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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.

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.

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.

Code the behavior to /3 [malignant, invasive].

"Stromal invasion" means the cancer is invasive. "Stroma" is the supporting connective tissue around and between ducts. It is outside the duct basement membrane. If the tumor cells extend into the stroma, the proper behavior designation for the tumor is invasive.

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.

Use Rule PH21 to code the primary site to C778 [lymph nodes of multiple regions]. The spleen is not listed under the Primary Site(s) section in the Heme DB for diffuse large B-cell lymphoma. Per Rule PH21 code the primary site to 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. The spleen is a primary site for only a few lymphomas (noted in the Heme DB). Because the spleen filters blood, it is often reactive (splenomegaly) or frankly involved with the lymphoma. That reaction or involvement, however, does not affect the primary site coding. Only the involved nodes are used in 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.

Use Rule PH21 to code the primary site to C778 [lymph nodes of multiple regions]. The spleen is not listed under the Primary Site(s) section in the Heme DB for follicular lymphoma. Per Rule PH21 code the primary site to 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. The spleen is a primary site for only a few lymphomas (noted in the Heme DB). Because the spleen filters blood, it is often reactive (splenomegaly) or frankly involved with the lymphoma. That reaction or involvement, however, does not affect the primary site coding. Only the involved nodes are used in 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.

1. EBV positive B-cell lymphoproliferative disorder is reportable. Code EBV positive lymphoproliferative disorder as DLBCL [9680/3], the term "of the elderly" does not have to be part of the diagnosis.

• The term "iatrogenic" simply means that there is no known cause for the disease. Iatrogenic EBV positive lymphoproliferative disorder is reportable.

• EBV positive B-cell lymphoproliferative disorder is a synonym for DLBCL [9680/3]. You do not need to have the lymphoma (DLBCL) 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 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph..

Per Rule PH30, use the Heme DB, determine the histology when rules PH1-PH29 do not apply. Code diffuse large B-cell lymphoma, germinal cell type to 9680/3 [diffuse large B-cell lymphoma (DLBCL)][9680/3]. Under the Alternate Names section of the Heme DB, these two terms are synonyms that share the same histology code.

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 2007 to 2017 ONLY: Code the histology as papillary urothelial carcinoma [8130].NOTE: In the CAP checklist, the statement that the tumor has a papillary configuration is a further description of this tumor. This is supported by the pathologist's stage of pTa [noninvasive papillary carcinoma]. Use the information from the CAP checklist when available. The MP/H Rules will be revised to include the term "configuration" in the specific histology terms for in situ tumors.

The steps used to arrive at this decision are

Step 1: Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three (i.e., flowchart, matrix or text) and go to the Urinary Histo rules. The module you use depends on the behavior and number of tumors identified in the primary site. In this case, the patient has a single bladder tumor per the submitted information.

Step 2: Start at Rule H1 in the Single Tumor module. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H15. Stop at the first rule that applies to the case you are processing. Stop at Rule H7. Code the histology as 8130/2 (noninvasive papillary urothelial carcinoma) when the urothelial carcinoma is stated to have a papillary configuration.

For cases diagnosed 2018 or later, refer to the Solid Tumor Rules, https://seer.cancer.gov/tools/solidtumor/