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

Code the primary site to C77.8 [multiple lymph node regions, NOS].

Per Rule PH6, code the primary site to the involved lymph node region(s) when there is no bone marrow involvement or when it is unknown whether the bone marrow is involved. To determine the more specific lymph node subsite to code, use Rule PH21. It indicates one is to code the primary site to C778 [multiple lymph node regions, NOS] when multiple lymph node regions, as defined by the ICD-O-3 (see Table C1: Lymph Node/Lymph Node Chain Reference Table in Appendix C), are involved and it is not possible to identify the lymph node region where the lymphoma originated.

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 single primary, essential thrombocythemia [9962/3] diagnosed in 2007. The 2010 Heme DB and Manual should not have been used to determine the number of primaries in this case. The Heme DB applies only to cases diagnosed 2010 and later.

In order to determine the number of primaries, use the rules in place at the time of the subsequent 2009 diagnosis of primary myelofibrosis. Per the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, a diagnosis of essential thrombocythemia [9962/3] followed by a diagnosis of primary myelofibrosis [9961/3] is a single primary.

For cases diagnosed 2007 or later, accession two primaries, ductal with apocrine features in the left breast and lobular carcinoma in the right breast.

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). Go to the Breast MP rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. The patient has tumors in both the right and left breasts.

Rule M6 does not apply because inflammatory carcinoma involves only the left breast and the patient has a different histology in the right breast and there is no mention of inflammatory carcinoma in that breast. In this situation continue to the next applicable rule.

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.

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 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 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 rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.

In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).

Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.

The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.

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 the purpose of applying the MP/H rules, the term "synchronous" means that the two diagnoses occurred at the same time or less than or equal to 60 days apart.

The case in SINQ 20100025 was not synchronous. The first lesion in the renal pelvis [C65.9] occurred in 1/08 and the subsequent tumors were diagnosed in 5/09, more than one year apart. In this case, you do not go back to change the primary site code on the original abstract.

The case in SINQ 20110119 was diagnosed synchronously, the first lesion in the bladder [C67.9] was diagnosed in 11/09 and the second lesion in the renal pelvis [C65.9] was diagnosed in 12/09, less than 60 days apart. Because the lesions were synchronous, the primary site is coded urinary system, NOS [C68.9].

This diagnosis is not reportable. Cystadenoma is not reportable. The "in situ" description in this case does not make cystadenoma reportable.

According to our expert pathologist consultant, this is a "non-invasive, low grade, epithelial proliferation in an often cystic appendiceal tumor, 8480/1. If this has leaked or ruptured it can seed the peritoneal cavity causing pseudomyxoma peritonei."

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].