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Assign 8077/2 for high-grade squamous intraepithelial lesion, VIN 3 in this case.  The WHO Classification of Female Genital Tumors, 5th edition, states that squamous intraepithelial lesions (SILs) of the vulva are also known as vulvar intraepithelial neoplasia, HPV-associated.  The term squamous cell carcinoma in situ is not recommended.

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

Myeloproliferative syndrome and the myeloproliferative diseases were used in the past to describe myeloproliferative neoplasms. For cases diagnosed 2010 and forward, although the term "myeloproliferative syndrome" is not currently used to describe this disease, the synonyms "myeloproliferative syndrome" and "myeloproliferative disease" were added to the database for myelodysplastic/myeloproliferative neoplasm, unclassified [9975/3].

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.

For cases diagnosed prior to 1/1/2010:There are two primaries:

According to the Single versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, 9673 [Malignant lymphoma, lymphocytic, diffuse, intermediate] and 9680 [Malignant lymphoma, large B-Cell, diffuse] are separate primaries. Again, according to the table, 9680 [Malignant lymphoma, large B-Cell, diffuse] and 9591 [Malignant lymphoma, non-Hodgkin, NOS] are the same primary.

For cases diagnosed 1/1/10 and later, 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 the Histology field to 8201/3 [cribriform carcinoma]. The word "type" is a term that indicates majority of the tumor. The term "cribriform" would be a term used to determine the histology code.

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:

Code the Histology field to the majority of the tumor, which is 8140/3 [adenocarcinoma, NOS]. The terms "areas of" and "foci of" should be ignored because they are not terms that reflect the majority of the tumor. Therefore, we cannot use rule A on page 2 of Coding Complex Morphologic Diagnoses because this diagnosis does not represent a complex morphology.

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.

Use the instructions in the CS Manual, Appendix 1, page 62. This information will be added to the 2004 SEER manual in the next update.

Do not use the Determining Descriptive Tumor Size information from EOD for CS Tumor Size.

Code the Grade, Differentiation field to 1 [grade I] using the information from the BR score.

For cases diagnosed 1998-2003: Grade or differentiation information from breast pathology reports is used in the following priority order:

1. Terminology (well, moderately, poorly)

2. Histologic grade (grade I, grade II)

3. BR scores

4. BR grade

5. Nuclear grade

On the hierarchical list for coding breast grade, the first two priorities do not apply to this case, but the third (Bloom-Richardson scores) does. Add the BR information (2+1+1) for a total score of 4, which translates to BR low grade (code 1). The statement of "II/III" may be a typo that should state I/III.

Report germ cell neoplasia in situ (9064/2). Assign 999 for EOD Primary Tumor and assign 9 for SS2018.

This particular histology is in the Soft Tissue Abdomen and Thoracic schema where EOD PT 000 and SS2018 0 are not available. This histology will be moved to the Ovary schema after redefining certain schemas and thus making the more accurate choices for EOD and SS2018 available. The schema redefine is planned for 2024 implementation.

Intracholecystic papillary neoplasm (ICPN) with low-grade intraepithelial neoplasia is not reportable. The WHO assigns a behavior of 0 to these neoplasms.