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This patient was diagnosed in 2016 with renal cell clear cell and was coded to that. In 2018, the patient's liver lesion was resected and pathology revealed epithelioid angiomyolipoma perivascular epithelioid cell tumor (PEComa) (8714/3), a new term as of 2018. This was compared to the kidney slides and it was determined to be metastatic PEComa from the kidney. The physician's note states: The patient had a nephrectomy for a kidney tumor in 2016, excision of cutaneous melanomas, and resection of liver mass in 2018. These three cases were sent in consultation. The diagnosis of cutaneous melanoma was confirmed by a dermatopathologist of our department, (a separate report had been already issued). The kidney tumor is poorly differentiated composed of sheets of discohesive cells with markedly pleomorphic cells with frequent giant and bizarre cells. Most of the cells have abundant eosinophilic to clear cytoplasm. The nuclei are enlarged and pleomorphic. Multinucleated cells are numerous. Some cells have markedly enlarged nucleoli. Multifocal tumor necrosis is noted. Extensive lymphovascular invasion is observed. There are foci at the periphery of the tumor consisting of a proliferation of spindle cells with entrapped adipocytes consistent with minor element of unusual angiomyolipoma (see block A18). The liver tumor has histologic features that are similar to the poorly differentiated component of the kidney tumor.

The radiologist noted the mass was most compatible with a chondroid lesion, which is not reportable on its own, but can the subsequent term be used to accession this as reportable if only one malignant etiology is provided by the radiologist? Or does the statement imply that this is only one of several possible etiologies?

Example: Mastectomy specimen final diagnosis shows two foci of invasive ductal carcinoma including: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma (tumor #1, 1 cm, slices 6 and 7) and invasive ductal carcinoma, no special type (tumor #2, 1.2 cm, slices 9 and 10).

Summary Staging outlines, Tumor #1: Histologic Type: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma. As well as, Tumor #2: Histologic type: Invasive ductal carcinoma, no special type. Additional findings include ductal carcinoma in situ (DCIS): presently approximately 3.3 cm, spanning slices 10-13.

The behavior of the solid papillary carcinoma component will affect the provisional histology of the first tumor (8523/3) per Rule H17 vs. 8500/3 per Rule H7). Based on the response, we can determine whether this represents a single or multiple primaries (single primary per M13 vs. multiple primaries per M14).

We are confused by SINQs 20180097, 20150001, and 20140051.  The latter two indicate that “well-differentiated neuroendocrine neoplasms” of the duodenum and appendix are reportable because they’re synonymous with neuroendocrine tumor (NET).  Yet 20180097 states “primary hepatic neuroendocrine neoplasm” is NOT reportable unless there is documentation that it’s being used as a synonym for Primary Hepatic Neuroendocrine Tumor (PHNET).  In addition, we see in the 2021 ICDO-3.2 update that only “poorly differentiated neuroendocrine neoplasm” is listed with behavior code /3 and noted to be reportable for 2021+ on the companion annotated histology list.  Does reportability of neuroendocrine neoplasms depend on primary site, differentiation terminology within the histology name, or something else?  Our casefinding staff is hoping for a general reportability guideline to follow when they come across “neuroendocrine neoplasms” NOS.  For example, we have a 2020 pathology report for a core biopsy of a soft tissue pelvic mass with final diagnosis of low grade neuroendocrine neoplasm; there is no further clarification as to whether it is felt to be primary or metastatic, and we have no other associated records for this patient in our central registry.

Example: MRI found an intradural, extra-axial mass at T12/L1 with possible intramedullary component. Resection of the intradural intramedullary and extramedullary spinal cord tumor found a capillary hemangioma pathologically. The microscopic description on the path report describes a tumor with extensive vascularity involving the dura.

Should we equate the statement of capillary to mean the tumor is arising in a blood vessel as we do for venous hemangioma (non-reportable per SINQ 20130001)? Or should it be reportable as C700, 9131/0 because it is described as involving the dura (intradural, intramedullary and extramedullary)?

In the ICD-O-3, pseudomyxoma peritonei has a behavior code of 6, indicating that it is malignant. Does this imply that pseudomyxoma peritonei is always a reportable malignancy? In the past, our pathologist consultant told us that pseudomyxoma peritonei is only a reportable malignancy if the underlying tumor is malignant. A benign cystadenoma of the appendix, for example, can rupture causing pseudomyxoma perionei. Does SEER agree with our pathologist consultant?

Example: Patient was found to have psuedomyxoma peritonei. Right hemicolectomy was done. Path reported an appendix with mucinous cystic tumor of undetermined malignant potential. A definite diagnosis of cancer can not be rendered.

We have questions regarding reportability of some terms/diagnoses after a review of EIN cases back to 2021. While some seem synonymous with EIN, others have different terms in the pathology report though the physician is treating as if they have the diagnosis.  

1. Atypical glandular epithelium

Scenario: Endometrium biopsy with ablation performed at Facility A on 8/7/2024 showed atypical glandular epithelium. Patient was sent to Facility B where the total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) on 9/20/2024 showed other reactive fibrosis and obliterated architecture compatible with history of ablation. Is atypical glandular epithelium synonymous with and coded as EIN?

2. Isthmic-type mucosa with focal severe atypia

Scenario: Endometrium biopsy showed isthmic-type mucosa with focal severe atypia.  Then Facility B did TAH/BSO that showed no evidence of high grade dysplasia, atypical hyperplasia, or carcinoma.

3. Simple hyperplasia without atypia 

Scenario: Endometrial biopsy pathology states simple hyperplasia without atypia and the TAH/BSO is either negative or has the same histology; however, the treating physician is stating EIN. 

4. EIN/CAH or focal EIN/CAH

Scenario: Biopsy showed EIN/CAH but the total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) pathology or the Mirena IUD treatment operative note states no EIN/CAH/Atypical hyperplasia. Are these reportable, similar to an in situ when the re-excision lumpectomy or mastectomy is negative or no residual disease?

Are neuroepitheliomatous neoplasms such as ganglioneuroma, gangliocytoma, ganglioglioma occurring along the spinal cord reportable? Are nerve sheath tumors such as neuroma occurring along the spinal cord reportable? Angioma?

Reference: SINQ 20051071; Primary Central Nervous System Tumors, NPCR Training Materials 2004

Since high grade squamous intraepithelial lesion (HGSIL) is reportable for the vulva in 2018 (per SINQ 20130185) but VIN II-III is not reportable, we need to clarify this reporting format seen in our area.