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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.

Code carcinoma with neuroendocrine differentiation (8574/3). MP/H rule H7 applies: code the higher ICD-O-3 code. There is non-small cell lung carcinoma (8046/3) and a carcinoma, NOS with neuroendocrine differentiation present (8574/3).

Sequence the brain tumor in the 60-87 series when you do not know the behavior. If you have reason to believe the brain tumor was malignant, sequence it in the 00-59 series.

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).

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.

Rituxan changed categories from chemotherapy to a biologic therapy/Immunotherapy agent effective with cases diagnosed January 1, 2013. See page 150 or page 164 in the 2015 SEER manual. The instruction in the 2014 SEER manual was incorrect regarding Rituxan.

Do not code AlloDerm as either a tissue or implant reconstruction, it is a graft material that usually accompanies implant reconstruction. Placement of a tissue expander is an indication of planned reconstruction. Additional information is needed to determine whether the reconstruction involves tissue or implant.

1. A tissue expander is not always equivalent to Implant reconstruction

2. AlloDerm is not equivalent to tissue reconstruction

3. The combination of AlloDerm and tissue expander is not equivalent to combined (tissue and implant) reconstruction

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.

Do not report a case based on UroVysion test results alone. Report a case when there is positive histology, a physician statement of malignancy, and/or the patient was treated for cancer.

1. Do not report the case.

2. Report the case based on the positive biopsy.

Per the WHO Tumors of Endocrine Organs, for thyroid primaries/cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult (1cm or less in diameter) and was found incidentally. WHO does not recognize the code 8341 and classifies papillary microcarcinoma of the thyroid as a variant of papillary thyroid and thereby should be coded to 8260. If the primary is thyroid and the pathology states papillary microcarcinoma or micropapillary carcinoma, code 8260 is correct. This information will be included in the upcoming revisions to the MP/H manual.