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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.

The 2016 WHO Classification of Tumours of the Urinary System appears to be moving away from using Fuhrman grading toward using WHO/ISUP grade. These seem like similar 4 grade staging systems; however, the SEER Manual specifically states to not use the Special Grade System table for WHO/ISUP. We are seeing the WHO/ISUP grade being used on 2016 pathology reports.

Examples of new grading for renal cell carcinomas

Histologic type: Clear cell renal cell carcinoma

Histologic grade (WHO/ISUP 2016): Grade 3 in a background of 2 (of 4).

And

Histologic type: Clear cell renal cell carcinoma

Histologic grade (ISUP): Grade 2.

The pathology diagnosis reads: Diagnosis Right Kidney, Laparoscopic Nephrectomy: 

-Renal Cell Neoplasm of Oncocytosis (pT1a, pNX See Comment and Template). 

-Surgical margins free of tumor.

Kidney, right, nephrectomy:

Tumor histologic type: Renal cell neoplasms of oncocytosis (see Note)

Sarcomatoid features (%) Not identified

Tumor size: 4 cm (greatest dimension largest tumor)

Other dimensions: 2.7 x 2.5 cm

Macroscopic extent of tumor: Limited to kidney

Focality: Multifocal

Number of tumors: 11 grossly visible, range 0.2 4 cm

Fuhrman grade: 2 of 4

Microscopic extent of tumor:

Perinephric fat invasion: Not identified

Renal sinus invasion: Not identified

Other: N/A

Renal vein involvement: Not identified

Adrenal gland present: No

Involved by tumor: N/A

Direct invasion or metastasis: N/A

Cancer at resection margin: Not identified

Location(s): N/A

Pathologic findings in nonneoplastic kidney: Multiple collections of oncocytic cells

Hilar lymph nodes present: No

Number of involved/number present: N/A

"Thank you for sending this fascinating case. In reviewing the H&E-stained slides, we recognize that multiple lesions of varying sizes are present within the specimen, some with features of oncocytoma, some with those of chromophobe RCC, and yet others with features of both. The immunohistochemical studies for CK7 performed at your institution serve to highlight this point with "mass #1" showing focal single cell staining typical of oncocytoma and "mass #2" showing a patchy and confluent staining pattern typical of chromophobe RCC. This second mass was also positive with special stain for Hales colloidal iron. As mentioned, the morphology varies somewhat in each tumor, however, every single mass is comprised of cells with eosinophilic (pink to bright red) cytopolasm. Some tumors show more tightly nested or sheet like growth, others are more tubular or microcystic. Another important feature, present on slides of renal cortex are microscopic tumorlets seemingly emanating from eosinophilic tubules. This finding, along with the presence of numerous oncocytic neoplasms is supportive of the above diagnosis. The absence of clinical features to suggest Birt-Hogg-Dube syndrome is noted. Although these tumors are not recognized in the current classification of renal tumors, we regard these neoplasms as being a distinct entity, unrelated to both oncocytoma and chromophobe renal cell carcinoma, and have applied the designation "renal tumor of oncocytosis" to such lesions (Gobbo S, et al. Renal cell neoplasms of oncocytosis have distinct morphologic, immunohistochemical, and cytogenetic profiles. Am J Surg Patholl 34:620-626, 2010). We concur that the expected behavior in these cases is one of indolence."

This patient was diagnosed with clear cell carcinoma of the right kidney in 2003, treated with nephrectomy. The tumor was limited to the kidney. An FNA of the pancreas in 11/07 was consistent with metastatic renal cell carcinoma. In 2009 the patient was diagnosed with a right renal fossa mass by CT. The mass was excised on 8/26/09 and showed, "recurrent renal cell ca, clear cell." The path specimen was labeled as, "soft tissue, rt renal fossa." The original 2003 slides were not reviewed and the renal fossa mass was not described as being metastatic.

If the renal fossa soft tissue mass is a new tumor, the MP/H rules for Other Sites directs you to code it as a new primary per rule M10 [Tumors diagnosed more than one (1) year apart are multiple primaries]. Would this be a new soft tissue tumor per rule M10? Or would this be a recurrence of the original kidney primary?

5/27/02 Transurethral resection of bladder tumor (TURBT)--papillary transitional cell carcinoma, +lamina propria, no muscle invasion. All urine cytologies in 2011 and 2012 (only follow up received) show no malignancy. 3/11/15 Lung fine needle aspirate--poorly differentiated carcinoma consistent with urothelial carcinoma. 4/30/15 Renal pelvis biopsy--low grade papillary urothelial carcinoma, no lamina propria invasion, no muscularis propria invasion.

Multilocular clear cell renal cell carcinoma is a specifc histologic type listed in the CAP cancer protocol for kidney, but not in the ICD-O-3 and it is not on the list of specific types of renal cell carcinomas in Table 1 of the kidney equivalent terms and definitions in the MP/H manual. There is a malignant multilocular cystic nephroma 8959 in Table 1, but I can't tell if this the same histology as what is stated in this path report.