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Code 0 for Surgical Procedure of Other Site in this case. The Surgical Procedure of Other Site field is used to capture surgery to destroy or modify cancer tissue that is not captured in other surgery fields.

Code the histology as well differentiated mucin producing adenoca based on the 4/10/13 pathology report.

Code histology from the pathology report of the procedure which removed the most tumor tissue -- this is from the MP/H general instructions for coding histology. We are assuming that the partial hepatectomy removed the most tumor tissue in this case.

Per WHO, mucin producing adenoca is a variant of cholangiocarcioma.

The code in Surgical Procedure of Primary Site should correspond to the most invasive, extensive, or definitive surgery when the patient has multiple surgical procedures of the primary site even if there is no residual tumor found in the pathologic specimen from the more extensive surgery. The timing of the procedures does not affect the code choice.

Assign code 27 for the first example. Assign code 24 for the second example.

Do not report primary acquired melanosis with atypia.

According to our expert pathologist consultant, "There has been a lot of debate in the literature about the diagnostic criteria, terminology, and natural history of primary acquired melanosis [PAM]. Your case comes down squarely on the main issue, which is whether PAM with atypia should be regarded as melanoma in situ. In most studies it appears that PAMs with no atypia or mild atypia do not progress to melanoma, and only a small percentage of those with severe atypia do so."  "PAM, even with atypia, is not melanoma in situ, and should not be reported."

For further information, see this article for a review of a large number of patients: Shields, Jerry A, Shields, Carol L, et al. Primary Acquired Melanosis of the Conjunctiva: Experience with 311 Eyes. Trans. Am Ophthalmol Soc 105:61-72, Dec 2007.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

For cases diagnosed prior to 1/1/2022

Low-grade appendiceal mucinous neoplasm (LAMN) is not reportable, even when it spreads within the peritoneal cavity, according to our expert pathologist consultant. Peritoneal spread of this /1 neoplasm does not indicate malignancy. It is still /1 when there is spread of LAMN in the peritoneal cavity.

The term neuroendocrine is often included with other histologies and usually means that neuroendocrine cells are present but not neuroendocrine tumor.

1.  If the neuroendocrine cells are stated to be either small cell or large cell, code that histology; however, neuroendocrine, NOS mixed with urothelial does not have an applicable mixed code. Code histology to 8120.

2.   Code histology to squamous cell carcinoma, nonkeratinizing, NOS (8072/3). The neuroendocrine component is not specified as either small cell or large cell.

3.  Code to 8523/2 per MP/H Rule H6 as intraductal mixed with other types of carcinoma present.

Note that while neuroendocrine differentiation can be identified, it seems to have no prognostic implications. We have consulted with our site specific Subject Matter Experts on how best to capture neuroendocrine, NOS when combined with other histologies. These instructions will be included in the revision of the MP/H rules including the wording of MP/H breast rule H6.

Follow the instructions in the 2014+ Grade Coding Instructions to code grade for cases diagnosed 2014 and later, http://seer.cancer.gov/tools/grade/   Instruction #4.a. states to code grade for in situ tumors when grade is specified. This instruction applies to bladder cases, as well as other in situ tumors.

1. Assign grade code 2

2. Assign grade code 4

See the note below the table in instruction #7.

Follow rule H3 and code squamous cell carcinoma for these cases unless you can obtain confirmation that these cases should be coded keratinizing squamous cell carcinoma from the lab and/or pathologist. Document this confirmation in your policies and procedures.

The MP/H rules were written with input from leading pathologists in each specialty area. Based on their expert opinion, we instruct registrars to code histology based on the information in the final diagnosis. The microscopic description may contain other terms, but the pathologist lists only the pertinent terms in the final diagnosis.

When the pathology diagnosis is a neuroendocrine tumor (/3) and the clinical diagnosis is an insulinoma (/0), report the case.  Although ICD-O-3 classifies insulinoma as /0, the most recent WHO classification lists it as /3. The pathologist and physicians for this case are likely guided by the WHO classification and as a result, would view both the NET diagnosis and the insulinoma diagnosis as malignant. You could report this case as 8240/3 or 8151/3.