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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).

There is a discrepancy between Notes 3 and 6 in the schemas Fallopian Tube, Ovary, and Primary Peritoneal Carcinoma for EOD Primary Tumor. Note 3 describes extension/discontinuous metastasis to the pelvic sites (code 450) and includes the sigmoid colon, rectosigmoid and rectum since these are all pelvic sites. However, Note 6 also includes rectosigmoid and sigmoid colon.

Note 6 is describing extension/discontinuous metastasis to the abdominal sites (600-750), so it should include rectosigmoid or sigmoid colon (since those are pelvic sites). Note 6 indicates, Intestine, large (except rectum). In the previous Collaborative Stage, the corresponding note used to also include: except sigmoid colon, rectosigmoid and rectum.

Did sigmoid colon and rectosigmoid get removed from the list here? That is, should Note 6 read, Intestine, large (except sigmoid colon, rectosigmoid, rectum)? Involvement of the sigmoid, rectosigmoid, or rectum via peritoneal seeding/metastasis is consistent with T2b disease and would correlate with code 450 (pelvic sites), not codes 600-750 (abdominal sites). Those codes only correlate with T3 and greater disease (i.e., peritoneal seeding/metastasis of the abdomen).

In the April 2019 Breast Solid Tumor Rules update, the Priority Order for Using Documentation to Identify Histology was changed, giving equal priority to the Final diagnosis / synoptic report as required by CAP (item 2B).

There are technically two histologies documented for the case above; a Not Otherwise Stated (NOS)/No Special Type (NST) (invasive mammary carcinoma, per final diagnosis text) and subtype/variant (invasive cribriform carcinoma, per CAP report). If we do not use the synoptic report with priority over the final diagnosis, Rule H14 indicates the histology would be the NOS histology (invasive mammary carcinoma) because the percentage of tumor is not given for the subtype. However, SINQ 20180045 states, In the CAP protocol, the term Histologic Type is a label where the histology that corresponds to the largest carcinoma is collected. According to the CAP protocol for invasive breast cancer, the histologic type corresponds to the largest carcinoma.

If the pathologist summarizes the findings in a synoptic report, should the specific Histologic Type identified have priority?

The patient was diagnosed in 2018 with met melanoma to the brain found via imaging. The staging procedure was a single axillary lymph node excision which was positive for metastatic melanoma. The exact site of the primary was never determined and the site code is C449. Is the axillary lymph node regional or distant? This affects how I code regional lymph nodes positive, regional lymph nodes examined, and scope of regional lymph node surgery or surgical procedure other site. Similar question was asked in the past (question # 20091101) but I have not found this question restated since the 2018 changes and just want to verify this is still what we are to do.

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

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.

According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded?

Core biopsy of left breast at 2:00: Invasive ductal carcinoma, Nottingham score 6/9.

Core biopsy of left breast at 4:00: Invasive mucinous carcinoma (variant of ductal carcinoma), Nottingham score 5/9.

Post neo-adjuvant mastectomy: Main (largest tumor): Invasive ductal carcinoma, upper outer quadrant grade 2. Secondary tumor: mucinous carcinoma, grade 1 at 4:00.

Example: An October 2017 glans penis biopsy final diagnosis was reported as: Undifferentiated (Warty-Basaloid) penile intraepithelial neoplasia.

In January 2018, an additional penile glans biopsy final diagnosis was reported as: At least squamous cell carcinoma (SCC) in situ (HGPIN). Foreskin circumcision on the same pathology report shows SCC in situ.

It is unclear whether the term undifferentiated is synonymous with high-grade for the purposes of determining penile intraepithelial neoplasia (PIN/PEIN) reportability and diagnosis date.

There is one tumor in the left lung that is acinar adenocarcinoma, 8551/3, and two tumors in the right lung, one of which is 8551/3 and a separate one that is mucinous adenocarcinoma 8253/3.

3/21/18- left robotic video assisted thoracoscopy with left lower lobe lobectomy: 2.5 cm adenocarcinoma, acinar predominant, margins negative

11/3/18- right upper lobe lobectomy: invasive mucinous adenocarcinoma, 1.7 cm, invasive adenocarcinoma, acinar predominant, 0.6 cm, margins negative

If you start by comparing the 8551/3 left lung tumor to the 8253/3 right lung tumor, M6 applies and these would be separate primaries (seq 01 and seq 02). How would we handle the third tumor, 8551/3, in the right lung? Seq 01: 3/21/18- left lung primary 8551/3 Seq 02: 11/3/18- right lung primary 8253/3 Is the right lung tumor 8551/3 a third primary, and if so, which M rule applies? I cannot find a rule that seems to fit completely. Rule M6 may apply if you were comparing the right 8551/3 tumor to the seq 02 8253/3 tumor. But how would you know to use the seq 02 histology code 8253/3 or seq 01 histology code 8551/3 for the comparison? I think M9 was designed for situations where you have multiple tumors involving both lungs but they didn't biopsy all of them. Is that correct? If so, then we would be able to bypass M9. Would M11 apply since we already took care of two of the tumors with rule M6? If M11 doesn't apply, it seems like you would get to M14.