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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.

Read the operative report and the pathology report and assign the surgery code that best represents the extent of the surgery. In this case, code 30 seems most appropriate. Do not assign the surgery code based only on the name of the procedure; use all information available to chose the most representative code.

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.

Noninvasive low grade (micropapillary) serous carcinoma (MPSC) of the ovary is reportable. Assign code 8460/2, applying the ICD-O-3 matrix concept to this noninvasive carcinoma. Noninvasive can be used as a synonym for in situ, ICD-O-3 behavior code /2. See page 66 in the softcover ICD-O-3. Low malignant potential (LMP) means that the neoplasm is not malignant, but has some chance of behaving in a malignant fashion. LMP can be used as a synonym for ICD-O-3 behavior code /1, see page 66.

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.

"Well-differentiated neuroendocrine neoplasm" of the appendix is reportable. According to the WHO classification of Digestive System Tumors, "Well-differentiated neuroendocrine neoplasm" of the appendix is synonymous with NET. WHO states on page 13 "The term 'neuroendocrine neoplasm' can be used synonymously with 'neuroendocrine tumor.'"

Neuroendocrine "tumor," or NET G1, is listed in the WHO classification as one of the malignant neoplasms of the appendix.

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.

Revised Answer

Assign code 22 when a patient has a lumpectomy and an additional margin excision during the same procedure.

According to the CoC, "Re-excision of the margins intraoperatively during same surgical event does not require additional resources; it is still 22. Subsequent re-excision of lumpectomy margins during separate surgical event requires additional resources: anesthesia, op room, and surgical staff; it qualifies for code 23."

SCC of skin, signet ring cell type, is not reportable to SEER. SCC's of skin classifiable to 8050-8084 are not reportable to SEER. See page 11 in the SEER manual, http://seer.cancer.gov/manuals/2015/SPCSM_2015_maindoc.pdf

Signet ring is a rare histological variant of SCC and is coded to 8070/3 according to the WHO classification for skin tumors.

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).