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This is a reportable case. Code the histology as malignant gastrinoma [8153/3].

Gastrinomas are usually malignant. This one is apparently present in a metastatic site (periportal lymph node) which confirms the malignancy.

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].

Abstract two primaries for this scenario per Rule M7. The first primary is the renal pelvis in Oct. 2006; the second primary is the ureter in Nov. 2009. The bladder tumor in July 2008 is not a new primary per Rule M8.

Compare the diagnosis date of the current (most recent) tumor to the diagnosis date of the original tumor. This applies even if the patient had six occurrences in-between these dates; you still compare the current tumor to the diagnosis date of the original tumor and ignore recurrences in this process.

See slide 6 of the Beyond the Basics presentation,

http://www.seer.cancer.gov/tools/mphrules/training_adv/SEER_MPH_Gen_Instruc_06152007.pdf.

Accession a single primary, the original ovarian serous carcinoma. The MP/H Rules do not apply to metastases.

Metastases: When cancer cells appear in other nodes or organs that are not the primary site they are metastatic cells. Discontinuous (separate from the primary tumor) masses or cells in regional lymph nodes, distant lymph nodes, or distant sites are always metastases. In this case, the sacral and pelvic masses are distant metastases. The pathologist does not have to compare cells to the original tumor slides; the discontinuous tumor mass/cells in any site other than the primary site are metastases.

Recurrence: For a disease to recur there are several criteria that must be met. First and most important, the patient must have had a disease-free interval (a tumor cannot recur if it has always been present). The other criteria are: the "new tumor" has to occur in the original primary site, it must be the same histology as the original tumor, AND must meet the timing requirements in the MPH rules for that organ/site.

For cases diagnosed 2021 or later, VIN II-III is reportable. Similarly, AIN II-III, VAIN II-III, etc. are reportable. For cases diagnosed 2021 or later, the primary resource for reportability is ICD-O-3.2. Squamous intraepithelial neoplasia, grade II is listed in ICD-O-3.2 as 8077/2 making it reportable. This applies to the various sites of intraepithelial neoplasia grade II including anus, vulva, and vagina.

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].

For cases diagnosed 2007 or later, accession two primaries, serous carcinoma of the right ovary and serous tubal intraepithelial carcinoma of the left fallopian tube based on the information provided.

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) and go to the Other Sites MP rules because neither the ovary nor fallopian tube 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. The patient has multiple tumors with ICD-O-3 topography codes that are different at the third character (Cxx) and therefore this case should be accessioned as a multiple primary.

It could be helpful to know the extent of involvement noted prior to neoadjuvant therapy and debulking surgery. For example, if the patient had widely metastatic disease throughout the entire pelvis prior to the initiation of treatment, the answer may have been different.

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

Per Rule PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney.

The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred more than 21 days after the CLL, it is a new primary per Rule M10.

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 9680/3 [diffuse large B-cell lymphoma (DLBCL)].

For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.

Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.

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 two primaries per Rule M11. Code the histology of one primary to 9680/3 [diffuse large B-cell lymphoma], the acute neoplasm. Code the histology for the second primary to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma], the chronic neoplasm.

Per Rule M11, abstract as multiple primaries when both a chronic and acute neoplasm are diagnosed simultaneously or less than or equal to 21 days apart AND there is documentation of two pathology specimens, one confirming the chronic neoplasm (bone marrow biopsy) and one confirming the acute neoplasm (stomach biopsy).

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.