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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.

Per Appendix F, monoclonal B-lymphocytosis of uncertain significance (MLUS) is not reportable.

Some papers point out that a lymphocyte count less than five thousand is equivalent to monoclonal B-lymphocytosis of uncertain significance (MLUS) or monoclonal B-cell lymphocytosis (MBL). A lymphocyte count of five to thirty thousand could be smoldering chronic lymphocytic leukemia (CLL). The diagnosis of MLUS is a benign process that does not meet the criteria for CLL.

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 primary site to C119 [nasopharynx] per Rule PH25.

Per our subject matter expert, use Module 7 Rule PH25 to code the primary site to an organ (nasopharynx and maxillary sinus) because an organ(s) and its regional lymph nodes are involved. The distant lymph nodes are simply part of the staging (the lymphoma has progressed to another lymph node region).

Diffuse large B cell lymphoma originating in the oral cavity and maxillofacial region is rare, but documented. The most common sites for this rare neoplasm are Waldeyer ring, tonsils, nasopharynx, base of tongue, and palatine tonsil. There are also rare cases of diffuse large B cell lymphoma originating in the maxillary sinus. The percentage of cases arising in the nasopharynx is greater than those originating in the maxillary sinus.

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 grade to 6 [B-cell] for the histology malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant [9680/3]. Under the Definition section for histology code 9680/3 it states there are morphologic variants of the disease: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.

Rule G3 in the Heme Manual confirms the grade listed in the Heme DB under its Grade section for the histology 9680/3. While the patient presented with a variant of DLBCL that is T-cell/histiocyte rich, it is still a B-cell phenotype. The grade is coded accordingly.

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, accession two primaries, ductal carcinoma with mucinous differentiation [8523/3] and ductal carcinoma, NOS [8500/3]. 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 have been developed for this primary.

Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. These tumors have ICD-O-3 histology codes that are different that the third (xxx) digit and are, therefore, multiple primaries.

Ductal carcinoma with mucinous differentiation is not a specific type of ductal carcinoma identified in either Table 1 or 2. (To locate Tables 1 and 2, go to Breast under the Terms & Definitions section of the manual.) It is ductal carcinoma mixed with another type of carcinoma (mucinous carcinoma in this case) see Table 3. Rule M11 does not apply.

Code the primary site to main bronchus [C340].

Primary small cell carcinoma in the thymus/mediastinum is rare. A bronchial lesion with extension into the mediastinum is much more likely. In a case like this, it is difficult to be sure exactly where the tumor arose, however, it is recommended the default site be the main bronchus when there is no information to the contrary.

For cases diagnosed 2007 or later, code the histology as lobular carcinoma, in situ [8520/2].

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 Histo rules because site specific rules exist for this primary.

Start at the SINGLE TUMOR: IN SITU CARCINOMA ONLY module, Rule H1. The rules are intended to be reviewed in consecutive order. Stop at the first rule that applies to the case you are processing. Code the histology to lobular carcinoma in situ [8520/2] because this is the only histologic type identified.

Pleomorphic lobular carcinoma is a variant of lobular carcinoma which does not have an ICD-O-3 code. It is still a lobular carcinoma. The identification of the variants of lobular carcinoma was a relatively recent discovery and the information was not available when the 2007 MP/H Rules were written. All of the lobular variants will be included in the next revision of the MP/H Rules.

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

This case is accessioned as a single primary. Code the histology to 9680/3 [diffuse large B-cell lymphoma] and diagnosis date to 2004. Per Rule M2, abstract as a single primary when there is a single histology.

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.