated papillary carcinoma is frequently associated with carcinoma NST, and we have not been collecting these as such. Encapsulated papillary carcinoma (8504) in column 3 now includes an indented entry, with invasive carcinoma, NST/invasive duct carcinoma 8504/3. However, most encapsulated papillary carcinomas are in situ or there is no definitive statement of invasive encapsulated papillary carcinoma, so when in situ and invasive tumors are present, we are instructed to code the invasive histology (which would be the invasive carcinoma (NST), 8500/3). How are registrars to arrive at the correct histology without a new H rule or a clarification regarding this update being documented in the change log? Does the same change/addition apply to solid papillary carcinoma? These are often also associated with carcinoma, NST. Again, without an explanation regarding the change mentioned above, it is difficult to understand why the change was made. This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
In situ encapsulated papillary arising in or with invasive carcinoma, NST (a single tumor) is a single invasive histology. Use rule H14 and code the histology per Table 3. A note as been added to the 2023 breast rule H8 instructing when there is a single tumor with histology of in situ encapsulated papillary with invasive carcinoma or solid papillary carcinoma with invasove, continue through the rules. See H14 and code the appropriate histology per Table 3. Histologic types are becoming more complex and often have both in situ and invasive components but have a single code to identify them. |
2020 |
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20210011 | Primary site: Is C720 the correct primary site for a diagnosis of a paraspinal neuroblastoma on autopsy in a nine month old with Noonan syndrome? See Discussion. |
Autopsy/Pathology Report (2020) excerpts External Examination Nervous System: There is an 8.5 cm mass located in the right thoracic paraspinal area. Final Anatomic Diagnosis Clinical History: Paraspinal mass suspicious for neuroblastic tumor (detected by imaging studies) Nervous System: Right thoracic paraspinal neuroblastoma, poorly differentiated |
Assign primary site code C473 for this case based on the information provided (peripheral nerves and autonomic nervous system of thorax). From our expert pathologist consultant: The origin of neuroblastomas is generally in the adrenal medulla or one of the sympathetic ganglia on either side of the vertebral column (although they have been reported in many other locations given the migration of the neural crest cells embryologically). |
2021 |
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20210035 | Update to current manual/Lymphovacular invasion--Thyroid: Are psammoma bodies only recorded as vascular invasion in papillary thyroid cancer cases? See Discussion. |
For example, total thyroidectomy specimen shows right lobe papillary thyroid carcinoma, 4.2 cm, unencapsulated, with numerous psammoma bodies in non-tumoral thyroid parenchyma, without angioinvasion; left lobe with papillary thyroid carcinoma, 0.6 cm, encapsulated, with capsular invasion, with intralymphatic psammoma bodies in non-tumoral thyroid parenchyma, without angioinvasion. The synoptic summary documents vascular invasion present (psammoma bodies only). |
If you are collecting lymphovascular invasion (LVI) for thyroid cases, record "vascular invasion present (psammoma bodies only)" as vascular invasion (code 1, Lymphovascular Invasion Present/Identified) in the LVI data item. Use a text field to specify that this is vascular invasion by psammoma bodies. |
2021 |
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20210009 | Solid Tumor Rules (2018, 2021)/Histology--Melanoma: In what situation will Rule H4 be used to code the histology to regressing melanoma? See Discussion. |
Rule H4 states: Code 8723/3 (malignant melanoma, regressing) when the diagnosis is regressing melanoma. However, if the diagnosis was strictly regressing melanoma or malignant melanoma, regressing, the first rule that applies is Rule H1 because regressing melanoma is a single, specific histologic type and Rule H1 states: Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H4. Should the H Rules be reordered? Or should an example of when one would use Rule H4 be added to clarify when to use this rule? |
Coding regressing melanoma has been an issue as registrars may not realize it is a reportable histology. Hence, H4 was written to reinforce correct histology. A note will be added to H1 instructing registrars to continue thru rules when the diagnosis is regressing melanoma. |
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 |
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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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20210004 | Solid Tumor Rules (2018)/Histology--Colon: What is the histology for a 2020 pathology report final diagnosis showing invasive adenocarcinoma, poorly differentiated with signet ring cell features and signet-ring cell carcinoma in the synoptic report? See Discussion. |
Since the synoptic report and final diagnosis are equal in priority, and the Solid Tumor Rules tell us to code the more specific histology, would this be coded to signet ring cell adenocarcinoma, 8490/3, even though the pathologist used features in the final diagnosis? There is no histology adenocarcinoma with signet ring cell features on the CAP Protocol, so the pathologist may check off the next closest histology " signet ring cell carcinoma " which would not be truly representative of the actual histology. Final Diagnosis: Proximal colon, segmental resection: Invasive adenocarcinoma, poorly differentiated, with signet ring cell features. Synoptic Report A: Colon and Rectum - Resection Specimen Procedure: Right hemicolectomy, Tumor Site: Right (ascending) colon, Histologic Type: Signet-ring cell carcinoma, Histologic Grade: G3: Poorly differentiated. |
Code histology to 8490/3 per H6. The December 2020 Solid Tumor Update includes addition of the following instructions to the "Priority Order for Using Documentation to Code Histology" section. Which document to use when there is conflicting information between the final diagnosis, synoptic report, or CAP protocol: When there are discrepancies between the final diagnosis and synoptic report, use the document that provides the more specific histology. This will likely be found in the synoptic report. The CAP Protocol should be used only when a final diagnosis or synoptic report are not available. Definitions for CAP Protocol, final diagnosis, and synoptic report can be found in the Definitions section. |
2021 |
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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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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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