SEER Inquiry System - Search

Abstract as a single primary: dermatofibrosarcoma protuberans [8832/3] of the left upper inner arm [C446] diagnosed in August 2008.

The rationale for this answer was provided by subject matter experts. The physician specialists for soft tissue and bone replied as follows:

Low-grade sarcomas tend to recur locally. Because this tumor recurred in same area, i.e. scar of prior surgery, and recurred in this period of time, this is a local recurrence. Dermatofibrosarcoma Protuberans is a low grade tumor which can recur many years following tumor excision.

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

This case should be accessioned as two primaries. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.

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 2021

According to our expert pathologist consultant, code using the terms in the pathology report. Do not try to second guess the pathologist.

1. Thymomas are /1 and not reportable unless the pathologist states "malignant". This includes A, AB, B1, B2, and B3. Any one of these types of tumors may exhibit invasion but do not code /3 based only on invasion.

This is a single primary, epithelioid sarcoma of the left upper limb, diagnosed in 2003. The sarcoma progressed over the years and the patient was never free of disease -- positive margins were documented at each surgical event. Per the 2004 SEER Manual coding rules in place at the time of pre-2007 recurrences, they would not be multiple primaries according to Rule 5, exception 1.

The occurrence in 2010 is also not a new primary. The steps used to arrive at this decision are as follows.

Open the Multiple Primary and Histology Coding Rules manual. For a soft tissue primary, use one of the three formats (i.e., flowchart, matrix or text) under the Other Sites MP rules to determine the number of primaries because soft tissue primaries do not have site specific rules.

Start with the UNKNOWN IF SINGLE OR MULTIPLE TUMORS module, Rule M1. The rules are intended to be reviewed in consecutive order within the module that applies for this case. In this module there is only one rule.

. This patient was never disease free and it is unknown if this tumor was the same tumor (single tumor) or multiple tumors. Abstract a single primary for this patient.

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

No. Polycythemia, NOS is not reportable.

Polycythemia (also known as polycythaemia or erythrocytosis) is a disease state in which the proportion of blood volume that is occupied by red blood cells increases. Blood volume proportions can be measured as hematocrit level. It can be due to an increase in the mass of red blood cells, "absolute polycythemia"; or to a decrease in the volume of plasma, "relative polycythemia".

The phlebotomy is a treatment for the excessive blood volume; therefore, a diagnosis of "polycythemia" without one of the modifying terms listed in the Heme DB under Alternative Names is not reportable.

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..

This should be accessioned as a single primary per Rule M8 which states to abstract as a single primary and code the acute neoplasm when both a chronic (MDS) and an acute (AML) neoplasm are diagnosed simultaneously or within 21 days AND there is documentation of only one positive bone marrow biopsy, lymph node biopsy, or tissue biopsy. Code the histology to 9895/3 [acute myeloid leukemia with myelodysplasia-related changes].

NOTE: When you search with quotation marks around the phrase, the database will only return results with that exact wording. To only return results for the expression trisomy 13, enter in the Heme DB. In this case, a search for "trisomy 13" returns no results. Therefore, it does not impact the coding of histology for this case.

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.

This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm.

Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3].

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 9837/3 [adult T cell leukemia/lymphoma] and the primary site to C778 [lymph nodes, multiple regions]. Per Rule PH8, code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. A statement of "mediastinal mass" and lymphadenopathy for lymphoma primaries is equivalent to lymph node involvement. To identify the more specific primary site, you need to move to Rule PH21 that indicates you are to code the primary site as multiple lymph node regions, NOS [C778] when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated.

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.