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The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma.

The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor.

Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states:

--Patient had a resection of the non-malignant tumor (not the same tumor) OR

--It is unknown/not documented if the patient had a resection.

This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor?

The Solid Tumor Rules instruct us not to use differentiation for coding histology unless it is specifically listed in the table. The terminology non-small cell carcinoma with neuroendocrine differentiation is not in lung histology Table 2.

However, SINQ 20150033, prior to Solid Tumor rules, indicates this diagnosis should be coded to 8574 (adenocarcinoma/carcinoma with neuroendocrine differentiation).

This presentation appears to represent distinctly different histologies. However, because the 2018 histology diagnosis is not in the table and the prior SINQ appears to disagree with current instruction, it is not clear how to apply the M rules to this case. The outcome of the histology coding will affect the number of primaries reported in this case.

After a little research, it appears as though Endometrial Dedifferentiated carcinoma is a relatively new term and is set to be included in ICD-O-3.2:

http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

If you look at the link on that page for All Additions, Changes, and Revisions to the ICD-O-3, 1st Revision for ICDO-3.2, there is 8020/3 Dedifferentiated carcinoma. Currently, 8020/3 is Carcinoma, undifferentiated, NOS. For 2018 diagnosis, would you use 8020/3 for a predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma as stated in the pathology: Uterus, bilateral ovaries and fallopian tubes; supracervical hysterectomy/BSO: Predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma in the endometrium, FIGO grade 1 Portion of omentum, omental/anterior abdominal wall/ round ligament/uterine/small bowel mesenteric tumor nodules all involved by dedifferentiated carcinoma. Synoptic reads as follows: Histological Type: Endometrioid carcinoma, NOS Dedifferentiated carcinoma predominantly Histological Grade: Endometrioid carcinoma, FIGO grade 1.

The patient is a 34 year old who presented with testicular pain radiating into the abdomen approximately 1 month before orchiectomy in 2018. CT abdomen/pelvis: Multiple focal sclerotic bone lesions. Given the lack of change from July 2014, these are likely benign bone islands. No adenopathy mentioned. He has no prior history of germ cell tumor nor any surgery for any tumor/cancer before this. Pathology: Testis, left, radical orchiectomy: - Demarcated scar tissue (1.3 cm), with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor. No evidence of germ cell neoplasia in situ (GCNIS). - Margins are unremarkable.

Patient has biopsy of prostate 12/28/2018 showing Gleason 5+5 adenocarcinoma. Liver biopsy on same date is metastatic small cell carcinoma consistent with prostate primary. Oncology consult states that liver biopsy is likely neuroendocrine conversion from prostate carcinoma. Patient also has bone metastasis and receives radiation, Lupron, Casodex, and chemotherapy of carboplatin and etopiside.

Per Solid Tumor Rules, we code histology from primary site over a metastatic site. Thus, the small cell carcinoma, which appears to be the focus of the chemotherapy is lost. Is it correct to code this as a single primary with an adenocarcinoma histology?

Both SINQ 20130221 and 20180088 instruct us to abstract multiple primaries when patient develops a metastatic small cell carcinoma of the prostate after being previously diagnosed with adenocarcinoma of the prostate.

There are two histologies provided in the final diagnosis, malignant spindle cell neoplasm (8004/3) and atypical fibroxanthoma (8830/3). There is a definitive diagnosis of the non-specific histology, but the more specific histology is only described using ambiguous terminology.

The external ear (C442) is included in the Head and Neck schema for diagnosis year 2018 and later. The Head and Neck Histology Rules indicate ambiguous terminology cannot be used to code a more specific histology. So ignoring the atypical fibroxanthoma, because it is modified by ambiguous terminology, we are left with a non-reportable site and histology combination (C442, 8004/3).

Diagnoses of malignant atypical fibroxanthomas are regularly diagnosed using the syntax above in our area. Follow-up with the physician or pathologist is generally not possible as these cases are received from dermatopathology clinics only. The pathology report is the only information that will be received. If the reportable diagnosis of malignant atypical fibroxanthoma is ignored per the current Solid Tumor Rules, incidence cases will be lost.

Example: Left thyroid lobectomy with final diagnosis

When the only mention of encapsulation is included in the tumor characteristics of the College of American Pathologists (CAP) summary, not the pathologist's choice of histologic type, what is the preferred histology?

ICD-O-3 Rule H states to use the topography code provided when a topographic site is not stated in the diagnosis. This topography code should be ignored if the tumor arose in another site. For the following brain and central nervous system (CNS) examples, should the suggested sub-site codes be assigned based on the histology, or should the primary sites be coded as C719 (posterior fossa or suprasellar brain) since the only information available was a tumor in these non-specific sites?

Example 1: Resection of a posterior fossa tumor proved medulloblastoma, WNT-activated. Although medulloblastoma has a site-associated code in the ICD-O-3 (C716, cerebellum), the only information available is that this was a posterior fossa tumor (C719).

Example 2: Resection of a suprasellar brain tumor proved pineoblastoma. The pathologist labeled this as a brain tumor, suprasellar. Although pineoblastoma has a site-associated code in the ICD-O-3 (C753, pineal gland), the only information available is that this was a suprasellar brain tumor (C719).