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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.

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.

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 one primary. Per Rule PH26, code the primary site to bone marrow (C421) when lymphoma is present only in the bone marrow. (We assumed all available physical exams, scans, and other work-up were negative for lymph node, tissue, or organ involvement.) Histology is coded to 9680/3 [Diffuse large B-cell lymphoma (DLBCL)]. Under the Alternate Names section of the Heme DB, a synonym for DLBCL is B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma.

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 reportable case. Even though the pathology from the orchiectomy stated mature teratoma, NOS, the presence of lymph node metastases proves that this tumor is malignant. Code the histology as 9065/3 [germ cell tumor non-seminomatous].

The majority of germ cell tumors show the presence of multiple histologies. While the original tumor showed only mature teratoma, there were obviously yolk sac cells that were not detected on the sections taken from the primary tumor. Both teratoma and yolk sac are germ cell tumors. This explains why the pathologist gave you the diagnosis of germ cell tumor. The classification of "non-seminomatous" simply means that there was no seminomas present in the mixture of germ cell histologies.

For cases diagnosed 2007 or later:

Endometrioid adenocarcinoma with squamous differentiation is coded to 8570 [Adenocarcinoma with squamous metaplasia].

The following row needs to be added to Table 2 in order to be able to correctly use the MP/H rules to reach this conclusion.

Column 1:   Endometrioid adenocarcinoma

Column 2:   Squamous metaplasia

                    Squamous differentiation

Column 3:   Adenocarcinoma with squamous metaplasia

Column 4:   8570

The change will be made in the next revision of the rules.

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

Macrocytic anemia is not reportable. Anemia refers to a condition of having a low count of red blood cells. The term "macrocytic" refers to the enlarged size of the red blood cells. Macrocytic anemia is usually caused by vitamin deficiencies, alcohol use, medications or thyroid disorders.

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.

Record the neoadjuvant therapy only for the first primary and do not record the neoadjuvant therapy for the incidental new primary found on surgery. 

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: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

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.

Per Rule M10, this case should be accessioned as two primaries when a neoplasm is originally diagnosed as a chronic neoplasm (is follicular lymphoma (FL), grade 2) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.

Code the histology for the first primary to 9691/3 [follicular lymphoma (FL), grade 2] and the primary site to bilateral C509 [breast, NOS]. FL can start as an extranodal disease; breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes.

Per Rule M4, abstract the 2010 disease process as a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Per Rule PH11 the primary site is coded to C779 [lymph nodes, NOS] and the histology is coded to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Rule PH11 states one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

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.