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Report Produced: 07/30/2024 18:18 PM

Report Question ID Question Discussion Answer Year
20190007

Reportability--Skin: Is atypical intradermal smooth muscle neoplasm (AISMN) of the skin reportable? The comment on the path report states: Atypical intradermal smooth muscle neoplasm (AISMN) was previously termed "cutaneous leiomyosarcoma."

Atypical intradermal smooth muscle neoplasm (AISMN), previously termed "cutaneous leiomyosarcoma," is not reportable. It is classified as a borderline, /1, neoplasm.

 2019
20190063

Solid Tumor Rules (2018)/Histology--Sarcoma: How is histology coded for a CIC gene rearrangement sarcoma? See Discussion.

According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded?

Code as undifferentiated round cell sarcoma (8803/3).

The CIC rearrangement exists as a distinct molecular and clinical subset of small round cell tumors, and though similar, is felt to be a distinct entity from Ewing sarcoma. According to WHO Classification of Soft Tissues and Bone, 4th Edition, CID-DUX4 is a recurrent gene fusion associated with pediatric round cell undifferentiated soft tissue sarcoma (USTS). Although the genes involved in the fusion are different from those in Ewing sarcoma, the CIC-DUX4 protein has been shown to upregulate genes of the ETS family of genes thus providing a molecular link between Ewing sarcoma and round cell USTS. In contrast, there are strong arguments to suggest that Ewing-like sarcomas represent a separate and distinct entity.

 2019
20190057

Reportability/Histology--Penis: Are and (PeIN) equivalent to PeIN3 and thus reportable? See Discussion.

Appendix E1 of the 2018 SEER manual references a similar diagnosis as being reportable for vulva and vagina only. However, the WHO Classification of Tumors of the Urinary System and Male Genital Organs (4th ed) does include high grade penile intraepithelial neoplasia as a synonym for 8077/2.

Penile intraepithelial neoplasia, grade III (PeIN III) and squamous cell carcinoma in situ of the penis are reportable. If possible, query the physicians as to whether "high grade penile intraepithelial lesion" or are synonymous with one of the reportable terms. If no further information can be obtained, report the case as C609 8077/2, and use text fields to document the details.

 2019
20190095

Reportability/Histology--Thymus: Is a thymoma a malignancy if described as having separate tumor nodules within peri-thymic adipose tissue? See Discussion.

Patient had a thymectomy including pericardial fat for a mediastinal mass found incidentally during lung screening. Final diagnosis is WHO B3 thymoma. Staging Summary lists transcapsular invasion: "Present, as separate tumor nodules within peri-thymic adipose tissue." Tumor extension is stated to be "Confined to thymus, including peri-thymic adipose tissue." The pathologist staged this resection as pT1a pNX with no mention of mets. Clinically, there are no noted metastatic sites and no further treatment is planned.

Report this case as a malignant thymoma. Our expert pathologist consultant reviewed this case and in his opinion, the "separate tumor nodules within peri-thymic adipose tissue" fit registry reporting criteria for separate tumor nodules making this a malignant thymoma.

 2019
20190033

Update to current manual/Neoadjuvant therapy/Pathologic tumor size--Breast: When a patient with invasive breast cancer is started on neoadjuvant therapy and at surgery is found to have only residual in-situ disease, do we record the size of the in-situ tumor for Pathologic Tumor Size? See Discussion.

I understand that we are to record the Clinical Tumor Size in Tumor Size Summary because of the neoadjuvant therapy, but the SEER manual does not address what to record in the Pathologic Tumor Size after neoadjuvant therapy. Would we record 999 or the size of the in-situ tumor in the Pathologic Tumor Size field? Will there ever be a new data item added or changes to this current data item? By recording the Patholigic Tumor Size this way, there currently will not be any way to compare tumor size clinically versus after neoadjuvant therapy and assessing the response.

Note: this is an update to the 2018 SEER manual.

Assign 999 in Pathologic Tumor Size when neoadjuvant therapy has been administered. We can explore the possibility of another data item in the future.

