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Do not report angiomyxoma. ICD-O-3.2 assigns 8841/0 to this benign tumor. This includes superficial and deep (aggressive) angiomyxoma.

Assign code 8825/3. Apply the ICD-O-3 Matrix Concept, Rule F, page 29 of the hardcover ICD-O-3. The WHO Classification of Soft tissue and Bone, page 85, lists low grade myofibroblastic sarcoma, also called myofibrosarcoma, 8825/3.

For cases diagnosed 1998-2003:

No. Never code the aggregate size in the Size of Primary Tumor field when the pieces removed come from TWO OR MORE tumors. If there is a clinical statement regarding the size of two or more tumors, code this field to the size of the largest tumor.

The aggregate size can only be used to code the Size of Primary Tumor field when the PATHOLOGIST estimates the size of the tumor from the pieces of ONE tumor removed by the surgeon.

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: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.

This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.

Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.

Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.

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 1998-2003:

Code the EOD-Extension field to 32 [30 + involvement of the spleen; III S]. The spleen is counted twice (once as the spleen and a second time as a lymph node region below the diaphragm). As a result, the EOD-Extension field is coded to reflect involvement of lymph node regions on both sides of the diaphragm plus involvement of the spleen. See Note 1 on the EOD scheme that states "Any lymphatic structure is to be coded the same as a lymph node region."

For tumors diagnosed prior to 2007:

The best code is 8381/3 [Endometroid adenofibroma, malignant]. According to our pathologist consultant: "Serous 'fibroadenoma' is not exactly standard terminology. I would guess the pathologist is looking at an adenofibroma with more fibro and less adeno and thus has changed the terminology around. The combination of the benign serous and malignant edometrioid is also a bit unusual. Each of the proposed codes is defendable, but I prefer endometrioid adenofibroma, 8381/3, because it puts the tumor in the adenofibroma category (less common) yet still identifies the malignant element (endometrioid), even though it does lose the serous. But anyone wanting to look at malignant adenofibromas would find the case, and we would carry it under the appropriate malignant component."

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.

Default the coding of the Primary Site field for chordomas to the bone where the tumor began in the body if the primary site is not clearly stated to be soft tissue. Bone is often the primary site for chordomas.

Based on advice from pathologist consultants: This is one of those situations where we can be quite comfortable with a default, in this case to bone, not soft tissue. Chordoma is a tumor arising in the nucleus pulposis, presumably from remnants of notochord - thus its exclusive origin is in the sacrococcygeal region, spheno-occipital region, and vertebral bodies, otherwise known collectively as the axial skeleton. Any "chordoma" in soft tissue (with no relationship to axial skeleton) is probably a myxoid chondrosarcoma or parachordoma (extremely rare).

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.Assign CS Extension code 15 [Invasive tumor confined to subepithelial connective tissue (tunica propria, lamina propria, submucosa, stroma)]. The information provided confirms invasion of the lamina propria (code 15) but is not definitive enough to assign a code higher than 15.

For tumors diagnosed prior to 2007:

Code the Histology field to 8522/3 [infiltrating duct and lobular carcinoma] per rule 1 of the Coding Complex Morphologic Diagnoses, because the tumor is both lobular and ductal.

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.

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).