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--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--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 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--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--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--Soft Tissue: Is atypical spindle cell neoplasm, primitive myxoid mesenchymal tumor of infancy (PMMTI) from the soft tissue of the leg in August of 2019, reportable?
Primitive myxoid mesenchymal tumor of infancy (PMMTI) is reportable. PMMTI is listed in the new WHO 5th edition Classification of Soft Tissue and Bone Tumors under round cell sarcomas. This is a variant of BCOR sarcomas. There is a new ICD-O histology code assigned for cases diagnosed in 2022 or later (9368/3). Code this 2019 case to round cell sarcoma, undifferentiated 8803/3. Use text fields to explain the details.
Reportability/Histology--Testis: Is neoplasm consistent with carcinoid type of monodermal teratoma reportable as a teratoma, NOS, and if yes, what is the histology code?
Carcinoid type of monodermal teratoma or well differentiated neuroendocrine tumor (carcinoid), monodermal teratoma of the testis is reportable. Assign 8240/3 according to the WHO classification for this neoplasm.
Reportability/Histology--Testis: Is micropapillary serous borderline tumor reportable? Pathology states Testis (C621) radical orchiectomy: Micropapillary serous borderline tumor.
We consulted an expert genitourinary pathologist who advises that micropapillary serous borderline tumor of the testis is reportable. He states "it is the same neoplasm as in the ovary. It arises from tissue (tunica vaginalis) surrounding the testis so is a paratesticular neoplasm."
Please note: not all borderline tumors are reportable and this diagnosis is an exception because it is assigned /2 in ICD-O-3.2. It is reportable for cases diagnosed Jan 1, 2021 and later.
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