Report | Question ID | Question | Discussion | Answer | Year |
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20120064 | Reportability--Heme & Lymphoid Neoplasms: If hemophagocytic lymphohistiocytosis treated with several rounds of chemotherapy is reportable, what is the primary site? |
Patient was diagnosed with hemophagocytic lymphohistiocytosis on blood and bone marrow biopsy. This was also referred to in the chart as hemophagocytosis and hemophagocytic syndrome. Hemophagocytic syndrome is listed in the Heme DB as 9724/3. The patient had several rounds of fairly aggressive chemotherapy. Would the correct primary site for histology 9724/3 be C421 [bone marrow], or C779 [lymph nodes, NOS]? See SINQ 20100113. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. Per Appendix F, HLH is caused by an over stimulated immune system (infection, etc.). It is a clinical syndrome associated with a variety of underlying conditions. To be reportable, a child's diagnosis must state "fulminant hemophagocytic syndrome" to be reportable (9724/3). This is not the situation in this case. "Hemophagocytic lymphohistiocytosis" 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. |
2012 |
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20120082 | MP/H Rules/Multiple primaries--Breast: How many primaries are accessioned if the patient has two tumors in the left breast, one that is ductal carcinoma with mucinous differentiation and the other is ductal carcinoma, NOS? See Discussion. |
The final diagnosis from the left mastectomy was multifocal invasive ductal carcinoma (mpT1cN0) with associated intermediate grade ductal carcinoma in situ located between the invasive foci. Larger 2:00 focus: moderately differentiated ductal carcinoma with mucinous differentiation (1.4 cm). Smaller 3:00 focus: moderately to poorly differentiated ductal carcinoma (1.2 cm). The histologies of the invasive foci should be coded 8523/3 and 8500/3 respectively. To determine the number of primaries, does rule M11 apply which indicates this should be a single primary even though ductal with mucinous differentiation is not in Tables 1 or 2? Or does rule M12 apply because there is a difference in the third digit of histology and thus means this should be reported as a multiple primary case? |
For cases diagnosed 2007 or later, accession two primaries, ductal carcinoma with mucinous differentiation [8523/3] and ductal carcinoma, NOS [8500/3]. The steps used to arrive at this decision are: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Breast MP rules because site specific rules have been developed for this primary. Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. These tumors have ICD-O-3 histology codes that are different that the third (xxx) digit and are, therefore, multiple primaries. Ductal carcinoma with mucinous differentiation is not a specific type of ductal carcinoma identified in either Table 1 or 2. (To locate Tables 1 and 2, go to Breast under the Terms & Definitions section of the manual.) It is ductal carcinoma mixed with another type of carcinoma (mucinous carcinoma in this case) see Table 3. Rule M11 does not apply. |
2012 |
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20120088 | MP/H Rules/Multiple primaries--Head & Neck: How many primaries are accessioned and what rule applies if a patient has an extensive tumor in the left ethmoid sinus and a separate tumor in the right maxillary sinus? See Discussion. |
MRI and CT Neck Impression: Extensive tumor mass which likely originated within the left ethmoid sinus and extends intracranially via the cribriform plate into the anterior cranial fossa. There is involvement of the left orbit and extension into the superior aspect of the left maxillary sinuses as well as the nose. Second enhancing lesion within the right maxillary sinus measures almost 2 cm. The second mass within the floor of the right maxillary sinus, with similar imaging characteristics, is consistent with malignant involvement. The patient has an extensive ethmoid sinus tumor, biopsy showed squamous cell carcinoma. The ethmoid sinus is not a paired organ. The patient also has a small maxillary tumor with no histologic confirmation, Hem/Oncology chart notes state the right maxillary sinus mass is carcinoma. The maxillary sinus is a paired organ. Per the AJCC Manual (AJCC Manual for Staging, 7th edition, page 70), both the ethmoid and maxillary sinuses are further identified by their laterality (left and right). Why aren't the ethmoid sinuses a paired organ for the MP/H Rules? What MP rule applies to this case? |
For cases diagnosed 2007 or later, accession a single primary. The steps used to arrive at this decision are: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Head and Neck MP rules after determining the histology of each tumor - (8070/3 [squamous cell carcinoma] and 8010/3 [carcinoma, NOS]) because site specific rules have been developed for this primary. Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. Abstract a single when one tumor is carcinoma, NOS [8010] and another tumor is a specific carcinoma, squamous cell carcinoma [8070] because the ethmoid sinus (site of origin) is not a paired site per the MP/H rules. We will review the list of paired organs for the next edition of the MP/H Rules. |
2012 |
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20120048 | MP/H Rules/Primary site: Can you clarify how you interpreted the term "synchronous" to appropriately code the primary site to C68.9 [urinary tract] for SINQ 20110119 and did not use that code for SINQ 20100025 when both cases used MP/H Rule M8 to determine the number of primaries? See Discussion. | In SINQ 20100025 a patient was diagnosed with multiple urinary system tumors over a year apart. Rule M8 applies (single primary) and the primary site was left coded to the original primary site, C65.9 [renal pelvis]. In SINQ 20110119 a patient is diagnosed with multiple urinary system tumors within a month of each other, again rule M8 applies (single primary) and the primary site was coded to C68.9 [urinary system, NOS].
