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For cases diagnosed 2007 or later, apply rule M8 and abstract this case as multiple primaries.

Rule M7 does not apply when each ovary has a distinctly different histology, even when both histologies are with the specified code range. This clarification will be added to the next version of the rules.

The CPC Validity Status of the site morphology combinations of C569/8441/3 and C569/8441/2 was revised from Valid to Unlikely with the latest release of the Version v24A Edits Metafile. As a result, this site and morphology combination will now require an over-ride flag to be set.

Code as 8461/3 (high-grade serous carcinoma) or 8460/3 (low-grade serous carcinoma) if at all possible. Use 8441/3 (serous carcinoma, NOS) only if it cannot be distinguished as low grade or high grade. The codes for high-grade serous carcinoma and low-grade serous carcinoma are relatively new. High-grade serous carcinoma and low-grade serous carcinoma are very different tumors and pathologists should state whether it is high grade or low grade. Please make every attempt to use the newer codes. If unable to determine high gade versus low grade, assign 8441/3 and override the edit.

The files on the CPC website are currently being updated, and CPC*Search will be updated to reflect the changes sometime this Fall.

For cases diagnosed 2007 or later, accession two primaries, carcinosarcoma [8980/3] of the left ovary and serous carcinoma [8441/3] of the right ovary.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). After determining the histology of each tumor (8980/3 and 8441/3), go to the Other Sites MP rules because ovary does not have site specific rules developed

Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. Stop at the first rule that applies to the case you are processing.

Review Table 1 (Paired Organs and Sites with Laterality) to determine whether ovary is a paired site. To locate Table 1, go to Other Site under the Terms & Definitions section of the manual. Ovary is listed as a paired site. Accession multiple primaries when there are tumors on both sides (right and left) of a site listed in Table 1 (Paired Organs and Sites with Laterality).

Carcinosarcoma [8980/3] is not an epithelial tumor of the ovary within the range of 8000-8799 and, therefore, Rule M7 does not apply.

Abstract this case as a single primary. Code as 8044/3 (small cell carcinoma, hypercalcemic type) listed in the Other Sites Solid Tumor Rules, Table 13. Small cell carcinoma, large cell variant, is a subtype of small cell carcinoma, hypercalcemic type. This table does not include all possible histologies.

WHO Classification of Female Genital Tumors, 5th edition, states: Small cell carcinoma of the ovary, hypercalcemic type, is rare, accounting for < 1% of ovarian tumors. Small cell carcinomas, hypercalcemic type, are usually large, with a mean size of 15 cm (range: 6–26 cm). Large cells are present (in varying numbers) in half of these tumors, which are designated “small cell carcinoma, large cell subtype” if the large cells are predominant (which is rare).

Assign code 8461/3 for high-grade papillary serous carcinoma.

Based on the information provided, this case of adult granulosa cell tumor is malignant and reportable. According to our expert pathologist consultant, "though granulosa cell tumor NOS/ adult NOS is 8620/1, the presence of peritoneal implants or metastases, and/or lymph node metastases indicates the tumor is malignant, and it should be coded /3."

Note that the presence of implants or metastases does not indicate malignancy in the case of low malignant potential ovarian epithelial tumors. Our path expert explains "in contrast, by convention the behavior of borderline/LMP ovarian epithelial tumors is determined by the ovarian primary, and is /1, even though there may be peritoneal implants/metastases, or metastatic disease in lymph nodes. The treatment may vary in these circumstances, but to my knowledge the decision as to the tumor designation remains based on the primary tumor."

According to the MP/H rules, code histology to 8010/2 [intraepithelial carcinoma] for cases diagnosed 2007-2014. Borderline mucinous tumor is not reportable to SEER.

The steps used to arrive at this decision are:

Go to the Other Sites Histo rules found in the Multiple Primary and Histology Coding Rules Manual.

Start at the SINGLE TUMOR: IN SITU ONLY module, rule H1. Code the histology when only one histologic type is identified. The only reportable histology in this case is intraepithelial carcinoma [8010/2].

For tumors diagnosed prior to 2007:

Code histology to 8310/3 [Clear cell adenocarcinoma, NOS]. This is consistent with the WHO Classification of Tumours and reflects the current practice of placing less emphasis on "cyst-" prefix for ovarian malignancies.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.