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For cases diagnosed 1998-2003:

If only the extramedullary site is involved, such as colon, code the Primary Site field to the site of origin. Granulocytic or myeloid sarcoma is an exception to the rule that all leukemias should be coded to bone marrow as the primary site. Granulocytic sarcoma is a deposit of malignant myeloid cells in a site other than bone marrow (extramedullary). For EOD staging, granulocytic sarcoma [9930/3] is included in the Hematopoietic, Reticuloendothelial, Immunoproliferative and Myeloproliferative Neoplasms scheme and the Extension field is coded to 10 when the lymph nodes are negative, since it (like solitary plasmacytoma) is a localized deposit of tumor.

However, if the regional lymph nodes associated with the extramedullary primary site are involved, code the EOD-Extension field to 80 [Systemic disease] because the disease is no longer an isolated deposit of malignant granulocytes (in other words, it is not localized).

The EOD-Lymph Nodes field is coded to 9 regardless of whether or not the lymph nodes are involved because that is the only allowable code for that field.

Assign primary site C421.

The Site-Specific Data item (SSDI) Manual, Rai Classification section, states: Per confirmation from medical oncologists, Rai stage is only recorded for patients who have bone marrow and/or peripheral blood involvement. Per the Hematopoietic Rules, primary site would be C421 (See Hematopoietic Manual, Module 3: Rules PH 5, 6). A new code has been added to the 5 SSDIs (code 5) to use when primary site is not C421.

For tumors diagnosed prior to 2007:

Code the site to C76.3 [Pelvis, NOS]. Code the histology to 8010/34 [Carcinoma, NOS, high grade].

Unless there is better information available regarding the site, assign C76.3. The information provided above does not indicate the exact site of the mass.

Code the histology based on the consensus opinion stated above.

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.

According to our subject matter expert physician, this unusual case is most likely a sino-nasal tumor (some variant of esthesioneuroblastoma [olfactory neuroblastoma]). Code to nasal cavity [C300] as indicated in ICD-O-3 by site-associated topography code attached to the morphology code for olfactory neuroblastoma [9522/3].

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

Code to primary site to C447 [skin of leg]) per Rule PH25 and histology to 9702/3 [anaplastic large cell lymphoma, ALK-negative]. Per the Abstractor Notes section in Heme DB, these are the usual presentations for this disease. It also states this disease presents with peripheral node involvement and is often generalized with infiltrates in the bone marrow, liver, spleen, and extranodal tissue. Less frequently involved sites are lung, salivary gland and CNS.

Per PH25, code the primary site to the organ when the lymphoma is present in an organ (skin, right leg) and that organ's regional lymph nodes (inguinal). Distant lymph nodes or other organs may also be involved, but should be disregarded for coding primary site.

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 M2, this is a single primary because there is a single histology. Code histology to 9823/3 [CLL/SLL]/

The distinction of CLL vs. SLL cannot be made on bone marrow biopsy in isolation. The pathologist cannot make a diagnosis of CLL vs SLL without having peripheral blood counts available for review. If the patient was treated for CLL in the past, that may alter the peripheral counts seen in 2010 (e.g., lymphocytosis). The distinguishing feature is peripheral lymphocytosis in CLL (not seen in SLL). The disease looks the same and both will often have bone marrow involvement and lymph node involvement. If the patient had true CLL in 2005, then any subsequent lymph node (or other) biopsy consistent with CLL/SLL remains consistent with the original diagnosis of CLL. I would not change the original CLL code.

I agree with the previous response. We have to assume the 2005 diagnosis included a peripheral blood supporting that diagnosis. Otherwise, CLL and SLL look the same in nodes and marrow. The interplay between the two "diseases" is expected. This is why they are considered a single disease.

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 as an oropharyngeal primary site and histology as solitary plasmacytoma (9734/3) based on consultation with our hematological expert.

The WHO Classification of Hematopoietic and Lymphoid Tissues defines multiple myeloma as "bone marrow plasma cell percentage >60%." There are several other factors, but the bone marrow involvement is the key point for your case. The pathologist also states that the bone marrow is consistent with "plasma cell neoplasm," which by itself is not the same as multiple myeloma.

This case has 5-10% involvement by plasma cell neoplasm. This does not meet the bone marrow qualifications for multiple myeloma and is consistent with the pathologist's statement that there is minimal bone marrow involvement.

We will be updating the Hematopoietic and Lymphoid Neoplasms Database and Manual to clarify this (2025 updates).

Assign C221 (intrahepatic bile duct) as the primary site for cholangiocarcinoma (8160/3).  Our expert GI pathologist confirms that even when intrahepatic bile ducts are not specifically mentioned, intrahepatic cholangiocarcinoma originates in the intrahepatic bile ducts.

The CPC Validity Status of the site morphology combinations of C569/8441/3 and C569/8441/2 was revised from Valid to Unlikely with the latest release of the Version v24A Edits Metafile. As a result, this site and morphology combination will now require an over-ride flag to be set.

Code as 8461/3 (high-grade serous carcinoma) or 8460/3 (low-grade serous carcinoma) if at all possible. Use 8441/3 (serous carcinoma, NOS) only if it cannot be distinguished as low grade or high grade. The codes for high-grade serous carcinoma and low-grade serous carcinoma are relatively new. High-grade serous carcinoma and low-grade serous carcinoma are very different tumors and pathologists should state whether it is high grade or low grade. Please make every attempt to use the newer codes. If unable to determine high gade versus low grade, assign 8441/3 and override the edit.

The files on the CPC website are currently being updated, and CPC*Search will be updated to reflect the changes sometime this Fall.

For mesothelioma, NOS (9050) and epithelioid mesothelioma (9052) of the peritoneum for females, assign C481, C482, or C488 as appropriate based on the site of origin in the medical documentation. The Primary Peritoneal Ca schema is assigned and you will need to complete the SSDIs for FIGO staging, CA-125 PreTx Interpretation, and Residual Tumor Volume Post Cytoreduction.

If the histology is 9051 or 9053 with primary site of C481, C482, or C488 for females, the Retroperitoneum schema is assigned. The only SSDI for this schema is Bone Invasion.