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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).

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.

Code the diagnosis date for this case as 04/10/2014. Code the diagnostic confirmation as histologically confirmed. The clinician is using all of the information available to determine the diagnosis, including the biopsy and resection.

Code the histology to 8342/3, thyroid oncocytic (oxyphillic) papillary carcinoma.

Yes, Breast rule M10 applies. This case is a single primary.

Follow the MP/H rules even though the "pathologist specifically states the tumors are morphologically different" so that situations like this are reported consistenty accross cancer registries, regions, and states for consistent national reporting.

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.

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.

For cases diagnosed 2018 and later

Differentiated penile intraepithelial neoplasia (differentiated PeIN), is reportable (8071/2).

Please note: Penile intraepithelial neoplasia, grade 3 (PeIN 3) is also reportable to SEER (C600-C609, 8077/2).

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).

Code the primary site to pulmonary artery, C493. According to the WHO classification of tumors, intimal sarcomas are malignant mesenchymal tumors arising in large blood vessels. They show mostly intraluminal growth with obstruction of the vessel. They may occur in the pulmonary vessels or, less often, in the aorta.