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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.

Use the three-grade system table in instruction #7.b to code grade for the situations you describe. Use the Grade Coding Instructions in order. Instruction #7.b (three-grade system) comes before instruction #8 (terminology).

http://seer.cancer.gov/tools/grade/

According to our expert pathologist consultant, yes, seminoma could transform to a mixed germ cell tumor with a somatic type malignancy. He advises us to code this case as 9061/3.

From our expert pathologist consultant: This occurs as "reprogramming" of the initial germ cell tumor/seminoma cell. The process is not understood, but genetic studies support this progression concept. Most often the next step is teratoma. It is out of the teratoma that the somatic malignancy usually comes. I do wonder about the possibility that this was really an embryonal carcinoma which resembles a seminoma - occasionally this can be a difficult separation. I wonder if they radiated the scrotum following the orchiectomy, also, given the scrotal recurrence.

Follow the guidelines in SINQ 20130022 for now. When the MP/H rules are revised, new instructions will be implemented.

See also SINQ 200120078 and 20110069.

This answer pertains to cases diagnosed prior to 2018. For 2018 and later cases, refer to the Non-Malignant CNS Solid Tumor Rules.

Note: This answer updates previous answers which have been removed from the SEER Inquiry System.

Intraosseous meningiomas are not reportable. You are correct, these are rare meningiomas originating in bone. The term "sphenoid wing meningioma" is sometimes used for an intraosseous meningioma of the sphenoid bone. Yes, it's possible to have a meningioma of the sphenoid wing on imaging that arises from the meninges NOT the bone. Read the available information carefully. When the site of origin is described as "along the sphenoid wing" or "overlying the sphenoid wing" report the meningioma. These descriptions indicate that the meningioma originates from the meninges covering bone rather than the bone itself. Meningioma arising in bone is rare enough, that when present, we would expect it to be clearly stated as such. In the absence of a statement indicating origin in bone, the meningioma is most likely arising from meninges covering the bone.

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).

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.

Code microwave tumor ablation as surgery. For lung, assign code 15.

This question was discussed by the technical advisory group – a small group of representatives from each standard setter which meets periodically. The group agreed on this consensus answer.

Abstract a single primary. Based on the information provided, this is a single tumor, and therefore a single primary, Rule M3. Code the histology to malignant phyllodes tumor.

According to our expert pathologist consultant, "The presence of a phyllodes tumor component identifies the whole thing as such. Stromal overgrowth/sarcoma is the usual identifier of malignancy in a phyllodes tumor. (If there were no phyllodes component we would be left with undifferentiated sarcoma, but that is not the case here. The diagnosis of malignancy in phyllodes tumor may be difficult/problematic when there is no overt stromal/sarcoma overgrowth as in this case.) As an aside, the behaviors of pure sarcoma and a phyllodes tumor such as we have here are similar, but we would lose the primary diagnosis if we just called this sarcoma."

Lymphangioma of the brain or CNS is not reportable. Lymphangioma is a malformation of the lymphatic system. Even though it has an ICD-O-3 code, do not report it.