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

This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm.

Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [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.

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.

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

Per Rule PH30, use the Heme DB to determine the primary site and histology when PH1-PH29 do not apply. Per the Abstractor Notes section in the Heme DB, PTLD commonly involves lymph nodes, GI tract, lungs, and liver. Although CNS involvement is rare, in solid organ recipients the CNS may be the only site of involvement or may be associated with multi-organ involvement. Code the primary site to C719 [brain, NOS] and the histology to 9971/3 [post-transplant lymphoproliferative disorder (PTLD)]

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 tumor in 2010 represents a recurrence; it is not a new primary. This second tumor would be coded as a new primary ONLY if the pathology report states that it originated in breast tissue that was still present on the chest wall. When there is no mention of breast tissue in a subsequent resection, the later occurring tumor is regional metastases to the chest wall (i.e., a recurrence of the original tumor).

In turn, this means that there was at least a focus of invasion present in the original tumor that was not identified by the pathologist. The behavior code on the original abstract must be changed from a /2 to a /3 and the stage must be changed from in situ to localized.

Assuming the same primary site for the 2008 lesion, according to the current MP/H rules the high grade fibrosarcoma [8810/3] is a new primary per Head & Neck MPH rule 11 because it is a different histology.

The revised MP/H rules will include tables to define tumors that de-differentiate (transform) and recur with what is seemingly a different histology. Although the rules will be changed in the future, we must use the rules in place at this time for this case.

SEER will answer the question about the number of primaries to accession. Submit questions about stage to the CoC CAnswer Forum.

For cases diagnosed 2007 or later: Accession two primaries: a mixed adenocarcinoma, acinar and papillary types [8255/3] and a bronchioloalveolar carcinoma, non-mucinous [8252/3].

The steps used to arrive at this decision are:

Determine the histology code for each tumor prior to applying the Multiple Primary Rules to determine the number of primaries to accession. There are two non-small cell carcinomas, NOS; the histology code for these two tumors will be 8046/3. There is a single adenocarcinoma with acinar and papillary subtypes tumor, the histology for this tumor will be 8255. There is a single bronchioloalveolar carcinoma, non-mucinous subtype tumor; the histology for this tumor will be 8252/3.

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Lung Multiple Primary rules to determine the number of primaries.

Start at the MULTIPLE TUMORS module, Rule M3, because this patient has multiple tumors. The rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3] are multiple primaries.

Perform a second pass through the Multiple Primary rules to determine whether the two non-small cell carcinomas [8046/3] are multiple primaries or manifestations of the same primaries identified in Step 3.

Start at Rule M3 again because this patient has multiple tumors. Again, these rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's non-small cell carcinomas, NOS [8046/3] are a single primary when compared to the adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3]. Both of these histologies are more specific types of non-small cell carcinoma per the Lung Histology Groups and Specific Types Chart (Chart 1).

You can also apply Rule M10 for both non-small cell carcinoma, NOS [8046/3] compared to adenocarcinoma with acinar and papillary subtypes [8255/3] and non-small cell carcinoma, NOS [8046/3] compared to non-mucinous bronchioloalveolar carcinoma [8252/3].

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.

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

This case should be accessioned as two primaries. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. 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.

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

This case should not be accessioned. In situ lymphoma is not reportable for any of the standard setters (CoC, NPCR, or SEER). In the Case Reportability Instructions, the NOTE under Rule 3 states, "Do report in situ (/2) lymphomas."

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.