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SEER SINQ 20031051 applies to cases diagnosed before 2007 and advises:  Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.

For tumors diagnosed 2007 or later, refer to the MP/H rules.

Patient has diagnosis of melanoma with spindle cell features found in a right lower lobectomy specimen. Chart notes indicate this is metastatic from a cutaneous primary of unknown site. Further work up includes a biopsy of the tip of the nose, which is diagnostic for in situ melanoma. Should this be abstracted as two separate primaries, one for an invasive melanoma of unknown primary site and the other for an in situ melanoma of the skin on the tip of the nose? Which MP/H Rule would apply?

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."

In Appendix C- Breast (SEER Manual 2015) it states under the codes for TOTAL MASTECTOMY (Codes 40-49, 75): For single primaries only, code removal of involved contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item # 1294). [SEER Note: Example of single primary with removal of involved contralateral breast--Inflammatory carcinoma involving both breasts. Bilateral simple mastectomies. Code Surgery of Primary Site 41 and code Surgical Procedure of Other Site 1.] HOWEVER, underneath that it states code 76 is used for: 76 Bilateral mastectomy for a single tumor involving both breasts, as for bilateral inflammatory carcinoma. So

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.)

Unlike most other sites, there is no specific code for a radical surgical procedure of a skin primary. In this case, the patient was diagnosed with a sebaceous cell carcinoma of the lower eyelid skin by punch biopsy. The tumor was large and an orbital exenteration was planned. Despite the extensive surgery performed, skin margins were less than 1 cm. Is an orbital exenteration a "major amputation" (code 60) in this case? Given that the margins were not greater than 1 cm, codes 45 - 47 (which includes a minor (local) amputation) don't seem to apply. However, if this procedure cannot be classified as "minor amputation" then doesn't it seem overkill to refer to the procedure as a "major amputation"?

An alternative would be to code Surgery of Primary Site to 32 for the skin resection (punch biopsy followed by a gross excision of the lesion, margins less than 1 cm) and code Surgical Procedure of Other Site to 2 (non-primary surgical procedure to other regional sites) to record the removal of the globe and orbit as part of the orbital exenteration. Which is correct?