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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).

For cases diagnosed 1998-2003:

Code the EOD-Extension to 99 [Unknown] for this case until more information is received. The available information does not describe the primary site and there is a complete lack of staging information.

Head and neck cancers spread early and often to nodes. Do not code the EOD-Extension to localized when the information is as limited as it is for this example.

For cases diagnosed 1/1/2003 and after: A bone marrow transplant can be first course of therapy for cases in which there has been no progression of disease between the initial therapy (e.g., surgery, radiation, chemotherapy) and the bone marrow transplant. Code Hematologic Transplant and Endocrine Procedures field to 10-12 or 40 (depending on the type of bone marrow transplant performed).

Do not use leukemia treatment time guidelines when coding breast cancer treatment.

This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.Use the information from the pathologic evaluation to code CS Lymph nodes.

In the nodes evaluation field, assign code 6 [Regional lymph nodes removed for examination with pre-surgical systemic treatment or radiation and lymph node evaluation based on pathologic evidence]. See CS Lymph Nodes note 4.

Carcinoid type of monodermal teratoma or well differentiated neuroendocrine tumor (carcinoid), monodermal teratoma of the testis is reportable. Assign 8240/3 according to the WHO classification for this neoplasm.

"Likely cancerous" is NOT reportable.

The CoC, NPCR and SEER have agreed to a strict interpretation of the ambiguous terms list. Terms that do not appear on the list are not diagnostic of cancer.

Assign 8323/3, clear cell papillary renal cell carcinoma using the 2018 Kidney Solid Tumor Rules, Rule H1, as this is a single histology, a variant of renal cell carcinoma NOS.

For tumors diagnosed prior to 2007:

The best code is 8381/3 [Endometroid adenofibroma, malignant]. According to our pathologist consultant: "Serous 'fibroadenoma' is not exactly standard terminology. I would guess the pathologist is looking at an adenofibroma with more fibro and less adeno and thus has changed the terminology around. The combination of the benign serous and malignant edometrioid is also a bit unusual. Each of the proposed codes is defendable, but I prefer endometrioid adenofibroma, 8381/3, because it puts the tumor in the adenofibroma category (less common) yet still identifies the malignant element (endometrioid), even though it does lose the serous. But anyone wanting to look at malignant adenofibromas would find the case, and we would carry it under the appropriate malignant component."

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.