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This case should be accessioned as one primary.

Reportability is determined by the year of diagnosis. The original DLBCL was diagnosed in 2003 and the follicular lymphoma in 2009. The pre-2010 rules are used for both cases. Per the Single Versus Subsequent Primaries Table, these are the same primary. It is reported with the histology 9680/3 [diffuse large B-cell lymphoma]

Do not compare the DLBCL diagnosed in 2003 and the follicular lymphoma diagnosed in 2010 because the determination of the number of primaries for the two specific histologic types was done (as it should have been) using the rules in effect in 2009 when the follicular lymphoma was first diagnosed. The determination of a single or multiple primaries is made the first time the patient presents with the two different diseases; it is not changed when the same disease process reappears after 2010.

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

This is a single primary and the primary site and histology is coded as bone marrow [C421] and CLL/SLL [9823/3]. The code 9670/3 [malignant lymphoma, small B lymphocytes, NOS] used for SLL is now obsolete.

Per the Abstractor Notes section in the Heme DB indicates that SLL is, "usually associated with CLL and coded CLL/SLL 9823/3. Small lymphocytic lymphoma (SLL) is almost identical to CLL. A somewhat arbitrary distinction is drawn between them based on the relative degree of marrow and nodal involvement and the numbers of circulating cells."

Per the Definition section in the Heme DB it states that, "CLL by definition involves blood and bone marrow at time of diagnosis." Check the PRIMARY SITE and MODULE RULE sections that indicate the primary site is C421, Rule PH5. Per this rule, code the primary site bone marrow (C421) and code the histology B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) [9823/3] when the diagnosis is B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) AND peripheral blood is involved (the bone marrow may also be involved).

This may appear to contradict the physician's diagnosis, but the 2008 WHO no longer codes CLL and SLL as separate neoplasms, rather one neoplasm, CLL/SLL, which reflects the actual neoplastic process. Those patients with SLL usually manifest CLL during the neoplastic process and those patients with CLL usually manifest SLL during the neoplastic process. WHO recommends coding to CLL/SLL rather than coding two primaries when the other neoplasm manifests.

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..

Code the primary site to the involved lymph nodes [C770-C779]. Per Rule PH 8, it indicates you are to code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. Note 2 further states that the bone marrow may or may not be involved. If the bone marrow is involved, code this information in the CS Extension field.

Per the Abstractor Notes section in the Heme DB, this is a systemic disease with widespread lymph node involvement as well as involvement of the peripheral blood. In addition, systemic involvement of extranodal sites (including lung) are often involved.

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.

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.

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

Per Rule M2, this is a single primary because there is a single histology. Code histology to 9823/3 [CLL/SLL]/

The distinction of CLL vs. SLL cannot be made on bone marrow biopsy in isolation. The pathologist cannot make a diagnosis of CLL vs SLL without having peripheral blood counts available for review. If the patient was treated for CLL in the past, that may alter the peripheral counts seen in 2010 (e.g., lymphocytosis). The distinguishing feature is peripheral lymphocytosis in CLL (not seen in SLL). The disease looks the same and both will often have bone marrow involvement and lymph node involvement. If the patient had true CLL in 2005, then any subsequent lymph node (or other) biopsy consistent with CLL/SLL remains consistent with the original diagnosis of CLL. I would not change the original CLL code.

I agree with the previous response. We have to assume the 2005 diagnosis included a peripheral blood supporting that diagnosis. Otherwise, CLL and SLL look the same in nodes and marrow. The interplay between the two "diseases" is expected. This is why they are considered a single disease.

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 2007-2014:

Code the Primary Site field to C210 [Anus, NOS]. This is an unusual and rare presentation. According to our expert pathologist, "There is no ideal site code [for] this case. I would code to C210. In this location it can at least be located by anyone who wants to get a look at such lesions. Because of the unusual location of this tumor, I would like to be able to code it to perineum, but it will be totally lost in those site codes as they represent extensive areas beyond perianal (skin of trunk, soft tissue of pelvis, and pelvis, respectively)... I would not code to rectum [because it would be] lost among too many primary rectal carcinomas."

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

Per Rule PH30, use the Heme DB, determine the histology when rules PH1-PH29 do not apply. Code diffuse large B-cell lymphoma, germinal cell type to 9680/3 [diffuse large B-cell lymphoma (DLBCL)][9680/3]. Under the Alternate Names section of the Heme DB, these two terms are synonyms that share the same histology code.

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.