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The behavior code for hemangioblastoma can be coded to /3 when a pathologist indicates that the behavior is malignant. The behavior code should be based on a pathologist's opinion. It is usually not possible for a radiologist or patient care physician to make this determination clinically.

The histologic appearance of hemangioblastoma may resemble metastatic renal cell carcinoma; therefore, one will often see renal cell carcinoma listed as a possible diagnosis. This does not indicate that the hemangioblastoma is malignant. Do not code the behavior as /3 based on a differential diagnosis of renal cell carcinoma.

Assuming the same primary site for the 2008 lesion, according to the current MP/H rules the high grade fibrosarcoma [8810/3] is a new primary per Head & Neck MPH rule 11 because it is a different histology.

The revised MP/H rules will include tables to define tumors that de-differentiate (transform) and recur with what is seemingly a different histology. Although the rules will be changed in the future, we must use the rules in place at this time for this case.

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 hard-to-treat diseases such as DLBCL, the treatment plan outlined prior to treatment beginning may indicate, "The first course of treatment will be radiation and R-CHOP. If the R-CHOP does not achieve remission, we will use RICE." In other words, the first course treatment plan includes a second round of chemotherapy if the patient has not achieved a complete response after the R-CHOP and radiation. If the treatment plan was documented like this for the patient, the first course treatment includes R-CHOP, involved field radiation and RICE.

However, if there is no initial treatment plan in the medical record, all treatment provided after the date when "residual disease" or "failed to achieve remission" is documented in the medical record is either second or a subsequent course of therapy.

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

This is a single primary and the primary site and histology is coded as bone marrow [C421] and CLL/SLL [9823/3]. The code 9670/3 [malignant lymphoma, small B lymphocytes, NOS] used for SLL is now obsolete.

Per the Abstractor Notes section in the Heme DB indicates that SLL is, "usually associated with CLL and coded CLL/SLL 9823/3. Small lymphocytic lymphoma (SLL) is almost identical to CLL. A somewhat arbitrary distinction is drawn between them based on the relative degree of marrow and nodal involvement and the numbers of circulating cells."

Per the Definition section in the Heme DB it states that, "CLL by definition involves blood and bone marrow at time of diagnosis." Check the PRIMARY SITE and MODULE RULE sections that indicate the primary site is C421, Rule PH5. Per this rule, code the primary site bone marrow (C421) and code the histology B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) [9823/3] when the diagnosis is B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) AND peripheral blood is involved (the bone marrow may also be involved).

This may appear to contradict the physician's diagnosis, but the 2008 WHO no longer codes CLL and SLL as separate neoplasms, rather one neoplasm, CLL/SLL, which reflects the actual neoplastic process. Those patients with SLL usually manifest CLL during the neoplastic process and those patients with CLL usually manifest SLL during the neoplastic process. WHO recommends coding to CLL/SLL rather than coding two primaries when the other neoplasm manifests.

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.

The following answers depend on what this pathologist means by "ductal (tubular)." According to the WHO classification, tubular is not a duct subtype. Check with the pathologist if possible to determine if the intended meaning is "tubular carcinoma" or "duct carcinoma".

If the pathologist uses the expression "ductal (tubular)" as an equivalent of "tubular carcinoma": Accession two primaries, a tubular carcinoma [8211/3] and a ductal carcinoma in situ, solid type [8230/2].

For cases diagnosed 2007 and later, the steps used to arrive at this decision are:

Determine the provisional histologies of these tumors in order to apply the Multiple Primary rules. Open the Multiple Primary and Histology Coding Rules manual. For a breast primary, use the Breast Histology rules to determine the histology codes because there are site specific rules for breast primaries.

Determine the histology of in situ carcinoma, solid type ductal carcinoma in situ. Start at Rule H1. The rules are intended to be reviewed in consecutive order within the applicable Module. Code the more specific histologic term when the diagnosis is intraductal carcinoma and a type of intraductal carcinoma. Solid is a specific type of DCIS. The histology is 8230/2.

Determine the histology of the invasive carcinoma, tubular carcinoma. Start at Rule H10. Code the histology when only one histologic type is identified, Tubular carcinoma was the only type identified. The histology is 8211/3.

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual after determining the histology of each tumor. Start at the MULTIPLE TUMORS Module, Rule M4, because the patient has a single invasive tumor and separate foci of DCIS. These tumors have ICD-O-3 histology codes that are different at the third (xxx) number and are, therefore, multiple primaries.

If the pathologist uses the expression "ductal (tubular)" as an equivalent of "duct carcinoma": Accession a single primary, a duct carcinoma [8500/3].

For cases diagnosed 2007 and later, the steps used to arrive at this decision are:

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS Module, Rule M4 because the patient has a single invasive duct carcinoma and separate foci of solid type ductal carcinoma in situ. Multiple intraductal and/or duct carcinomas are a single primary. Table 1 identifies solid type as a specific type of intraductal carcinoma.

Go to the Breast Histology rules found in the Multiple Primary and Histology Coding Rules Manual. Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY Module, Rule H20. Code the invasive histology when both invasive and in situ tumors are present. Code the histology as 8500/3 [duct carcinoma].

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.

Urine cytology positive for malignancy is reportable. Code the primary site to C689 in the absence of any other information.

However, if a subsequent biopsy of a urinary site is negative, do not report the case.

For 2013 diagnoses and forward, report these cases when they are encountered. Do not implement new/additional casefinding methods to capture these cases.

As always, do not report cytology cases with ambiguous terminology.

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

Myelodysplastic disorder is a synonym for myelodysplastic syndrome (MDS). If no further workup is done or no additional information can be found, code the histology of myelodysplastic disorder to 9989/3 [MDS] for cases diagnosed 1/1/2010 and later.

Refer to the Abstractor Notes section in the Heme DB, Abstractor Notes for MDS. Myelodysplastic (disorder) syndrome is a NOS term. Usually when this diagnosis is made, the physician will conduct further tests to determine a more specific disease in the Myeloproliferative Neoplasms group. Other specific histologies include: refractory anemia with unilineage dysplasia, refractory anemia with ring sideroblasts, refractory cytopenia with multilineage dysplasia, refractory anemia with excess blasts, myelodysplastic syndrome with del(5q), childhood myelodysplastic syndrome. If a more specific disease is diagnosed, code to that specific neoplasm.

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.