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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 prior to 1/1/2010:

This is one primary. Code the histology according to the original diagnosis, mycosis fungoides. The physician states that this one disease process started as mycosis fungoides and progressed into lymphoma. A physician's statement has priority over other sources in determining the number of hematopoietic primaries.

In October 2006, a committee will begin working on multple primaries among hematopoietic diseases. The committee will provide further guidance on dealing with disease transformation and other issues.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For tumors diagnosed prior to 2007:

Code histology to 8721 [nodular melanoma]. Essentially, "plaque-like nodular spitzoid malignant melanoma" is nodular melanoma. Code 8721 is the most specific ICD-O-3 histology code available for this diagnosis.

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.

For tumors diagnosed prior to 2007:

Medullary is a subtype of duct and "with features of" is a term that indicates a majority of tumor. If this is an invasive adenocarcinoma with no in situ component, code to 8510/3 [Medullary adenocarcinoma]. If only one of the components is invasive, code that 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.

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.The CS Steering Committee is aware of this issue and is working to resolve it.

The histology code is 8720/3 [malignant melanoma, NOS].

There is no specific code for melanoma arising in melanocytic nevus. According to our pathologist consultant, this is likely because nevi are so common, melanoma arising in association with them is common and appears to have no bearing on prognosis or treatment. Most pathologists do not include the nevus in the diagnosis of melanoma, even when they see it.

Code melanomas arising in melanocytic nevi to the appropriate melanoma code, probably 8720, 8721, or 8743 in most cases.

An extracranial schwannoma is not reportable. The schwannoma must arise on the intracranial part of the nerve to be reportable.

For cases diagnosed 1998-2003:

When the prostate feels benign and the cancer is found incidentally at the time of the microscopic exam, code the EOD-Extension field to 10 [number of foci or % of involved tissue not specified]. Code as 13 (less than or equal to 5%) or 14 (greater than 5%) if percentage involved is given in the tissue resected. If the path report states "solitary focus of carcinoma" without mentioning the total amount of tissue resected, code extension to 13. If there is more than one focus, code extension to 10. Don't assign a code of 20 unless the tumor is clinically apparent.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code histology to 9732/3 [multiple myeloma]. The plasmablastic subtype/variant does have a prognostic indication, but the disease is still coded as multiple myeloma.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

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.

Code "small isolated tumor emboli" in the pericapsular lymphatics detected by H&E that are less than 0.2 mm as 05 [Regional lymph node(s) with ITC's detected on routine H & E stains].