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Code as a single primary with histology Diffuse Large B-Cell Lymphoma.

In this case, there are two NHLs in the same location at the same time. Apply Rule M4, this is one primary. Per Note 5 under Rule M4, go to Rules PH11and PH15 to assign primary site and histology.

Rule PH11 states to code to the site of the origin (axillary mass) and to diffuse large b-cell lymphoma (9680/3) when DLBCL and any other non-Hodgkin lymphoma (follicular in this case) are present in the same location at the same time.

Using the multiple primaries calculator in this situation will give you two primaries, which is the wrong answer. Use the rules before using the calculator.

To get to the manual, go to the "Help me code for dx year." section. Choose 2010 or later and the most current manual will appear. We recommend that you save a copy of the PDF on your computer.

For cases diagnosed 2007 or later:

Rule M17 under Other Sites applies. These are separate primaries based on their ICD-O-3 histology codes. Follicular carcinoma is coded 8330. Papillary carcinoma, follicular variant is coded 8340. The histology codes are different at the third number. Rule M6 does not apply because these diagnoses are more than 60 days apart.

Lymphangioma of the brain or CNS is not reportable. Lymphangioma is a malformation of the lymphatic system. Even though it has an ICD-O-3 code, do not report it.

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

Common variable immunodeficiency (acquired hypogammaglobulinemia) is not a reportable condition.

Common variable immunodeficiency represents a group of approximately 150 primary immunodeficiencies that have a common set of symptoms but different underlying causes, both benign and malignant.

The case is not reportable unless this immunodeficiency diagnosis is accompanied by a diagnosis of a cancer or a reportable hematopoietic or lymphoid neoplasm.

See Appendix F: Non-Reportable List for Hematopoietic Diseases.

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 prior to 1/1/2010:

The best code currently available for this entity is 9727/3 [precursor cell lymphoblastic leukemia].

The WHO classification refers to this as "Blastic NK-cell lymphoma." The 2005 WHO-EORTC classification for cutaneous lymphomas states that blastic NK-cell lymphoma may be derived from a plasmacytoid dendritic cell precursor. They suggest more appropriate terms for this condition may be "CD4+/CD56+ hematodermic neoplasm," and "early plasmacytoid dendritic cell leukemia/lymphoma." According to WHO, this is a rare form of lymphoma.

Willemze, et al. WHO-EORTC classification for cutaneous lymphomas. Blood, 15 May 2005. Volume 105, Number 10.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

Yes. Bilateral inguinal lymph nodes are coded as two chains/regions.

For cases diagnosed 1998-2003:

Code the EOD-Extension field to 10 [localized disease, solitary plasmacytoma only] for all cases of solitary plasmacytoma.