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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

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.

Trichoblastic carcinoma of the skin is not reportable. The WHO classification lists trichoblastic carcinoma as a synonym for basal cell carcinoma, 8090/3. Basal cell carcinoma of the skin is not reportable. See page 11 in the SEER manual, http://seer.cancer.gov/manuals/2015/SPCSM_2015_maindoc.pdf.

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.

Apply Kidney rule H5 and code the clear cell (8310/3) which is the specific type of renal cell. Multilocular is a variant of clear cell which is a variant of renal cell carcinoma. As of yet, no new ICD-O morphology code as been proposed for this specific histology. It will be addressed in the revised rules.

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.

"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.

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."

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.