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SINQ 20081076 states carcinoid tumorlets of the lung are not reportable and are defined as being less than 5 mm in diameter and benign. Per the WHO Classification of Digestive Tumours, microcarcinoid tumors are precursor lesions/nodules measuring greater than 0.5 mm, but less than 5 mm (0.5 cm). Is the term microcarcinoid tumor equivalent to carcinoid tumorlet, and therefore not reportable? Or is a microcarcinoid tumor a reportable type of neuroendocrine tumor (NET)?

Example: Patient is diagnosed with grade 1 well-differentiated neuroendocrine tumor of the ileum, confirmed on ileocolic resection in 2023. The final diagnosis is a 2.8 cm ileal mass, with focal lymph-vascular invasion and a single 0.6 cm tumor deposit within mesenteric fat; primary tumor completely resected with widely negative margins and 10 regional nodes negative for malignancy.

According to AJCC, mesenteric masses less than 2 cm should be stated in the pathology report as being present and collected by registrars but do not affect stage. EOD Regional Nodes has a code for large mesenteric masses greater than 2 cm only. How should we record these smaller tumor deposits if they are not supposed to affect stage?

According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis.

12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen.

Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma.

12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma.

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?