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20100075 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned when a 1/27/10 bone marrow biopsy, FISH and cytogenetics reveals chronic myelogenous leukemia (CML), BCR/ABL positive, t(9;22)(q34;q11) and a 4/15/10 bone marrow biopsy reveals B-acute lymphoblastic leukemia (Blast phase of CML)? | 1/27/10 BM biopsy: CML BCR/ABL+ FISH positive for BCR/ABL and cytogenetics showing the t(9;22)q34q11.2 translocation. Treated with Imatinib. 4/15/10 BM biopsy: B-acute lymphoblastic leukemia (Blast phase of CML). Would the term "blast phase of CML" indicate the 4/15/10 bone marrow biopsy showed CML or would a new primary be abstracted with histology coded 9811/3 [B lymphoblastic leukemia/lymphoma, NOS]?
Applying rule M10, this is a new primary, but note 2 states transformations are defined in the Heme DB. The Abstractor Notes section indicates CML has three phases: chronic, accelerated, and the blastic phase or blast crisis. The accelerated phase can last weeks to months. In the chronic phase the involvement is usually limited to blood, bone marrow and spleen although the liver may be infiltrated. During the blastic phase, lymph nodes and tissue may be involved. The blastic phase is a disease progression from the chronic phase. The disease, however, remains the same histology, chronic myelogenous leukemia. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case represents a multiple primary per Rule M15 which states you are to use the Heme DB Multiple Primaries Calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14.
The histology for the first primary is coded to 9875/3 [chronic myelogenous leukemia, BCR-ABL1 positive].
The histology for the second primary is 9811/3 [B lymphoblastic leukemia/lymphoma, NOS] in the absence of further documentation that the B-ALL was also positive for the t(9;22) translocation.
The histology code 9806/3 [Mixed phenotype acute leukemia with t(9;22)(q34;q11.2); BCR-ABL1] cannot be used for the second primary because there is no documentation that the B-ALL diagnosed on 04/15/2010 also had the t(9;22) translocation and this histology cannot be used in patients ." Per the Definition section in the Heme DB, in order to use histology code 9806/3 "This leukemia meets the criteria for mixed phenotype acute leukemia (MPAL) in which the blasts also have t(9;22) translocation of BCR-ABL1 rearrangement. Some patients with chronic myeloid leukemia may develop or even present with a mixed blast phase that would meet criteria for MPAL; however, this diagnosis should not be made in patients known to have had CML."
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 |
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20100018 | Reportability/Heme & Lymphoid Neoplasms--Hematopoietic, NOS: Is light chain disease reportable if it is treated with chemotherapy agents? See Discussion. | A patient was diagnosed in 2010 with light chain disease based on SPEP and urine testing. Bone marrow aspiration and biopsy were done. Flow cytometry, cytogenetic studies and FISH for plasma cell disorders are all normal. Medical oncologist states diagnosis is light chain disease. Patient was started on Revlimid, dexamethasone and Velcade.
In reviewing the case reportability instructions, this seems to fall under Instruction 1, note 1. Immunoglobulin deposition disease, preferred term for light chain disease, is coded as 9769/1. This is normally a non-reportable diagnosis, but the patient was given cancer-directed treatment. Would this case be accessioned using the above morphology code and primary site of bone marrow [C42.1]? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case is not reportable. The histology is 9769/1 [light chain disease] in the Heme DB.
Light chains are produced in neoplastic plasma cells (multiple myeloma) and are called Bence-Jones proteins. The physician did the cytogenetic studies and FISH to rule out plasma cell disease. 50-60% of people with Light-chain deposition disease (LCDD) have an associated lymphoproliferative disorder, most commonly multiple myeloma. The remaining patients develop LCDD in the setting of progression of monoclonal gammopathy of unknown significance (MGUS) with no evidence of neoplastic plasma cell proliferation. This patient falls in this category, MGUS, which is not reportable. |
2010 |
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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 |
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20100066 | MP/H Rules/Multiple Primaries--Breast: How many primaries should be accessioned if two tumors are present in the same breast, a 1.7 cm colloid carcinoma and a 1.5 cm colloid carcinoma with infiltrating ductal carcinoma? See Discussion. | If a patient has two masses in the same breast with different histology codes and different sizes, should this be accessioned as two primaries? Or should this be a single primary based on the largest tumor size or numerically higher histology code?
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For cases diagnosed 2007 or later, abstract this case as two primaries. Mucinous/colloid carcinoma of the breast is rare. The first tumor describes (1.7 cm) fits this criteria because the pathologist simply says mucinous carcinoma. The diagnostic criteria for mucinous carcinoma is that pools of extracellular mucin make up at least 1/3 of the volume throughout the tumor mass. If focal areas are not at least 33% mucinous, the designation is a mixed mucinous/ductal. That fits the second tumor (1.5 cm).