In both cases, rule M8 applies. However, the tumors were not diagnosed synchronously (e.g., one month apart in one case and greater than one year apart in the other). When the SINQ answer states, "same time" or "synchronous" does this mean during the same event? If not, what is the time range for "same time" or "synchronous"?
Please clarify when it is appropriate to code the primary site to C68.9 [urinary system, NOS] for Rule M8 and when it is not. |
For the purpose of applying the MP/H rules, the term "synchronous" means that the two diagnoses occurred at the same time or less than or equal to 60 days apart.
The case in SINQ 20100025 was not synchronous. The first lesion in the renal pelvis [C65.9] occurred in 1/08 and the subsequent tumors were diagnosed in 5/09, more than one year apart. In this case, you do not go back to change the primary site code on the original abstract.
The case in SINQ 20110119 was diagnosed synchronously, the first lesion in the bladder [C67.9] was diagnosed in 11/09 and the second lesion in the renal pelvis [C65.9] was diagnosed in 12/09, less than 60 days apart. Because the lesions were synchronous, the primary site is coded urinary system, NOS [C68.9]. |
2012 |
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20120063 | Reportability--Pancreas: Are neuroendocrine "tumors" reportable and are they synonymous with neuroendocrine "carcinoma"? See Discussion. | Example: Pancreatic mass that probably represents a neuroendocrine tumor is staged as cT2N0M0. | According to the World Health Organization (WHO) pancreatic neuroendocrine tumors (NET) are malignant. They are reportable.
For pancreas primaries, code NET, G1 (well differentiated) to 8240/3; NET G2 (moderately differentiated) to 8249/3; and nonfunctional NET, GI or G2 to 8150/3. The histology code for neuroendocrine carcinoma (NEC) is 8246/3, large cell NEC is 8013/3 and small cell NEC is 8041/3. |
2012 |
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20130149 | MP/H Rules/Histology--Testis: What is the histology code for a testis primary with embryonal carcinoma (70%), yolk sac tumor (30%), and a focus of seminoma (<1%)? See Discussion. | The right orchiectomy specimen showed a mixed histology tumor. The retroperitoneal lymph nodes showed teratoma, NOS only. Does the presence of teratoma in the lymph nodes change the histology coding?
The MP/H Rules for Other Sites, Table 2 (Mixed and Combination Codes) does not include the combination of embryonal carcinoma, yolk sac tumor and seminoma. SINQ 20110013 does state the combination of embryonal carcinoma and yolk sac tumor should be coded to histology 9065/3 [germ cell tumor, nonseminomatous]. In this case, is the focus of seminoma comprising <1% included when coding the histology? If the seminoma is included, Table 2 still does not address this combination. |
Code the histology to mixed germ cell tumor [9085/3] per Rule H16; code the appropriate combination/mixed code when there are multiple specific histologies.
According to the WHO Classification of Tumors of the Male Genital Organs, tumors of more than one histologic type (mixed forms) can occur in any combination of various germ cell histologies including embryonal, yolk sac, teratoma, and choriocarcinoma. Mixed teratoma and seminoma is included under histology code 9085/3 [mixed germ cell tumor] in the ICD-O-3. The revised MP/H rules will expand on these mixed testicular histologies.
Priority for coding histology is using the diagnosis from the primary site (when possible) over the histology from a metastatic site. The presence of teratoma, NOS in the retroperitoneal lymph nodes does not change the histology code. |
2013 |
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20130040 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a patient has a history of chronic myeloid leukemia diagnosed in 1993 followed by a diagnosis of acute myeloid leukemia arising in chronic myelogenous leukemia, blast phase? See Discussion. |
12/1993 Bone marrow biopsy: Chronic myeloid leukemia t(9;22) (q34;q11).
09/2011 Bone marrow biopsy: Abnormal cytogenetic & FISH support persistent involvement by chronic myelogenous leukemia.
12/2011 Peripheral blood, flow cytometry: Involvement by acute myeloid leukemia arising in chronic myelogenous leukemia (CML, blast phase, 30% blasts by manual diff.).
Is the 12/2011 diagnosis a new primary? If not, why don't Rules M8-M13 apply when the Heme DB Abstractor Notes section for CML indicates that when there is a chronic, accelerated and blast phase that develops later in the course of the disease, change the histology code to the more specific diagnosis?
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For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as a multiple primary: chronic myelogenous leukemia t(9;22) (q34;q11) [9863/3] diagnosed in 1993 and acute myeloid leukemia [9861/3] diagnosed in 2011 per Rule M15.
