SEER Inquiry System - Search

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 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 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 diagnostic confirmation to 8 [clinical diagnosis only] in this case.

Per the Heme DB, JAK-2 is only positive in about 50% of essential thrombocythemia (ET) patients. In addition, a positive JAK-2 test does not identify the type of myeloproliferative disease (MPN) the patient has, only the presence or absence of the JAK-2 mutation.

The WHO guidelines for diagnosing ET are: elevated platelet count over months and the elimination of other causes for an elevated platelet count (such as polycythemia vera (PV), chronic myelogenous leukemia (CML), idiopathic myelofibrosis, or myelodysplastic syndrome (MDS)); the absence of Philadelphia chromosome, BCR/ABL fusion gene; and del(5q), t(3;3)(q21;26),inv(3)(q21q26)).

Subsequently, the physician rules out any underlying causes of thrombocytosis such as an inflammation or infection, other neoplasms, and prior splenectomy.

Ultimately, there is a diagnosis of exclusion. In other words, all other causes for the elevated platelet count have been excluded. The physician assembles the information from the blood counts, bone marrow and JAK-2 testing along with the information that excludes all other diseases and makes a clinical diagnosis of ET.

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 18, code the primary site to C775 [pelvic lymph nodes]. Per Rule PH18, code the primary site to the specified lymph node region when the site of lymphoma is described only as a mass. This rule also indicates that the Code pelvic lymph nodes [C775] when the lymph nodes are described as a pelvic mass.

This rule has been effect for SEER for over 20 years. It is based on the fact that a number of lymphomas that originate in nodes are not diagnosed until those nodes become matted or fixed. The presentation is then one of a "mass" in those nodal regions.

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.

Do not accession the 2011 diagnosis of Langerhans cell histiocytosis. In the Abstractor Notes section of the Heme DB is indicates this is reportable for cases diagnosed 2010 and later. However, this patient was initially diagnosed prior to 2010 when it was not a reportable disease process.

The histology code for Langerhans cell histiocytosis has not changed over time. The histology code for cases of Langerhans cell histiocytosis diagnosed prior to 2010 was also 9751 per the ICD-O-3. The only change since 2010 was in the behavior code for this disease. It changed from borderline [/1] to malignant [/3]. The current disease represents a recurrence of the previous Langerhans cell histiocytosis. Per the Multiple Primary rules, Rule M2, a single histology is a single primary. The original diagnosis was made before the disease was reportable; do not report the disease recurrence or progression as a new primary.

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 a single primary per Rule M15. The histology is coded to 9698/3 [follicular lymphoma, grade 3] diagnosed in 2006. The 2011 diagnosis of follicular lymphoma, grade 2 [9691/3] is not a new primary.

Follicular lymphoma, grade 2 [9691/3] is listed under the Same Primaries section of the Heme DB for 9698/3 [follicular lymphoma, grade 3]. To confirm this, Rule M15 indicates we are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries because none of the rules from 1-14 apply. Per the calculator, these histologies represent the same primary.

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.