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The WHO Classification of CNS tumours lists glioblastoma with primitive neuroectodermal tumor component as a subtype of glioblastoma and assigns 9440/3. Also referred to as glioblastoma with a primitive neuronal component.

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).

Rule H8 applies, code the histology with the numerically higher ICD-O-3 code which is papillary transitional cell carcinoma, 8130.

Based on the information provided, there is a single bladder tumor, papillary urothelial carcinoma with squamous cell differentiation. Urinary sites rule H12 does not apply because this is a single tumor, not multiple tumors. In the single tumor H rules, H3 does not apply as this rule does not include papillary transitional cell carcinoma. Rule H4 is papillary carcinoma or papillary transitional cell carcinoma and refers you to Table 1. Table 1 does not list papillary urothelial carcinoma with squamous cell differentiation because there is no ICD-O-3 code for this histology. Table 1 does list transitional cell carcinoma with squamous differentiation as code 8120, however, the papillary transitional cell carcinoma is the higher code, 8130. We will review this situation for the next version of the 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 Mets Eval code refers to the method used to evaluate the site farthest from the primary site. The correct code may not be the highest eval code. For example 1 above, if the liver is the site farthest from the colon primary that was evaluated for distant mets, code the CS Mets Eval code to the method used to evaluate liver. Code surgical evaluation as 1.

Assuming this is all of the information about possible distant metastatic sites for the examples above, code CS Mets at DX as 00, and CS Mets Eval as 1 for each.

Please note: imaging of farther sites should also be included when CS Mets at DX is coded. For example, if there was also a negative chest X-ray, the CS Mets at DX field would be 00 but the CS Mets Eval field would be 0 because the CXR documents that there are no mets beyond the immediate area of the tumor.

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

No. Polycythemia, NOS is not reportable.

Polycythemia (also known as polycythaemia or erythrocytosis) is a disease state in which the proportion of blood volume that is occupied by red blood cells increases. Blood volume proportions can be measured as hematocrit level. It can be due to an increase in the mass of red blood cells, "absolute polycythemia"; or to a decrease in the volume of plasma, "relative polycythemia".

The phlebotomy is a treatment for the excessive blood volume; therefore, a diagnosis of "polycythemia" without one of the modifying terms listed in the Heme DB under Alternative Names is not reportable.

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.

Assign histology code 9505/1 [Ganglioglioma, NOS].

Rosette-forming glioneuronal tumor of the 4th ventricle is a new WHO entity. There is no current ICD-O-3 code for this. The best code available at this time is 9505/1.

For cases diagnosed prior to 1/1/2010:Yes. Code the Grade, Differentiation field to 8 [NK cell] rather than 5 [T-cell]. Code the Histologic Type to 9719/38 [NK/T-cell lymphoma, nasal and nasal-type with Cell indicator of NK (8)].

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 cases diagnosed prior to January 1, 2008, enter the date of the lymph node biopsy (excisional biopsy or biopsy NOS) as the Date Therapy Initiated for a lymphoma when the biopsy is the first or only therapy performed.

Code Reason for No Surgery of Primary Site as 0 [Surgery of the primary site was performed] and the biopsy of a lymph node is coded to 25 in Surgery to Primary Site.

Do not code a fine needle aspiration or core needle biopsy in Surgery of Primary Site.