First course treatment/Other therapy: How is Sirolimus (Rapamycin) to be coded when given with known chemotherapy agents in a clinical trial? See discussion.
The SEER*Rx Database lists Sirolimus as an ancillary agent under the Category section, but as an mTOR inhibitor under the Subcategory. The Remarks section indicates Sirolimus (AKA Rapamycin) is an immunosuppressant, but is also a type of serine/threonine kinase inhibitor. Other types of kinase inhibitors (including Temsirolimus) are types of Chemotherapy. Although the Coding section states this drug should not be coded, Primary Sites (NSCLC and glioblastoma) are listed for this drug.
The SEER*Rx Database page for this drug is confusing. Please address the following.
1) Should Sirolimus not be coded when it is being given as a kinase inhibitor or an immunosuppressant?
2) If Sirolimus is ever treatment, should it be coded only for the primary sites listed?
3) If Sirolimus is given as part of a non-blind clinical trial for another site other than NSCLC or glioblastoma, should the Other Therapy field be coded to 2 [experimental - other treatment]?
Sirolimus is used to treat GVHD (graft versus host disease) and is not coded as treatment. Even though the sub-category is mTOR inhibitor it does not automatically mean it is a chemotherapeutic agent. Sirolimus affects cells differently than Temsirolimus. The chemical compounds differ between these drugs. In order to code rapamycin, the drug given must be stated to be either the analog or ester compound. The SEER*RX database has been corrected and NSCLC/glioblastoma are no longer listed for sirolimus.
We researched clinical trials and found several that include sirolimus with other chemotherapy drugs for patients who either have received or will be receiving bone marrow transplants for hematologic diseases. In this case it is not coded. There are a few trials that are looking at sirolimus as a treatment for bladder, prostate, nerve sheath tumors, MDS, and AML. For these cases it would be coded in Other (code 2).
MP/H Rules/Histology--Brain and CNS: What is the histology code for a tumor originating in the cerebellum and extending into the fourth venrticle described as a glioblastoma with primitive neuroectodermal tumor component (WHO Grade IV)?
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.
Reportability--Thyroid: Is a final diagnosis of "non-invasive follicular thyroid neoplasm with papillary-like nuclear features" (NIFTP) reportable when the diagnosis comment states this tumor was historically classified as encapsulated follicular variant of papillary thyroid carcinoma? See Discussion.
The term "non-invasive follicular thyroid neoplasm with papillary-like nuclear features" is now being used, instead of the previous classification of an encapsulated malignant thyroid tumor. Recent evidence supports a very minimal risk of aggressive behavior for these tumors, and pathologists in our area are no longer classifying these as malignant in the final diagnosis.
As of January 1, 2021
Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) C739 is no longer reportable for cases diagnosed 1/1/2021 forward. See the ICD-O-3.2 material on the NAACCR website,https://www.naaccr.org/icdo3/#1582820761121-27c484fc-46a7
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Answer for cases diagnosed 1-1-2017 to 12/31/2020
Report NIFTP and assign ICD-O-3 morphology code 8343/2. See the NAACCR document, page 3, https://20tqtx36s1la18rvn82wcmpn-wpengine.netdna-ssl.com/wp-content/uploads/2017/01/What-You-Need-to-Know-for-2017.pdf
MP/H Rules/Histology: What is the correct histology code for a NUT midline carcinoma?
Code histology to 8010/3.
NUT carcinoma is identified by the NUTM1 gene rearrangement.
NUT midline carcinomas (NMC) are lethal and morphologically indistinguishable from other poorly diff carcinomas. They are epithelial tumors which can range from undifferentiated carcinomas to carcinomas with prominent squamous differentiation.
A new proposed ICD-O-3 code has been suggested for NUT tumors but it is not yet approved for implementation. Do not use the new code until it is approved for use in the United States.
Primary site/MP/H Rules/Histology: What is the appropriate site and histology code for a tumor described as a "Large mass In suprasellar cistern encroaching into sphenoid & ethmoid sinuses", with the pathology described as "INI-1 deficient sinonasal undifferentiated carcinoma"? Of note, this patient has a history of a pituitary adenoma, resected overseas a few months prior to this diagnosis.
The primary site is unclear. The lesion is intracranial, but this may not be the primary site. In the absence of any additional information, assign C390, 8020/3. According to WHO, sinonasal undifferentiated carcinoma can involve the nasal cavity, maxillary antrum, and/or ethmoid sinus.
SMARCB1 (INI-1) is a tumor-suppressor gene located on chromosome 22q11.2. Tumors that showed loss of expression were SMARCB1-deficient tumors which are characterized by nests, sheets, and cords of cells without any histologic evidence of specific (eg, squamous or glandular) differentiation.
Diagnostic confirmation: When a CT guided Fine Needle Aspiration is performed and the pathology report indicates smears and cell block were prepared, if the diagnosis is positive for cancer, can you code diagnostic confirmation as 2 (positive cytology) because of the cell block?
Yes, assign diagnostic confirmation code 2 for diagnosis based on smears and cell block from CT guided FNA. This reply pertains to solid tumors.
Reportability--Appendix: Is a mucinous cystic neoplasm with high grade dysplasia of the appendix reportable? See discussion.
The language appears similar to the mucinous cystic neoplasm of the pancreas with high grade dysplasia (8470/2), which was clarified to be reportable in 2014.
WHO does not list MCN as a histology for the appendix. This case should be clarified with the pathologist.
For pancreas specifically, the term "mucinous cystic neoplasm (MCN) with high grade dysplasia" replaced the term "mucinous cystadenocarcinoma, noninvasive" according to WHO. MCN with high grade dysplasia of the pancreas is reportable because it is used in place of the now obsolete terminology. If we did not make the new terminology reportable, trends over time could be affected.
Grade--Kidney: Should WHO/ISUP grade for renal cell carcinoma be coded for cases diagnosed 2016 and later? See discussion.
The 2016 WHO Classification of Tumours of the Urinary System appears to be moving away from using Fuhrman grading toward using WHO/ISUP grade. These seem like similar 4 grade staging systems; however, the SEER Manual specifically states to not use the Special Grade System table for WHO/ISUP. We are seeing the WHO/ISUP grade being used on 2016 pathology reports.
Examples of new grading for renal cell carcinomas
Histologic type: Clear cell renal cell carcinoma
Histologic grade (WHO/ISUP 2016): Grade 3 in a background of 2 (of 4).
And
Histologic type: Clear cell renal cell carcinoma
Histologic grade (ISUP): Grade 2.
Do not record WHO/ISUP grade in the grade/differentiation field.
Designated fields for this grade system are being proposed for future implementation.
MP/H Rules/Histology--Testis: How should histology be coded for a testicular primary with a combination of teratoma, yolk sac tumor and embryonal carcinoma? See discussion.
Patient had a radical orchiectomy with the final diagnosis of "Mixed germ cell tumor with the following features -- histologic type: Mixed germ cell tumor (teratoma 50%, yolk sac tumor 25%, and embryonal 25%)."
Assign 9085/3. Code this combination of teratoma, yolk sac tumor, and embryonal tumor in the testis to mixed germ cell tumor (9085/3) based on the WHO Classification of Tumors of the Male Genital Organs.
First course treatment/Radiation Therapy--Prostate: How do you code fiducial markers for prostate cases?
Do not code fiducial markers as a form of radiation treatment; rather, code the radiation therapy in the radiation treatment section. Fiducial markers are small metal spheres, coils, or cylinders that are placed in or near a tumor to help guide the placement of radiation beams during treatment.