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Epithelioid angiomyolipoma (AML) [8860/0] of the kidney is not reportable unless stated to be malignant.

If the pathologist confirms this is a malignancy, apply ICD-O-3 Rule F (Matrix principle) and assign the behavior code /3. If confirmation is received, accession the case using the morphology code 8860/3 [malignant angiomyolipoma].

For cases diagnosed 2007 or later, code the primary site to ovary [C56.9] and the histology to papillary carcinoma, follicular variant [8340/3].

The steps used to arrive at this decision are:

Refer to the 2012 SEER Manual for help to determine the primary site. This neoplasm is arising in a teratoma of the ovary. Per the 2012 SEER Manual, in this case the site is coded to ovary [56.9] because that is where the tumor originated. Although the teratoma contains thyroid tissue, it arose in the ovary. Teratomas are unusual in that they contain all three germ cell layers from which an embryo forms. It is not unusual to have malignancies that are usually primary to the thyroid, liver, brain, lung, etc., originate in a teratoma.

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Other Sites Histology rules because site specific rules have not been developed for this primary.

Start with the SINGLE TUMOR: INVASIVE ONLY module, rule H8. The rules are intended to be reviewed in consecutive order within a module. Code the histology as papillary carcinoma, follicular variant [8340/3].

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

Per the Abstractor Notes in the Heme DB, primary effusion lymphoma (PEL) is unusual in that the majority of cases arise in body cavities, such as the pleural, pericardial, and peritoneal cavities. Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to pleura C384 when the neoplasm arises in the pleural cavity, to pericardium C380 when it occurs in the pericardium, and to peritoneal cavity C482 when it occurs in the peritoneum.

Typically only one body cavity is involved. However, if multiple regions are positive for PEL as in this case, code the primary site to C809 per Rule PH27. Rule PH27 indicates one is to code the to primary site C809 when there is no evidence of lymphoma in lymph nodes AND the physician in the medical record that he/she that the lymphoma in an

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.

Code the primary site to C179 [small intestine, NOS] for multiple invasive tumors of the small intestine accessioned as a single primary.

The steps used to arrive at this decision are:

Step 1: Go to the Primary Site subsection located in Section IV of the 2012 SEER Manual titled "Description of This Neoplasm."

Step 2: Apply instruction 5. "Code the last digit of the primary site code to '9' for single primaries, when multiple tumors arise in different subsites of the same anatomic site and the point of origin cannot be determined." Code the primary site to C179 [small intestine, NOS].

When multiple tumors arising in different subsites are accessioned as a single primary, the primary site is coded to the NOS code, in this case small intestine, NOS [C179]. The level of invasion does not determine the primary site, unless one or more of the tumors is in situ and another is invasive.

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

Secondary polycythemia vera is not reportable. See Appendix F.

Primary polycythemia vera is a condition in which there is an overproduction of blood cells due to a neoplastic process. Secondary polycythemia vera is an over production of red blood cells caused by a co-morbidity, in this case, volume depletion.

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.

Apply rule PH22 to code the primary site to C779 [lymph nodes, NOS].

While the pathology does not indicate that this particular case represents a B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt variant, in the Abstractor Notes section of the Heme DB for diffuse large B-cell lymphoma it does indicate that its presentation, "may have lesions in ileocecal region or jaws. Bone marrow and peripheral blood may be involved. Patients present with lymphadenopathy or mass lesions in extranodal site." This patient does have involvement of two extranodal sites and involvement of regional lymph nodes for only one of those sites.

PH22 indicates one is to code the primary site to C77.9 [lymph nodes, NOS] when lymphoma is present in multiple organs and lymph nodes that are not regional for that organ and the origin cannot be determined even after consulting the physician. There are two extranodal sites of involvement and only one chain of lymph nodes is regional to one of those sites so this rule applies.

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 histology to 9591/3 [B-cell lymphoma, NOS].

