Reportability/Histology--Soft Tissue: Is a diagnosis of Myofibroblastoma with sarcomatous transformation a reportable malignancy? See Discussion.
Patient was diagnosed in September 2022 via excision of a 12 cm pelvic mass with final diagnosis of Myofibroblastoma with sarcomatous transformation.
Diagnosis comment states, “Most of the tumor is composed of conventional features of myofibroblastoma. However, a focal area demonstrates increased cellularity, fascicular growth and increased mitotic activity (up to 11 per 10 hpf), consistent with sarcomatous transformation (morphologically low to intermediate grade).”
Is this sarcomatous transformation describing a malignant transformation from an otherwise benign histology? If so, how should histology be coded in this case?
Do not report the case. The histology is 8825/0 based on the example provided and not reportable. Myofibroblastoma with sarcomatous transformation is a rare, benign condition, sometimes referred to as sarcomatous features. A malignant tumor would be referred to as a myofibroblastic sarcoma.
Reportability/Histology--Small intestine: Is a neuroendocrine microtumor of the duodenum a reportable tumor? See Discussion.
This comment was added to the pathology report by the pathologist: A focus of neuroendocrine microtumor measured 350 micrometers, qualifying as a neuroendocrine microtumor. Focus was immunohistochemically positive for chromogranin and synaptophysin and negative for gastrin. The Ki-67/CD45 immunostain showed <1% positivity in microtumor.
Neuroendocrine microtumor of the duodenum is reportable as 8240/3. "Microtumor" pertains to the size/amount of NET and not to a histologic type.
Reportability/Histology--Skin: Is dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous overgrowth, DFSP with fibrosarcomatous component Grade 2, or DFSP with focal myxoid features (2022) reportable for 2021-2022 diagnoses?
Yes. DFSP with fibrosarcomatous overgrowth and DFSP with fibrosarcomatous component Grade 2 are synonymous with fibrosarcomatous DFSP (8832/3). Our expert pathologist also advises that DFSP with focal myxoid features is the same as DFSP, myxoid (8832/3).
Reportability/Histology--Skin: Is a malignant spindle cell neoplasm consistent with atypical fibroxanthoma reportable for cases diagnosed 1/1/2023 and later, after thorough immunohistochemical work-up? See Discussion.
Appendix E1 in both the 2023 and 2024 SEER Program Coding and Staging Manual (SPCSM) lists these malignant spindle cell neoplasms, consistent with atypical fibroxanthoma, as reportable when other tumors have been ruled out with immunohistochemistry. This contradicts both SINQ 20190102 and the Solid Tumor Rules (STRs) general instructions indicating ambiguous terminology (e.g., “consistent with”) cannot be used to code the more specific histology when there is a NOS (malignant spindle cell neoplasm, 8004/3) and a more specific (malignant atypical fibroxanthoma, 8830/3) histology.
These tumors are typically diagnosed and treated in dermatology offices, so further chart review or confirmation by a physician is not possible for central registries.
As non-melanoma skin primaries are included in the Other Sites schema, and this schema was updated for cases diagnosed 2023 and later, which instruction applies to 2023+ diagnoses? Should these continue to be collected per Appendix E1 despite the conflict with the STR Manual and SINQ? If these are reportable, should the SINQ and STR Manual be updated to reflect this? Or should these be non-reportable per the STR Manual and SINQ?
Report malignant spindle cell neoplasms consistent with atypical fibroxanthoma as directed by Appendix E.1 of the 2023 and 2024 versions of the SEER Manual using 8830/3 (fibroxanthoma, malignant).
We will update the answer in SINQ 20190102. While the Other Sites Solid Tumor Rules address coding an NOS and specific histology sub-type/variant, this situation is not specifically addressed. We will also review the rules.
Reportability/Histology--Skin: Is 'skin, left temporal scalp, low grade adnexal carcinoma, probable sweat gland origin' reportable as 8400/3, skin of temple?
Assign 8390/3 for adnexal carcinoma of skin. 8390/3 is reportable, including 8390/3 of skin.
Reportability/Histology--Pituitary Gland: How are Rathke cleft cyst and Rathke pouch tumor distinguished and are they both reportable?
Rathke cleft cyst is not reportable. Cysts are not neoplastic. However, Rathke pouch tumor (C751, 9350/1) is a reportable neoplasm for cases diagnosed 2004 and later. The Rathke pouch is coded to the pituitary gland. Benign and borderline pituitary tumors have been reportable since 2004.
Reportability/Histology--Penis: Is a diagnosis of undifferentiated penile intraepithelial neoplasia (PeIN) reportable for cases diagnosed in any year? See Discussion.
Example: An October 2017 glans penis biopsy final diagnosis was reported as: Undifferentiated (Warty-Basaloid) penile intraepithelial neoplasia.
In January 2018, an additional penile glans biopsy final diagnosis was reported as: At least squamous cell carcinoma (SCC) in situ (HGPIN). Foreskin circumcision on the same pathology report shows SCC in situ.
It is unclear whether the term undifferentiated is synonymous with high-grade for the purposes of determining penile intraepithelial neoplasia (PIN/PEIN) reportability and diagnosis date.
Report undifferentiated penile intraepithelial neoplasia (PeIN) (8077/2). WHO Classification of Tumors of the Urinary System and Male Genital Organs, 4th edition, lists basaloid (undifferentiated) penile intraepithelial neoplasia and warty (Bowenoid) penile intraepithelial neoplasia as a variants of PeIN.
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.
Reportability/Histology--Pancreas: Is well-differentiated neuroendocrine tumor (M8240/3) as stated on a pathology report reportable or can the clinical information be used as an adjunct to the path report, which further states the specific type of neuroendocrine tumor is an Insulinoma, therefore, NOT reportable (M8151/0)? See discussion.
The diagnosis date is 2/24/14. The pathology report of the pancreas shows: Well-differentiated neuroendocrine tumor (NET), low grade (WHO G1 of 3). Addendum: Ki-67 confirms low grade of pancreatic endocrine tumor (less than 2% Ki-67/MIB-1 index). Chromogranin confirms the endocrine nature of the tumor. The Pre and Post Op Diagnosis is pancreatic neuroendocrine tumor consistent with insulinoma. AJCC Stage as noted on path report: pT1, pNX, pM.
The physician states: The patient has a well-documented insulinoma. Biochemistries confirmed the disease and it is localized in the tail of the pancreas.
The issue with NETs is that pathology report reflects what is seen or what is quantified under the microscope; often, there is a specimen without the accompanying medical history and clinical signs. Many of these NETs are specified on the basis of the hormone, as usually measured in the blood, that is overproduced, something not seen microscopically. All of the islet cell tumors are NETs. The insulinoma in the example above is a well-differentiated NET that is causing insulin to be over-produced. Thus, the diagnoses are not discordant; insulinoma is a more specific NET.
When the pathology diagnosis is a neuroendocrine tumor (/3) and the clinical diagnosis is an insulinoma (/0), report the case. Although ICD-O-3 classifies insulinoma as /0, the most recent WHO classification lists it as /3. The pathologist and physicians for this case are likely guided by the WHO classification and as a result, would view both the NET diagnosis and the insulinoma diagnosis as malignant. You could report this case as 8240/3 or 8151/3.