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Code histology to combined carcinoid and adenocarcinoma (8244/3). The tumor is a mix of adenocarcinoma and carcinoid.

Code to metaplastic carcinoma, 8575/3. According to our expert pathologist consultant, "The term 'metaplastic stroma' implies that at least a portion of the carcinoma has undergone a 'metaplastic' change from epithelial in appearance to 'stromal' in appearance. I assume this is what CAP means by 'Invasive mammary carcinoma with matrix production,' which the WHO equates to metaplastic carcinoma."

Yes, abstract this as two separate primaries, an invasive melanoma of unknown primary site and an in situ melanoma of the skin on the tip of the nose. Rule M3 applies.

Atypical cartilaginous tumor of bone is not reportable. The WHO terminology is "atypical cartilagenous tumor/chondrosarcoma grade I." WHO classifies this entity as low malignant potential (behavior code /1).

Chondrosarcoma grade II or grade III is reportable based on the WHO classification of malignant (behavior code /3).

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.

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).

Note: Answer verified Sept. 2019, still valid for current cases.

Code all mets at diagnosis fields to 0 when the Stage is I, II, or III.

When the lymphoma is Stage IV, one of the mets at dx fields (other than Mets at Dx-Distant lymph nodes) needs to be coded to 1. Stage IV indicates that there is multiple extralymphatic organ involvement, diffuse involvement of an organ; liver, brain, lung or bone involvement, or bone marrow involvement.

For bone, brain, liver, and lung, code these as 1 when these sites are involved and they are not the primary site. This is the same instruction for solid tumor neoplasms.

For mets at dx-distant lymph nodes, always code to 0. For lymphomas, lymph node involvement is included in stage and not based on whether they are regional or distant.

For mets at dx-other, code to 1 for bone marrow involvement or if there is multi extralymphatic organ involvement.

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/

This case is not reportable based on the information provided. The histologic diagnosis is "metastatic histologically benign smooth muscle neoplasm." The physicians seem to agree with the histologic diagnosis, benign metastasizing leiomyoma (BML). The WHO classification and ICD-O-3 assign 8898/1 to "metastasizing leiomyoma." WHO states "This resembles a typical leiomyoma but it is found in the lungs of women with a history of typical uterine leiomyomas." A recent article states "Because of the hormone-sensitive characteristics of BML, treatments are based on hormonal manipulation along with either surgical or medical oophorectomy." Tamoxifen treatment is in keeping with the BML diagnosis.

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.