SEER Inquiry System - Search

The SEER*Rx Database lists Sirolimus as an ancillary agent under the Category section, but as an mTOR inhibitor under the Subcategory. The Remarks section indicates Sirolimus (AKA Rapamycin) is an immunosuppressant, but is also a type of serine/threonine kinase inhibitor. Other types of kinase inhibitors (including Temsirolimus) are types of Chemotherapy. Although the Coding section states this drug should not be coded, Primary Sites (NSCLC and glioblastoma) are listed for this drug.

The SEER*Rx Database page for this drug is confusing. Please address the following.

1) Should Sirolimus not be coded when it is being given as a kinase inhibitor or an immunosuppressant?

2) If Sirolimus is ever treatment, should it be coded only for the primary sites listed?

3) If Sirolimus is given as part of a non-blind clinical trial for another site other than NSCLC or glioblastoma, should the Other Therapy field be coded to 2 [experimental - other treatment]?

This diagnosis is not listed in the ICD-O-3 though it is listed as code 9509/1 for this specific tumor in the 2007 WHO classification of Tumours of Central Nervous System. (See link:  http://link.springer.com/article/10.1007/s00401-007-0243-4/fulltext.html.)

The physician is calling it an indolent tumor, pT3/NX/M0 stage 3 with positive margins. Is the correct code C509, 8502/3?

A segmental resection pathology report states "benign mucosal endocrine proliferation consistent with a 0.3 cm duodenal carcinoid tumor." The diagnosis comment further states, "the separate small endocrine lesion is histologically benign, consistent with a 3 mm carcinoid tumor." This seems to be an example of a description of a microcarcinoid tumor referenced in SINQ 20160011. However, in this new case the pathologist specifically states the tumor is benign.

The WHO definition of microcarcinoid indicates this is a precursor lesion, which seems to indicate it is not malignant. However, SEER's previous answer stated we should report these tumors because the ICD-O-3 definition of carcinoid is 8240/3. Do you think that the mention of the term "benign" in the pathology report is actually related to the size of this lesion? Is the reference to benign mucosal endocrine proliferation referring to the WHO classification (making the case reportable as stated in SINQ 20160011), or is this a situation in which we should apply the Matrix Rule and the case is nonreportable?

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."