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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).

Assign C211 for anal verge. Anal verge is defined as the lower (distal) end of the anal canal, junction between the skin of the anal canal and the perianal skin, http://www.seer.cancer.gov/manuals/2015/AppendixC/rectosigmoid/coding_guidelines.pdf

If there is no further information about where the cancer originated, assign C220. Use ICD-O-3 as the source for coding topography. The topography code associated with combined hepatocellular and cholangiocarcinoma (8180/3) is C220 when there is no other information available, according to ICD-O-3.

Pseudotumor cerebri is not reportable. It is not a neoplasm. The pressure inside the skull is increased and the brain is affected in a way that appears to be a tumor, but it is not a tumor.

Report this case as a basal cell carcinoma, noninvasive, of the nasal cavity, based on the information provided.

The polyp was removed from the nasal cavity (C300) which is a reportable site for basal cell carcinoma.

Code Lupron given for breast cancer in the "Other" treatment field using code 6 (other-unproven). Lupron is still not an approved hormone treatment for breast cancer and should not be coded in the hormone field.

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.

Idiopathic hypereosinophilia is not reportable.

Hypereosinophilic syndrome is a different entity and is a synonym for chronic eosinophilic leukemia.

Accession the new tumor in the transplanted organ as you would any other new/second primary. As transplants have become more common especially for liver, lung, and kidney, we are seeing more of these types of cases. We are adding instructions to the revised MP/H rules on coding subsequent primaries when they occur in a transplanted organ. We are also looking at adding a data field that will identify cancers/tumors which arose in a transplanted organ. We feel this is important to track for analysis. Until the revised MP/H rules are implemented, we will look at adding general coding instructions to the SEER Program Manual for transplants.

The neoplasm is reportable if it originated in the spinal cord or is intradural (within the spinal dura; spinal nerve roots are intradural). If there is not enough information to determine the exact site of origin, do not report the case.