After searching the Heme DB for the term , no B-cell leukemia/lymphoma NOS code is found. However, the diagnosis of B-cell lymphoblastic leukemia/lymphoma is found. This case scenario does not specify that this is a lymphoblastic leukemia/lymphoma; therefore, the histology code 9811/3 [B-cell lymphoblastic leukemia/lymphoma, NOS] cannot be applied.

A subsequent search of the Heme DB for the term returns "Non-Hodgkin lymphoma, NOS" [9591/3]. Under the Alternative Names section of the Heme DB, B-cell lymphoma, NOS, is a synonym for Non-Hodgkin lymphoma, NOS. Therefore, the B-cell lymphoma NOS code [9591/3] is the most appropriate histology code to use for this case.

This will be added to the next revision of the Heme DB and Manual.

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 or later, the correct histology code for both cyst associated renal cell carcinoma and cystic renal cell carcinoma is 8316/3. The histology code for cystic renal cell carcinoma, clear cell type is 8255/3.

The steps used to arrive at these decisions are:

Step 1: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Kidney Histology rules because site specific rules have been developed for this primary.

Step 2: For the first histology, cyst associated renal cell carcinoma, start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at Rule H5. According to this rule you are to use Table 1 if you have a renal cell carcinoma and mention of a more specific renal cell type. To locate Table 1, go to Kidney under the Terms & Definitions section. Per Table 1, titled Renal Cell Carcinomas and Specific Renal Cell Types, "cyst associated" is a specific type of renal cell carcinoma. Code the histology to 8316/3 [cyst associated renal cell carcinoma].

Step 3: For the second histology, cystic renal cell carcinoma start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at Rule H5. As in the previous example you are to use Table 1 if you have a renal cell carcinoma and mention of a more specific renal cell type. Per Table 1 "cystic" is a specific type of renal cell carcinoma. Code the histology to 8316/3 [cystic renal cell carcinoma].

Step 4: For the third histology, cystic renal cell carcinoma, clear cell type, start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at Rule H6 which states you are to code histology to 8255 (adenocarcinoma with mixed subtypes) when there are two or more specific renal cell carcinoma types. To determine whether "clear cell" and "cystic" are types of renal cell carcinoma use Table 1 again. According to Table 1, both cystic and clear cell are specific types of renal cell carcinoma. Code the histology as 8255/3 [adenocarcinoma with mixed subtypes].

Mature teratomas in the ovary are benign [9080/0].

For testis, mature teratoma in an adult is malignant (9080/3); however, mature teratoma in a child is benign (9080/0).

With regard to the thyroid issue, from the information above, the medullary carcinoma in the patient's thyroid is clearly a separate event. According to our expert pathologist consultant, "thyroid tissue is one of the many tissue types that may be seen in teratomas. When the teratoma has exclusively or predominantly thyroid tissue the term struma ovarii is used Adenoma or carcinoma of the thyroid type may be seen in this thyroid tissue. If medullary carcinoma were present in the thyroid tissue in the ovary/teratoma, there would be mention of it in the path report."

For cases diagnosed 2007 or later, accession two primaries, carcinosarcoma [8980/3] of the left ovary and serous carcinoma [8441/3] of the right ovary.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). After determining the histology of each tumor (8980/3 and 8441/3), go to the Other Sites MP rules because ovary does not have site specific rules developed

Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. Stop at the first rule that applies to the case you are processing.

Review Table 1 (Paired Organs and Sites with Laterality) to determine whether ovary is a paired site. To locate Table 1, go to Other Site under the Terms & Definitions section of the manual. Ovary is listed as a paired site. Accession multiple primaries when there are tumors on both sides (right and left) of a site listed in Table 1 (Paired Organs and Sites with Laterality).

Carcinosarcoma [8980/3] is not an epithelial tumor of the ovary within the range of 8000-8799 and, therefore, Rule M7 does not apply.