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The site/type edit identifies unlikely combinations of primary site and histologic type.

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

This case should be accessioned as a single primary. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].

The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.

The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."

According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.

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.

Abstract as a single primary: dermatofibrosarcoma protuberans [8832/3] of the left upper inner arm [C446] diagnosed in August 2008.

The rationale for this answer was provided by subject matter experts. The physician specialists for soft tissue and bone replied as follows:

Low-grade sarcomas tend to recur locally. Because this tumor recurred in same area, i.e. scar of prior surgery, and recurred in this period of time, this is a local recurrence. Dermatofibrosarcoma Protuberans is a low grade tumor which can recur many years following tumor excision.

Code the histology to 8082/3 [urothelial carcinoma, plasmacytoid].

The Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later. Unfortunately, in this case there is no current rule that directs you appropriately to Table 1 from Rule H7 to find this histology combination. We need to add an example under Rule H7 that instructs you to "See Table 1" for an urothelial carcinoma diagnosis that mentions a more specific cell type (e.g., plasmacytoid). We will add a reference to Table 1 in Rule H7 in the updates to MP/H Rules.

Histology is sarcomatoid carcinoma [8033/3]. This case was sent to the lung physician experts because of the difficulty in trying to apply the current MP/H rules. Their rationale for the coding decision follows:

"This pathologist has diagnosed a sarcomatoid carcinoma, and then listed all of the subtypes associated with that diagnosis. I would go with the primary diagnosis, sarcomatoid carcinoma. The inclusion of squamous cell differentiation would exclude spindle cell and giant cell as diagnoses, so the pathologist is using them descriptively. We have no basis for picking one of the subtypes and sarcomatoid carcinoma covers all of the diagnoses given."

See the glossary in the Lung Equivalent Terms and Definitions for Sarcomatoid carcinoma: A group of tumors that are non-small cell in type and contain spindle cells and/or giant cells. Depending on the histologic features the tumor may be designated: pleomorphic carcinoma [8022/3]; spindle cell carcinoma [8032/3]; giant cell carcinoma [8031/3], carcinosarcoma [8980/3]; or pulmonary blastoma [8972/3].

For cases diagnosed 2007 or later:

Code 8523/2 [intraductal carcinoma mixed with other types of in situ carcinoma]. Rule H6 should apply to this case.

The wording in the Rule H6 needs to be clarified to handle a case of intraductal carcinoma with one or more subtypes that are not ductal. This will also require a modification to Table 3. A row needs to be added to the table labeled, "Intraductal and one or more of the histologies in Column 2." The Column 3 text for the newly added row would read, " Intraductal mixed with other types of carcinoma." The appropriate histology code to be reported per Column 4 would be 8523/2. This will be done in the next revision of the rules.