For this case, you must get the histology codes for both tumors in order to use the Multiple Primary rules. Per H14 the first tumor is coded mucinous carcinoma [8480/3]. Per H17 the second tumor is coded duct carcinoma mixed with any other carcinoma [8523/3]. Now go to the MP rules. Per M12 abstract this case as multiple primaries because the ICD-O-3 histology codes are different at the second and third digit. |
2010 |
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20100029 | MP/H Rules/Histology--Corpus uteri: How should histology be coded and how many primaries should be accessioned for an endometrial primary in which curettings showed malignant mixed mullerian tumor (carcinosarcoma) but hysterectomy specimen showed endometrioid adencarcinoma? See Discussion. | The pathology report COMMENT for the hysterectomy specimen stated that the previous curettage was reviewed. The findings are compatible with malignant mixed mullerian tumor. No residual features of malignant mixed mullerian tumor are found in the current resection, which shows FIGO grade I adenocarcinoma in the wall of the uterus. The malignant mixed Mullerian tumor appears to have been removed with the curettage. There is no information available regarding the number of tumors in these specimens. | For cases diagnosed 2007 or later, abstract a single primary. Rule M1 applies because there is no information on the number of tumors and there is no way to know whether the curettage sample was from a separate tumor or from the tumor in the hysterectomy specimen.
Apply rule H17 and code histology to 8980/3 for malignant mixed Mullerian tumor [Carcinosarcoma, NOS]. |
2010 |
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20100090 | MP/H Rules/Histology: How is histology coded for a diagnosis of "poorly differentiated endometrioid adenocarcinoma intermixed with osteoid sarcomatous component, consistent with malignant mixed mullerian tumor with heterologous (osteosarcoma) elements"? Is malignant mixed mullerian tumor synonymous with carcinosarcoma? See Discussion. | Given that there is no mixed code for these histologies, can the numerically higher code be used per H17 (malignant mixed mullerian tumor [8950/3]) using the logic of the MP/H rule for other sites? If so, should this histology be coded as 8980/3 [carcinosarcoma] rather than 8950/3 [malignant mixed mullerian tumor]? | For cases diagnosed 2007 or later, code histology to 8980/3 [carcinosarcoma]. Recent literature states that carcinosarcoma is synonymous with mixed mullerian tumor. Mixed mullerian tumor is an obsolete term and should not be used. | 2010 |
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20100064 | Histology--Heme & Lymphoid Neoplasms: How is histology to be coded for acute lymphoblastic leukemia (ALL) and/or precursor B acute lymphoblastic leukemia (Pre-B ALL) for cases diagnosed 2010 and later? The Heme Database has two histology codes for this disease, both 9811/3 and 9836/3, which is the correct histology code? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code histology to 9811/3 [B lymphoblastic leukemia/lymphoma, NOS].
See the Abstractor Notes section in the Heme DB, when determining how to code histology for a case. It indicates the code 9811/3 is effective for cases diagnosed 2010 and forward. The 9836/3 is listed as obsolete and refers you to code 9811/3. Make sure to check for a specific subtype of B lymphoblastic leukemia/lymphoma [9812/3 - 9818/3] before assigning the NOS code [9811/3].
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. |
2010 | |
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20100109 | Reportability--Ovary: Does the ICD-O-3 term "stromal endometriosis" [8931/3] always imply a reportable malignant disease process if the pathologist also states there is "no evidence of carcinoma" in the same report? See Discussion. | ROS Final Diagnosis: LSO: Ovary with an endometriotic cyst (1.2 cm) and stromal endometriosis with multifocal papillary syncytial eosinophilic, clear cell and tubal metaplasia, no evidence of carcinoma.
COMMENT: There is extensive endometriosis involving the ovarian stroma and the ovarian surface. The ovarian stroma contains multiple cystic endometrial glands and surrounding endometrial type stroma with variable amounts of hemorrhage. There are non-cystic foci of endometriosis comprised of small, irregular glandular structures within the stroma. The lining of larger cyst/cysts is involved by a single layer of cuboidal to columnar cells with markedly eosinophilic cytoplasm in areas of serous (tubal) metaplasia and papillary projections suggestive of papillary syncytial metaplasia. Within these areas there is epithelial tufting and stratification, raising the consideration of proliferative/borderline change (which we cannot entirely exclude), however, given the background of endometriosis and morphologic similarity to papillary syncytial metaplasia in the endometrium, we favor that this is a non-neoplastic reactive change. There is no evidence of carcinoma. |
This case is not reportable. The pathologist states that there is no evidence of carcinoma. The ICD-O-3 matrix system applies, giving the pathologist the final say on behavior. | 2010 |
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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 |
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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 |
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