SEER Inquiry System - Search

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.

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 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?

The language appears similar to the mucinous cystic neoplasm of the pancreas with high grade dysplasia (8470/2), which was clarified to be reportable in 2014.

The patient is diagnosed by bilateral mammograms suspicious for malignancy in both breasts. A biopsy is done on one breast and is positive. The physician states that he will not biopsy the contralateral breast, as the patient has consented to bilateral mastectomy. The patient receives neoadjuvant chemo, follow by bilateral mastectomies. Both breasts are negative for residual cancer, stated as a complete response. Based on "suspicious for malignancy" can we accession two primaries and assume bilateral complete response?

8/1/2016 Left Abdomen biopsy: Early melanoma in situ (C445-2, 8720/2).

9/2/2016 Upper back: Superficially invasive malignant melanoma (C445-9, 8720/3).

Does rule M4 apply and multiple primaries should be reported or does rule M8 apply and a single primary should be reported?

Patient had radical resection of pelvic giant cell tumor of bone in August 2012. Final diagnosis clarified that no features to suggest a frankly malignant giant cell tumor were identified.

July 2013 left upper lobe nodules were removed and found to be consistent with multifocal metastatic lung involvement with a previous pelvic giant cell tumor of bone. However, the pathology report comment specifies there are no histological high-grade features to suggest a malignancy:

While SINQ 20091087 may apply, these metastases clearly arrived in the lung by hematogenous spread. The previous SINQ note refers to a case where the implants/metastases can seed the surrounding pelvic and abdominal structures by rupture of the tumor or intraoperative tumor spillage. That type of spread is not quite the same as the current case showing tumor cells leaving the primary tumor/site and travelling through the blood to implant in the lungs.

This specific histology (cribriform-morula variant of papillary thyroid carcinoma) is not found in the ICD-O and is not mentioned in the 2007 MP/H Manual. However, per a web search it appears that this is a distinct type of papillary thyroid carcinoma (http://erc.endocrinology-journals.org/content/24/4/R109.full).

Example: Right lobectomy shows thyroid epithelial neoplasm, pending consultation.

Consultation: Thyroid gland, right lobe: papillary thyroid carcinoma, favor cribriform-morula variant.

Consultation Comment: IHC stains argue against medullary carcinoma. The histologic features of growth patterns and cytologic atypia (with rare grooves and pseudoinclusions) and the immunohistochemical profile support a diagnosis of papillary thyroid carcinoma, favoring the cribriform-morula variant. It is important to note that a significant number of patients with this variant of papillary thyroid carcinoma have been associated with familial adenomatous polyposis syndrome.

Patient is an 11 month old with brain biopsy showing final diagnosis of pineal anlage tumor. How are behavior and histology coded for this rare tumor?