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

First, note that myelodysplastic syndrome (MDS) is a term that includes a number of diseases. Refractory anemia, NOS and refractory anemia with ringed sideroblasts are types of MDS. These two diseases are an NOS and a more specific disease, which is accessioned as one primary per Rule M7.

Next, assess the change from refractory anemia to AML. In checking the Heme DB, AML is listed under transformations for refractory anemia with ringed sideroblasts. This patient has a chronic disease (refractory anemia with ringed sideroblasts) and an acute disease (AML). Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.

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.

This is a single primary. The only rules used to determine the number of primaries are the MP/H rules for cases diagnosed 2007 or later. Do not use other information such as Oncotype Dx to determine the number of primaries for a patient. Oncotype is used to determine whether the cancer is likely to recur AND whether the cancer would benefit from chemotherapy.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. Once in the manual, locate the Breast MP rules under one of the three formats (i.e., flowchart, matrix or text).

Start with the MULTIPLE TUMORS module, Rule M4. The rules are intended to be reviewed in consecutive order within the module from Rule M4 to Rule M13. You stop at the first rule that applies to the case you are processing.

The patient has two tumors in the same breast with the same histology. Abstract a single primary for this patient.

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

Idiopathic and autoimmune types of thrombocytopenia are not reportable. Thrombocytopenia and thrombocythemia are not synonyms. Cytopenia and cythemia have different definitions. See Appendix F: Non-Reportable List for Hematopoietic Diseases.

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 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.

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

The PH rules for coding lymphomas (Module 7) refer to a lymph node region as defined by the ICD-O-3. Per the Appendix C, , the ICD-O-3 lymph node region for "pelvic" is C775. In this case, there is one lymph node region involved (bilaterally). Per Rule PH20, code the specific lymph node region when multiple lymph nodes within the same lymph node region (as defined by the ICD-O-3) are involved, C775. Per Note 1 under Rule PH20, use this rule when there is bilateral involvement of lymph nodes.

This same table in Appendix C also provides information on how left and right pelvic lymph nodes are categorized by AJCC for purposes of coding stage. If the left and right pelvic lymph nodes are positive for lymphoma, it is involvement of two regions. The case is coded as Stage II.

Keep in mind that the ICD-O-3 definition of regions is used to code the primary site, while the AJCC definition of regions is used to code stage.

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.

Multiplicity Counter field was initially coded 02. Change the code to 03 because the subsequent, additional tumor was determined to be the same primary. Update the Multiplicity Counter field only once. If additional tumors are determined to be the same primary for this case, it is not necessary to update this field again.

Date of Multiple Tumors field was initially coded November 2007. Multiple tumors were present at the time of the initial diagnosis. Do not change the date of this field when additional tumors are subsequently diagnosed. This data item reflects the earliest date that multiple tumors were present. See example 2 under #3 on page 81 of the 2010 SEER manual.

For hard-to-treat diseases such as DLBCL, the treatment plan outlined prior to treatment beginning may indicate, "The first course of treatment will be radiation and R-CHOP. If the R-CHOP does not achieve remission, we will use RICE." In other words, the first course treatment plan includes a second round of chemotherapy if the patient has not achieved a complete response after the R-CHOP and radiation. If the treatment plan was documented like this for the patient, the first course treatment includes R-CHOP, involved field radiation and RICE.

However, if there is no initial treatment plan in the medical record, all treatment provided after the date when "residual disease" or "failed to achieve remission" is documented in the medical record is either second or a subsequent course of therapy.

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.