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

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (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 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.

Code to mucinous (8480) since that is the only clearly invasive component of this diagnosis.

According to our pathologist consultant, "Encysted papillary carcinoma is the same thing as intracystic papillry carcinoma, which I think of as an intraductal papillary carcinoma which has greatly expanded the duct to form a cyst-like structure. It generally behaves in an in-situ rather than an invasive fashion. The only clearly invasive component is the mucinous carcinoma, which is what I would code."

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.

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

No. Per Appendix F, erythrocytosis of an unknown cause is not reportable.

The diagnosis must state "erythrocytosis megalosplenic" to be reportable (9950/3).

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.

This is accessioned as one primary and the histology is coded to 9732/3 [multiple myeloma].

To arrive at this answer, it is important to first try to determine how many different unique neoplasms there are to correctly identify the number of primaries to report. Per the Heme DB, plasma cell leukemia is an obsolete term. The current term and histology code for this diagnosis is 9732/3 [plasma cell myeloma]. Plasma cell myeloma and multiple myeloma are synonyms per the Heme DB. Therefore, per Rule M2 a single primary exists when there is a single histology. That takes care of the multiple myeloma/plasma cell myeloma and plasma cell leukemia, but not the plasmacytoma.

In checking the Heme DB, the terms plasma cell myeloma and multiple myeloma are not synonyms for plasmacytoma. Therefore, we are left to determine whether the multiple myeloma/plasma cell myeloma vs the plasmacytoma represents one or two primaries.

Under the Transformation section of the Heme DB, it indicates that plasmacytoma (a chronic disease process) transforms to multiple myeloma (an acute disease process). Per Rule M9, abstract a single primary and code the acute histology when both a chronic and an acute neoplasm are diagnosed simultaneously. The histology is coded to the acute neoplasm when there is no information on the biopsy regarding which is the "later" histology. This update will be added to the Heme 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 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Thrombocytopenia NOS is not a reportable diagnosis per Appendix F. Thrombocytopenia and Refractory Thrombocytopenia are not the same disease.

Thrombocytopenia is caused by a decreased number of platelets in the blood. Non-malignant causes include disseminated intravascular coagulation (DIC), drug-induced non-immune thrombocytopenia, drug-induced immune thrombocytopenia, hypersplenism, immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura, and infections of the 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.

For tumors diagnosed prior to 2007:

There are two primaries in this example:

1. Invasive lobular carcinoma [8520/3]

2. In situ Paget disease of nipple [8540/2].

There is no combination code for lobular carcinoma and Paget disease.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

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.

This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.Seeding, talcum powder appearance, salting and studding are synonymous with implants. When the size of implants is not stated, but operative report and scans state "seeding," "talcum powder appearance," "salting," and "studding" the CS extension code choice will depend on the location of the seeding, talcum powder appearance, salting, or studding.

The word "miliary" is not documented as a synonym for implants.

The term miliary does not affect the CS extension code choice according to the current CS instructions.