SEER Inquiry System - Search

Insulinoma, NOS is reportable for cases diagnosed 2021 and later. However, the diagnosis of insulinoma is most frequently made with clinical correlation of the patient's clinical syndrome and serum hormone levels. Despite a pathological diagnosis of NET, this will clinically be stated as insulinoma based on the functional type of tumor. At the largest facility in our area, all pathology reports with a diagnosis of insulinoma over the last year only provide a pathological Final Diagnosis of NET (either G1 or G2), but elsewhere specify, Functional Type: Pancreatic neuroendocrine tumor, functional. Correlation with Clinical Syndrome and Elevated Serum Levels of Hormone Product: Insulin-producing (Insulinoma).

For 2021 and later, it seems this should be accessioned as insulinoma (8151/3), but one cannot arrive at that histology using the current Other Sites (MP/H) H Rules. Following the existing rules, one would code the histology to NET, G1 or NET, G2 (8240 or 8249) per Rule H6. There are technically two specific histologies to consider: NET (either 8240 or 8249) and insulinoma, NOS (8151). Following the H Rules, Rule H6 instructs one to code the histology with the numerically higher ICD-O-3 code (8240 or 8249).

Coding this histology to NET (8240 or 8249) does not seem to reflect the most accurate classification of this tumor, but applying the current rules, this is the only histology that can be coded. There is no current guideline in the Other Sites schema or the ICD-O-3.2 Implementation Guidelines instructing us to ignore the pathological diagnosis of a NET for these tumors (even though insulinomas are NETs). The only SINQ that currently exists (SINQ 20150019) states the histology can be coded as either a NET or an insulinoma in these cases. How are registrars to consistently code histology for these tumors without a rule clarification?

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

The previous SEER Program Coding and Staging Manual included Appendix C that has Coding Guidelines for some sites. The breast guidelines specifically instructed one to code the subsite with the invasive tumor when the pathology report identifies invasive tumor in one subsite and in situ tumor in a different subsite or subsites. The current Breast Solid Tumor Rules Table 1: Primary Site Codes refers one back to the SEER Manual and COC Manual for a source document priority list but does not make mention of invasive vs. in situ on that final version of the source document.

In addition, the Colon Solid Tumor Rules currently contains no Site Coding Section/Table. However, the Lung Solid Tumor Rules do and also refer one to the SEER/COC Manuals for document priority lists. The Urinary Solid Tumor Rules has both the Primary Site Codes Table and an additional section called Priority for Coding Primary Site, which does not reference a document priority list or other manuals. Unfortunately, there is additional information in Appendix C Bladder Coding Guidelines that may have been used in the past regarding site source priority.

Could the remaining applicable Appendix C information be consolidated into the Solid Tumor Rules consistently among all the sites to lessen the need for additional manual referencing? Also, is there a reason one site includes the Priority Site Coding instructions and others do not?

The 2024 Solid Tumor Rules, Table 5: Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids contain notes that say beginning 1/1/2022, keratinizing or non-keratinizing SCCs, HPV positive or HPV negative, are coded 8085 or 8086, respectively, for sites listed in the Head and Neck Solid Tumor Rules, Table 5 only.

Table 5 introductory section also states for cases diagnosed 1/1/2023 forward: “When the diagnosis is a subtype/variant of squamous cell carcinoma and HPV status is also noted, code the subtype/variant.” This latter instruction is also included in Other Sites Table 23 (Penis and Scrotum Histologies) as a “Penis Coding Note.”

Do these instructions ONLY apply to sites on those tables (and only to Penis or to Scrotum also in Table 23)? How should we code HPV-related keratinizing/non-keratinizing or other subtype/variant SCCs, for sites NOT on those tables, given the fact that only the more common histologies are listed in the Solid Tumor tables?  For example, we recently reviewed a case with HPV-positive basaloid squamous cell carcinoma of the anus (C21.0).

The pathology report says: Right knee tumor:

A. discrete subcutaneous mass 3.5x5.2x1.4 cm malignant spindle cell neoplasm (see Comment)

B. A focus of subcutaneous malignant neoplasm is identified in the superior resection margin.

C.All other margins are clear.

The comment mentions that the specimen has been sent to Mayo Clinic and the Mayo clinic consult says, "we still believe that the diagnosis of spindle cell carcinoma is correct. Obviously the differential diagnosis involves melanoma and sarcoma also. The results of the immunoperoxidase stains strongly support the prior diagnosis of a carcinoma."

A 17 year old exchange student was brought into the hospital with appendicitis.  The patient had an appendectomy; there was no follow up treatment.  5/27/2006 pathology report of vermiform appendix:  Adenocarcinoid appendix <5 mm tumor limited to appendix.

The patient has no record in Lexis Nexus and no social security number.  The address is a post office box; additionally, the patient’s birthplace is Switzerland and is lost to follow up.

Final Pathologic Diagnosis:

Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment)

Size of DCIS: 0.7 CM.

Margins: Negative.

Comment

Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised.

12/01/2020 post op visit with surgeon:

Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion.

PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management.

1. Patient presented with a subcutaneous nodule in right axilla. Pathologic impression by initial and reviewing pathologists is that the lesion represents a breast adenocarcinoma arising in ectopic mammary parenchyma. Subsequent breast biopsies were negative.

2. Patient presented with right branchial cleft cyst. The pathologist states the cyst is a primary thyroid adenocarcinoma arising in an ectopic focus of thyroid tissue. The subsequent total thyroidectomy is negative.