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Default the coding of the Primary Site field for chordomas to the bone where the tumor began in the body if the primary site is not clearly stated to be soft tissue. Bone is often the primary site for chordomas.

Based on advice from pathologist consultants: This is one of those situations where we can be quite comfortable with a default, in this case to bone, not soft tissue. Chordoma is a tumor arising in the nucleus pulposis, presumably from remnants of notochord - thus its exclusive origin is in the sacrococcygeal region, spheno-occipital region, and vertebral bodies, otherwise known collectively as the axial skeleton. Any "chordoma" in soft tissue (with no relationship to axial skeleton) is probably a myxoid chondrosarcoma or parachordoma (extremely rare).

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 1998-2002:

Code the Reason for No Cancer-Directed Surgery field to 1 [Cancer-directed surgery was not recommended] for patients who present with either a primary site or histology for which surgery is not a standard treatment. Also use code 1 for those patients who present with distant disease for a primary site that is typically treated surgically. Patients with distant metastasis typically do not have surgery performed as part of first course of treatment.

Code 2 [Contraindicated due to other conditions] is used when surgery would normally be recommended for the site (given the current stage of the tumor) but other medical conditions pose too much of a risk for the patient to undergo surgery.

Many of these terms are not specified in the codes and definitions in ICD-10-CM. This is because ICD-10-CM does not have the same granularity as ICD-O-3.2. There are a few sites where intraepithelial neoplasia II and/or III are mentioned.

Even though ICD-O-3.2 classifies these as /2 (in-situ), for the intraepithelial neoplasia that are listed in ICD-10-CM, Grade II is designated as benign, while Grade III is designated as in-situ. It is not clear if medical coding will change the Grade II to an in-situ code.

All the in-situ codes (except cervix) are included in the casefinding list. Grade III is included with the in-situ codes; however, there is no guarantee that medical coders will code them as in situ. High grades are coded as in-situ in ICD-10-CM.

For those where there is no specific intraepithelial neoplasia code, the benign codes will cover any benign lesion for that site. This would make for a lot of review using the codes for casefinding. Most of the benign codes were removed from the casefinding list a couple of years ago to make it more manageable. 

Use the casefinding list as a guide for these neoplasias. It is not the most definitive source due to the lack of specificity of ICD-10-CM. It is not possible to map every single histology to a specific code. It is also not known how medical coders across the U.S. are coding these neoplasias. For that reason, pathology should remain the foremost casefinding resource used.

The casefinding team will need to review the prepared list below and determine what codes to add. Any updates will be incorporated in the FY2025 updates (October 2024.)

For cases diagnosed 2003 and later:

Assign surgery codes 45, 46 and 47 only when the margins are documented to be more than 1cm. Use the most appropriate code from 30-36 if re-excision or wide excision followed a biopsy. Use a code from the 20's series if the procedure is called a "biopsy."

Code Hormone therapy as 01 [Hormone therapy administered as first course therapy] when thyroid replacement therapy is part of the first course of treatment for follicular or papillary thyroid cancer following thyroidectomy.

Thyroid hormone replacement therapy has a treatment effect on differentiated (follicular and papillary) carcinomas of the thyroid. This treatment effect is not seen for most medullary and undifferentiated thyroid cancers.

Code "embolization" (or hepatic artery embolization, HAE) to a metastatic site in Surgical procedure of Other Site. Assign code 1 [nonprimary surgical procedure performed].

This procedure was previously coded as other therapy, experimental. Code as surgery as of July 2005.