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A patient received a kidney from her son. The son's kidney had a cyst which was removed prior to the transplant and later determined to be renal cell ca. Who do we report, the donor or the recipient?

2015 Rt kidney nephrectomy pathology states: congenital cellular mesoblastic nephroma, tumor sz 5.9cm, tumor limited to kidney, extension into pelvicalyceal system, margin not applicable, LVI negative. Per PubMed.gov: (In newborns) among the low-grade malignant tumors, congenital mesoblastic nephromas can be successfully treated with simple nephrectomy. Per ScienceDirect: ...currently thought that cellular mesoblastic nephroma is actually a renal variant of infantile fibrosarcoma.

Kidney, right interpolar neoplasm, partial nephrectomy: Oncocytic renal neoplasm of low malignant potential (ORNLMP).

Within part B, right interpolar kidney neoplasm, the neoplasm shows oncocytic features, with abundant granular eosinophilic cytoplasm and enlarged vesicular nuclei with prominent central nucleoli. The cells are arranged in small nests and tubules with hypocellular fibrous stroma identified within the background. Scattered binucleated cells are present, and rare cells with irregular nuclear membranes are present. No perinuclear halos or prominent cell membranes are present. Given the histologic features, the neoplasm is best classified as an oncocytic renal neoplasm of low malignant potential (ORNLMP).

We are aware that the WHO 4th edition for urinary tumors has changed the behavior of “clear cell papillary renal cell carcinoma” to /1 but registries are to continue collecting as /3.

While the diagnosis in our case is stated as “tumor” it does seem like the pathologist may be using the new WHO terminology of “tumor” rather than “carcinoma,” so we are not sure if behavior is /3 or /1.

First, reportability of clear cell papillary renal cell carcinoma changed from 8323/3 to 8323/1. Although it does not appear the standard-setters implemented this change, note of the conflict between the ICD-O-3.2 and the Solid Tumor Rules (STR) is not included in the Implementation Guidelines or STR. The current Note for clear cell papillary renal cell carcinoma (8323) was left in Table 1, so this presumably is still reportable. It would be helpful if the conflict with ICD-O-3.2 was addressed, especially since the existing Note refers to changes made back in 2016 (not 2018 or 2021).

Second, is the term sarcomatoid renal cell carcinoma still coded as a synonym for renal cell carcinoma (8312) because sarcomatoid is referring to a pattern of differentiation or 8318 (renal cell carcinoma, sarcomatoid)? The STR, Table 1, lists sarcomatoid renal cell carcinoma as 8312, but the ICD-O-3.2 lists this as 8318. The Note in Table 1 still indicates WHO/IARC and College of American Pathologists agree that sarcomatoid carcinoma is a pattern of differentiation, not a specific subtype, of renal cell carcinoma. This appears to conflict with WHO/IARC ICD-O-3.2 Coding Table as it provides a different, specific histology code for this malignancy. How can WHO/IARC classify this both a pattern of renal cell carcinoma and a separate, specific histology?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

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.