 2019
20190102

Solid Tumor Rules/Histology--Head & Neck: What is the histology code of an external ear lesion when the dermatopathology report is the only available information (follow-up with the physician or pathologist is not possible) and the final diagnosis is malignant spindle cell neoplasm, most consistent with atypical fibroxanthoma? See Discussion.

There are two histologies provided in the final diagnosis, malignant spindle cell neoplasm (8004/3) and atypical fibroxanthoma (8830/3). There is a definitive diagnosis of the non-specific histology, but the more specific histology is only described using ambiguous terminology.

The external ear (C442) is included in the Head and Neck schema for diagnosis year 2018 and later. The Head and Neck Histology Rules indicate ambiguous terminology cannot be used to code a more specific histology. So ignoring the atypical fibroxanthoma, because it is modified by ambiguous terminology, we are left with a non-reportable site and histology combination (C442, 8004/3).

Diagnoses of malignant atypical fibroxanthomas are regularly diagnosed using the syntax above in our area. Follow-up with the physician or pathologist is generally not possible as these cases are received from dermatopathology clinics only. The pathology report is the only information that will be received. If the reportable diagnosis of malignant atypical fibroxanthoma is ignored per the current Solid Tumor Rules, incidence cases will be lost.

By definition, atypical fibroxanthoma (AFX) is a diagnosis of exclusion. Markers of specific differentiation must be negative. As written in your example, neither histology is reportable for skin. If possible, clarify the behavior of the AFX (8830/1) with the pathologist to determine reportability of the case.

 2019
20190106

Tumor Size--Esophagus: Can information from the endoscopy procedure that implies a size of 3 cm for Tumor Size--Clinical be used for Esophagus? See Discussion.

1-28-2018 CT Scan: 2.4 cm mass

2-15-2018 Endoscopy: Mass was present 22 to 25 cm. Biopsies were taken with cold forceps for histology; biopsy positive.

For the case you describe, we would record the clinical tumor size stated on the CT report.

The priority order for clinical tumor size is as follows.

1. Biopsy or operative (surgical exploration) report

2. Imaging

3. Physical exam

We do not recommend coding tumor size based on an inferred tumor size from a description such as "Mass was present 22 to 25 cm." Look for an actual measurement of the mass, or a stated tumor size. Use text fields to record details.

 2019
20180103

Histology/Grade--Small intestine: For a 2017 diagnosis, is the grade/differentiation field coded 1 or 9 when the diagnosis is well-differentiated neuroendocrine tumor (NET) (carcinoid)? It seems as though the term well-differentiated defines type of neuroendocrine tumor so they can diagnosis the carcinoid. See Discussion.

5/15/17 Duodenal bulb, biopsy: Fragments of duodenal mucosa with well differentiated neuroendocrine tumor (carcinoid), extending to the edge of specimen and peptic duodenitis in the submitted tissue. No significant intraepithelial lymphocytosis.

Assign grade code 1 for well-differentiated NET (8240/3). Well-differentiated is synonymous with NET, grade 1, according to WHO Classification of Tumors of the Digestive System.

 2018
20180010

Diagnostic confirmation--Heme & Lymphoid Neoplasms: Is Diagnostic Confirmation coded as 5 (positive laboratory test/ marker study) or code 8 (clinical diagnosis only) for a case that has a positive JAK2 mutation, and based on the results of the JAK2, the physician diagnosed the patient with polycythemia vera? There were no blood smears or bone marrow biopsies done.

Assign diagnostic confirmation code 5 for a positive laboratory test/marker study. A note was added to the Hematopoietic manual to state that code 5 now includes cases with no histological confirmation but there is positive immunophenotyping or genetic studies.

 2018
20180031

First Course of Treatment/Other Therapy: Where do you code Optune TTF therapy? What needs to be included in the text portion to document this treatment?

Code OPTUNE in the Other Treatment field. See NovaTTF in SEER*Rx (http://seer.cancer.gov/seertools/seerrx/). NovaTTF is the pre-FDA approval name for OPTUNE.

If OPTUNE was administered for recurrence, be sure NOT to record it in the first course of treatment fields. Check with CoC if you have questions about coding treatment for recurrence.

 2018