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Partial nephrectomy showed carcinoma, histologic type: succinate dehydrogenase-deficient renal cell carcinoma. This is not a term in the ICD-O, and is not a histology covered in the Kidney MPH rules. However, a recent web search indicates this is a specific type of RCC that was added to the 2016 WHO classification of RCC (per abstract: https://www.ncbi.nlm.nih.gov/pubmed/27179267) and makes up 0.05-0.2% of RCC cases (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4229399/).

Table 1 of the 2018 Kidney Solid Tumor Rules (STR) lists succinate dehydrogenase-deficient renal cell carcinoma as histology code 8312, but in the ICD-O-3.2 Coding Table it is listed as histology code 8311.

No changes were made in the Kidney STR. As a result, the histology change described in the ICD-O-3.2 Coding Table conflicts with Table 1. Succinate dehydrogenase-deficient renal cell carcinoma (SDHD) is listed in Table 1 as a synonym for renal cell carcinoma, NOS (8312). However, the ICD-O-3.2 Coding Table lists this as a related term for histology code 8311/3. This related term was not discussed in the Implementation Guidelines, and no change was noted in the STR.

While it seems we should continue to follow the STR, without clarification as to why this histology change was not implemented in STR, achieving consistency will be problematic if registrars jump straight to the ICD-O-3.2 Coding Table to code histology for cases diagnosed 2021 and later. If this code cannot be used for cases diagnosed prior to 2021, should that clarification be included in the STR?

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

Patient was diagnosed in July 2022 with biopsy confirmed left kidney renal cell carcinoma. Initially, partial nephrectomy was planned for February 2023 but canceled at the last moment due to the patient’s “history of narcotic use.” The details of that cancellation were otherwise unclear. It appears the treatment plan was changed due to patient non-compliance.

Patient then had cryoablation of the tumor in May of 2023. Subsequent imaging in October found residual tumor, but no disease progression was noted. Again, additional ablation was offered but patient decided on surgical treatment which did not occur until December 2023.

Is the cryoablation second course due to a change of plan if there is no disease progression, recurrence, or treatment failure?

If the cryoablation is first course treatment, then would the partial resection also be first course treatment because it was documented as the treatment plan?

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.

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."