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The diagnosis date is 2/24/14.  The pathology report of the pancreas shows: Well-differentiated neuroendocrine tumor (NET), low grade (WHO G1 of 3). Addendum: Ki-67 confirms low grade of pancreatic endocrine tumor (less than 2% Ki-67/MIB-1 index). Chromogranin confirms the endocrine nature of the tumor. The Pre and Post Op Diagnosis is pancreatic neuroendocrine tumor consistent with insulinoma. AJCC Stage as noted on path report: pT1, pNX, pM.

The physician states: The patient has a well-documented insulinoma. Biochemistries confirmed the disease and it is localized in the tail of the pancreas.

The issue with NETs is that pathology report reflects what is seen or what is quantified under the microscope; often, there is a specimen without the accompanying medical history and clinical signs. Many of these NETs are specified on the basis of the hormone, as usually measured in the blood, that is overproduced, something not seen microscopically. All of the islet cell tumors are NETs. The insulinoma in the example above is a well-differentiated NET that is causing insulin to be over-produced. Thus, the diagnoses are not discordant; insulinoma is a more specific NET.

The patient underwent a PTA biopsy of a lytic mass showing metastatic adenocarcinoma. Imaging revealed a large hepatic mass consistent with cholangiocarcinoma. The physician's impression on a physical exam note was the PTA biopsy was most consistent with intrahepatic cholangiocarcinoma. However, the PTA pathology report was reviewed at this facility and the final diagnosis was not stated to be cholangiocarcinoma, only adenocarcinoma, NOS.

The priority order for coding histology rules in the MP/H Manual indicates pathology has priority over documentation in the medical record. Following the rules in the MP/H Manual, the histology would be coded as 8140 [Adenocarcinoma, NOS]. While this may be technically correct, it seems that intrahepatic cholangiocarcinoma is often diagnosed as adenocarcinoma on biopsy, but further stated to be cholangiocarcinoma by the physician once other primary sites have been excluded. By applying the rules in the MP/H Manual, cases that seem better characterized as cholangiocarcinomas are being collected as adenocarcinoma, NOS. Should the histology be adenocarcinoma [8140/3] or cholangiocarcinoma [8160/3] for these cases?

Starting for cases diagnosed in 2024, the SPCSM manual no longer requires the data items for clinical and pathologic tumor size.  Instead, it appears to align with the CoC data item of Tumor Size Summary.  The two manuals contradict each other when it comes to coding tumor size summary for neoadjuvant chemotherapy (NAC) treated cancers.  

STORE states: "If neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999."  

2024 SPCSM states "If neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999." It continues to state     

 12. Assign code 000 when…. (a) no residual tumor is found…(i) Neoadjuvant therapy has been administered and the resection shows no residual tumor &

14. Assign code 999 when...(d) Neoadjuvant therapy has been administered and resection was performed. Do not use a post-neoadjuvant size to code pathologic tumor size; however, you may use the clinical tumor size if available

It seems that we will lose the value of the tumor size summary if we code 000 when NAC is administered and there is no residual disease. 

Example: Patient has a 90 mm triple positive breast tumor and is treated with neoadjuvant TCHP (docetaxel/carboplatin/ trastuzumab/pertuzumab). After completing neoadjuvant therapy, the patient has a mastectomy with no residual disease noted on the final pathology report. 

Using the 2024 SPCSM instructions, code 000 for Tumor Size Summary instead of 090 for the clinical tumor size of 90 mm tumor noted before NAC was administered. This has the potential to affect data analysis, research, and clinical trial accrual.

In the old 2007 Multiple Primaries/Histology rules, Lung Equivalent Terms and Definitions section, oat cell carcinoma (8042) was listed as one of the obsolete terms that was no longer recognized for small cell carcinoma.  That note is not in the current 2018 Solid Tumor Manual lung chapter, and ICDO-3.2 lists oat cell carcinoma as the preferred term for code 8042/3.  Would rule H4, Note 2 apply -- only one histology present, if not listed in Table 3 use ICD-O and all updates, to code oat cell carcinoma as 8042/3?

In our region, pathologists often describe histology using the term phenotype. However, the use of the term phenotype is not discussed in the Solid Tumor Manual.

Example: Final Diagnosis of a colon tumor is invasive adenocarcinoma with a mixed phenotype, and the Diagnosis Comment states: The majority of the disease is poorly differentiated/signet ring cell phenotype.

Would the histology be coded to 8490 (signet ring cell carcinoma), if the majority of the tumor is a more specific histology described by the term phenotype?

The MP/H Rules (Other Sites Terms and Definitions, Table 2) currently lists a separate mixed germ cell tumor code (9101) for germ cell tumors with choriocarcinoma plus teratoma, seminoma or embryonal carcinoma. Is this separate mixed germ cell tumor code still to be used now that all mixed germ cell tumors (9065 and 9085) have been collapsed into code 9085 for analysis per SINQs 20160056 and 20110013? The current WHO Classification for testis tumors does not list code 9101, but also collapses all seminomatous and nonseminomatous mixed germ cell tumors of more than one histologic type under code 9085.

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.