Use the diagnosis date to determine the appropriate manual and rules to follow to determine the histologies for this case. To determine the histology of the 1993 diagnosis, use the ICD-O-2. The Heme Manual & DB will be used to determine the number of primaries and the histology of the 2011 diagnosis of AML.
Rules M8-M13 in the Heme Manual cannot be applied to this case because no transformation occurred. CML does not transform to another 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. |
2013 |
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20130171 | Reportability--Heme & Lymphoid Neoplasms: Is "plasma cell neoplasm" a synonym for multiple myeloma and is it reportable? See Discussion. | Path report in the comment section states "plasma cell neoplasm such as monoclonal gammopathy of undetermined significance (MGUS)." | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Appendix F, plasma cell neoplasm is not a synonym for multiple myeloma. Plasma cell neoplasm is a disorder that has an abnormal number of plasma cells. MGUS is such a disorder, but it is not reportable.
According to WHO, 'Plasma cell neoplasms' is the umbrella term that includes MGUS, plasma cell myeloma, solitary plasmacytoma of bone, immunoglobulin deposition diseases, extraosseous plasmacytoma, and osteosclerotic myeloma. Of these, only plasma cell myeloma, solitary plasmacytoma of bone, and extraosseous plasmacytoma are reportable.
Note: This terminology was added to the 2012 Hematopoietic Manual and Database for 1/1/2012. This should not have been added. If the only diagnosis is "plasma cell neoplasm," this is not reportable. If the diagnosis is "plasma cell neoplasm c/w multiple myeloma (or another reportable disease)," then it would be a reportable disease.
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. |
2013 |
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20130172 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the histology for each if a bone marrow diagnosis reveals co-existent systemic mastocytosis and a lymphoplasmacytic neoplasm? See Discussion. | 11/7/12 Peripheral blood flow cytometry: small population of clonal CD5- CD10- B-cells consistent with a B-cell lymphoproliferative process.
1/16/13 Bone marrow final diagnosis: co-existent systemic mastocytosis and lymphoplasmacytic neoplasm.
B-cell component of lymphoplasmacytic neoplasm constitutes 20% of bone marrow cellularity and the plasma cell component approximately 20%. The differential diagnosis includes marginal zone lymphoma with plasmacytic differentiation and lymphoplasmacytic lymphoma.
Flow cytometry: kappa monotypic B-cells and plasma cells.
Comment: Co-existence of systemic mastocytosis and mature B-cell lymphoma meets the criteria for Systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD).
From our physician's progress note: KIT-D816V-positive, CD117+/CD25+ /SM-AHNMD(40% of the nucleated cells as spindled mast cells) but also seemingly two distinct lymphoid neoplasms, a CD5-negative/CD10-negative B-cell lymphoproliferative neoplasm consistent with occupying another 20% of the nucleated marrow space, together with an IgG-kappa-restricted (non-reportable diagnosis) occupying another 20% of the nucleated marrow space (and an accompanying 2.0 g/dl M-spike without hypercalcemia or anemia). |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Under the Alternate Names section of the Heme DB, systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD) is a synonym for systemic mastocytosis. Per Rule M2, this is one primary. Abstract a single primary when there is a single histology. Code the histology to 9741/3 [systemic mastocytosis].
Per the pathology report, the two diagnoses of systemic mastocytosis and mantle cell lymphoma meet the criteria for SM-AHNMD. The B-cell lymphoma is a symptom/marker of the AHNMD. In systemic mastocytosis with AHNMD, a myeloid or lymphatic malignancy is diagnosed with the SM. The prognosis is usually dominated by the non-mast cell malignancy.
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. |
2013 |
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20130160 | Histology--Heme & Lymphoid Neoplasms: Should the histology be coded to a therapy-related myeloid neoplasm when the physician states the diagnosis of acute myeloid leukemia is secondary to treatment with Imuran? See Discussion. | Patient has a diagnosis of AML for which the physician recommends a bone marrow transplant. The physician indicated the diagnosis is actually a secondary AML due to treatment with Imuran for polymyalgia rheumatica. The physician also stated this is a high risk type of AML. Imuran is not a chemotherapy agent per SEER*Rx. Can the histology be coded as 9920/3 (e.g., Therapy-related acute myeloid leukemia, NOS) when the patient has not been treated with chemotherapy for a reportable disease? The physician is a bone marrow transplant expert who states the AML is therapy-related disease. Bone marrow disease is a listed as a risk for treatment with Imuran. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code this histology to 9920/3 [therapy-related myeloid neoplasm] when the physician states the acute myeloid leukemia is therapy-related.
Therapy-related AML can result from any systemic therapy for benign or malignant diseases. In this case, AML resulted from immune system-suppressing therapy with Imuran for a benign disease, polymyalgia rheumatica. The drugs that induced the AML do not have to be listed in the SEER*Rx database.
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. |
2013 |