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In this case, the lymphoma arose in the posterior orbit and the primary site was coded as C696 (orbit, NOS). The mass directly extended to at least one "adjacent" site, the lacrimal gland. Should SS2000 be coded to 1 (localized) or 5 (regional, NOS) when an ocular adnexal lymphoma arises in the posterior orbit and extends to involve the lacrimal gland? Although both the posterior orbit and the lacrimal gland are parts of the orbit, they have separate ICD-O-3 topography codes. Should extension to multiple sites within the orbit be classified as localized disease?

The issue is what constitutes "adjacent" structures for a tumor that arises in the orbit. In an article published by the Indian Journal of Opthamology it states, "According to the Ann-Arbor staging system, lymphoma confined to the orbit is designated as Stage I, involvement of adjacent structures (sinuses, tonsil and nose) makes it Stage II." Does SEER agree with this definition of "adjacent" structures? Or are the lacrimal gland, ciliary body, retina, conjunctiva and/or choroid "adjacent" structures for a lymphoma stated to arise in the posterior orbit?

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?

Would you code an excisional biopsy as Surgery for the following case?

The patient presented with a large protruding polypoid anal canal mass. The diagnosis of malignancy was made following a procedure referred to by the surgeon as an excisional biopsy. The protruding portion of the anal canal mass was excised, but the deep margin was grossly involved. The PE exam after the "excisional biopsy" found a firm mass, 4 cm in length on DRE. Further work-up with imaging showed gross residual disease extending to adjacent skeletal muscle (external anal sphincter). Although the internal/protruding anal canal portion of the tumor was excised, there was clearly extensive residual tumor. The patient underwent definitive concurrent chemoradiation only; subsequent surgery was not planned or performed.

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."