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Assign as Stage IV as recommended by our expert hematological oncologist. This is a rare occurrence and this type of presentation does not fit the definition of intraocular extension. Stage IV is probably the best stage for this type of presentation, since there are two extranodal organs involved, even though they involve a bilateral site.

EOD Primary Tumor: 700

SS: 7 (Distant)

Assign 999 to EOD Primary Tumor if this is the only information you have for your case.The mention of nodules does not automatically mean that you have separate tumor nodules. There are many reasons for the appearance of nodules in the lung, some of which are not due to cancer. Unless you have further information on whether the physician has determined that they are related to the lung cancer, then assume that they are not related.

Assign 00 to EOD Mets. Do not code EOD Mets to 10 since you cannot determine whether those nodules are based on the tumor or not.

If you are able to obtain more information, then you can update the EOD Primary Tumor and EOD Mets.

Regarding the second question, if separate tumor nodules are noted, you cannot assume that they are due to tumor. Further information, or clarification, is needed on whether the separate tumor nodules are related to the lung cancer. Without further information, code EOD Primary Tumor to 999.

There is also some information in the CAnswer Forum since Separate Tumor Nodules are a Site-Specific Data Item: http://cancerbulletin.facs.org/forums/forum/site-specific-data-items-grade-2018/96061-lung-separate-tumor-nodules

Assign 999 for pathologic tumor size when patient has received neoadjuvant therapy, even when neo-adjuvant therapy is not completed. Describe the details in text fields.

Assign 9505/1 for Rosette-forming glioneuronal tumor. The new code, 9509/1, has not been implemented in the United States. 9505/1 is to be used until the new code is implemented. See page 7 of the NAACCR Guidelines for ICD-O-D Implementation, effective January 1, 2014, http://www.naaccr.org/LinkClick.aspx?fileticket=u7d3sB71t5w%3d&tabid=126&mid=466.

Assign 9413/0 for glioneuronal neoplasm.

We consulted with our expert neuropathologist about the histology "glioneuronal neoplasm." This term is relatively new and has not yet been recognized by WHO or assigned an ICD-O code. Until such time that WHO determines a code for this neoplasm, our expert instructed us to use 9413/0. Since this is not a recognized neoplasm it is not included in the solid tumor rules.

Assign 9380/1 for low grade glioma diagnosed 1/1/2018 forward and for low grade glioma diagnosed prior to 1/1/2018 assign code 8000/1 on the advice of our expert neuropathologists. The site/type combination of C71 _ and 9380/1 will flag histology/site/behavior edits which should be overridden.

Low grade glioma is an umbrella term or non-specific diagnosis, primarily seen on radiologic reports such as CT scans and MRIs. Often, the patient is actively followed with scans and surgical intervention delayed or not recommended. WHO Classification of Central Nervous System Tumors, 5th edition, does not recognize this term and indicates that tissue diagnosis (including genetic testing) is needed to provide a specific diagnosis. Since biopsy of these “neoplasms” is not routinely done, a definitive diagnosis is not available. Literature searches yielded conflicting information with some stating low grade gliomas are malignant with an indolent clinical course while others felt they were benign. Until such time as WHO proposes a code for this neoplasm, our expert neuropathologists recommend coding glioma, NOS with borderline behavior 9380/1. 

Assign 9362/3 for pineal anlage tumors. According to the WHO Classification of Tumors of the Central Nervous System, 4th edition, pineal anlage tumors, while extremely rare, share features with pineoblastoma. Although they have a distinct morphology, there is no other ICD-O-3 code for pineal anlage tumors.

Assign 9120/3 for epithelioid angiosarcoma.

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.                            

Assign 8802/34 to pleomorphic cell sarcoma/undifferentiated pleomorphic sarcoma. Pleomorphic is more important than undifferentiated when choosing the histology code in this case. Undifferentiated can be captured in the grade code.