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Report Produced: 04/29/2025 12:14 PM

Report Question ID Question Discussion Answer Year
20100053 Primary site--Heme & Lymphoid Neoplasms: How is primary site coded for a myeloid sarcoma (granulocytic sarcoma) arising in the chest wall in a patient that has a negative bone marrow biopsy? See Discussion.

Patient was diagnosed with Myeloid Sarcoma (granulocytic sarcoma) by chest wall biopsy. This is an extramedullary manifestation of acute leukemia and is not in the bone marrow (bone marrow is negative).

How should primary site be coded? The Heme DB states that almost any part of the body can be involved. It also states to not code primary site to C421. In this case the only involvement is the chest wall [C493]. However, use of the primary site code C493 triggers an edit error questioning this site/histology combination. If the primary site is coded to C421 [bone marrow], there is no edit error. Please explain the site code and rationale.

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

Unless there are scans showing involvement of a lymph node or tissue other than the chest wall, the histology should be coded myeloid sarcoma [9930/3] and the primary site to C493 [soft tissue of chest wall]. Per Rule PH 30, use the Heme DB to determine primary site and histology when rules PH1-PH29 to not apply. Override the edit.

Per the Abstractor Notes section in Heme DB, for myeloid sarcoma [9930/3] the most frequently affected sites are skin, lymph nodes, gastrointestinal tract, soft tissue, and testis.

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.

2010
20100052 Reportability/Primary Site: What is the reportability status and primary site for a papillary carcinoma of thyroid tissue arising in an otherwise benign mature monodermal cystic teratoma (struma ovarii)? See Discussion. Final diagnosis on the pathology report states, "One ovary showing mature monodermal cystic teratoma composed of thyroid tissue (struma ovarii)." The pathology COMMENT section states, "There is a 0.1 cm focus of thyroid tissue within the struma ovarii showing cytologic features of papillary carcinoma. This finding is likely of no clinical consequence."

A papillary carcinoma of thyroid tissue in benign struma ovarii (mature cystic teratoma) is reportable.

These ovarian tumors contain a diversity of tissues including hair, teeth, bone, thyroid, etc. This reportable malignancy arose in thyroid tissue within the ovarian tumor. Code the primary site to ovary. Code to the actual organ in which the cancer arose. This will keep the case in the appropriate category for surgery coding, regional nodes, staging, etc.

2010
20100050

Reportability--Colon: Would a carcinoid tumor, NOS, of the appendix with perineural or angiolymphatic invasion be reportable if there is no mention of malignancy in the pathology report?

Carcinoid, NOS, of the appendix diagnosed in 2015 or later is reportable.

For cases diagnosed prior to 2015

Carcinoids of the appendix are reportable when they meet any of the following conditions.

  • The pathologist designates the carcinoid as malignant

  • Regional lymph nodes are positive for MALIGNANT carcinoid (not reportable if lymph nodes are reported to be involved with benign carcinoid disease)

  • There are discontinuous metastatic implants or involvement

    • Note that the implants/involvement must be designated as malignant. Many benign tumors will spawn implants that are also benign. If implants are benign, this is not a reportable tumor.

    		2010
    		20100049

    Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted when a lymph node biopsy reveals "malignant lymphoma, peripheral T-cell type, with some features of angioimmunoblastic T-cell lymphoma and follicular T-cell lymphoma," the bone marrow biopsy was negative for involvement, and the oncologist states this patient has "peripheral T-cell lymphoma"? See Discussion.

    CT scan showed retroperitoneal and inguinal adenopathy. Right inguinal lymph node biopsy revealed "malignant lymphoma, peripheral T-cell type, with some features of angioimmunoblastic t-cell lymphoma and follicular t-cell lymphoma." Flow cytometry studies showed no evidence of B-cell lymphoma and atypical CD3+/CD10+/CD7-/CD4+/CD56+ T cells are detected (19%). The bone marrow biopsy was negative for involvement. Patient was staged as Stage II Peripheral T-Cell lymphoma by the oncologist and started chemotherapy.

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

    Code the oncologist's clinical diagnosis of peripheral T-cell lymphoma.

    The definition for this neoplasm is "A large group of lymphomas which we collectively refer to as peripheral T-cell lymphomas with the optional addition of "unspecified" to emphasize that these cases do not belong to any better defined entities. Attempts to distinguish between them on morphological basis have met with poor reproducibility."

    Per the Abstractor Notes in the Heme DB: Patients present with peripheral LN involvement. The diagnosis of PTCL, NOS is made ONLY when other specific entities have been explored.

    This fits your case; attempts to find a more specific disease (flow cytometry; BM biopsy) were negative and gave no further information that could be used to assign a more specific classification.

    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.

    		2010
    		20100048 Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a patient diagnosed with Langerhans cell histocytosis/eosinophilic granuloma involving both the seventh rib and the right temporal bone? See Discussion. Patient was diagnosed with Langerhans cell histiocytosis/eosinophilic granuloma following a biopsy of the seventh rib on 3/22/10. On 4/13/10 the patient had a right external ear canal mass (right temporal bone) biopsy with same diagnosis. Should the primary site be coded to bone, NOS [C419]?

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

    Per Rule PH30, use the Heme DB to determine the primary and code it to bone, NOS [C419]. Langerhans cell histiocytosis can occur as a solitary lesion, multifocal lesions, or multisystem disease. In this case, the patient has multifocal disease of the bone. The abstractor notes in the Hematopoietic DB were used as a reference for this answer.

    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.

    		2010
    		20100047

    Reportability--Heme & Lymphoid Neoplasms: Is "myelodysplasia" a reportable disease?

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

    The diagnosis of "myelodysplasia" is not reportable.

    Myelodysplasia covers a group of disorders that result in the inability to produce enough healthy mature blood cells. Those disorders include: anemia, leukopenia, thrombocytopenia, MDS, refractory anemia, refractory anemia with excess blasts in transformation, refractory anemia with ring sideroblasts, refractory anemia with excess blasts, chronic myelomonocytic leukemia, acute myeloid leukemia. Follow-back to the physician is necessary to determine whether or not a particular case represents a malignancy.

    "Myelodysplasia" is also listed in 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.

    		2010
    		20100046

    Heme & Lymphoid Neoplasms: Is a clinical remission sufficient to change the tumor status to "disease free" for a patient on long-term chemotherapy for a diagnosis of either a chronic hematologic disease, such as CML, or a myeloproliferative disorder, such as essential thrombocythemia? See Discussion.

    For some patients with chronic hematologic diseases, the disease/recurrence status could change frequently as chemotherapy is started and stopped over an extended period of time. Should the tumor status for these cases always be "not disease free"? When the physician documents the patient is in clinical remission, does their status change to "NED or disease free?" There seems to be a lot of variation across the US in how registrars are coding this field. Clarification would be appreciated.

    The term "disease free" is not used in a standard fashion for hematopoietic and lymphoid neoplasms.

    Code the cancer status to free of disease when the physician indicates NED. For hematopoietic and lymphoid neoplasms, a physician's statement of NED, disease-free, clinical remission or no evidence of disease at this time, should be recorded with cancer status to disease free. The term "disease free" or NED means that there is no clinical evidence of disease.

    		2010
    		20100045

    Histology--Heme & Lymphoid Neoplasms: How is histology coded for a pathologic diagnosis of "B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma" that was clinically referred to as a "double hit lymphoma"?

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

    Code histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Per the Alternate Names section in the Heme DB, B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is one of the synonyms for for DLBCL.

    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.

    		2010
    		20100044 Primary site--Heme & Lymphoid Neoplasms: Should the primary site be coded to C499 [Blood vessels, NOS] for a case of intravascular large B cell lymphoma, Asian variant [9712/3] found in the bone marrow and liver? See Discussion. Patient has biopsy proven intravascular large B cell lymphoma, Asian variant, (9712/3) in bone marrow and liver. The Hematopoietic Database does not give a primary site code. Should the primary site be coded C49.9 because, by definition, this lymphoma arises in the blood vessels?

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

    Code primary site to C499 [blood vessels]. The definition in the Heme DB does specify that this type of extranodal large B-cell lymphoma is characterized by lymphoma cells within the lumina of blood vessels with the exception of larger arteries and veins. The reason no primary site is specified is that Western variant can originate in the skin or CNS.

    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.

    		2010
    		20100043

    Primary site--Heme & Lymphoid Neoplasms: When only pathology reports are available, how should the primary site be coded when a both a bone marrow biopsy and colon biopsy demonstrate "mantle cell lymphoma"?

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

    For this case, code primary site to C189 [colon, NOS] per Rule PH24.

    Mantle cell lymphoma usually begins with lymph node involvement and spreads to other tissue. However, it can begin in a lymphocyte such as those in the GI tract. Per the Abstractor Notes section in the Heme DB, patients usually present with advanced disease. About half will have some combination of B symptoms. Swelling of lymph nodes and spleen are usually present. Bone marrow, liver and GI tract involvement occurs in a very high percentage

    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.

    		2010