| Report | Question ID | Question | Discussion | Answer | Year |
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20130121 | Reportability--Heme & Lymphoid Neoplasms: Is "early essential thrombocythemia" reportable? See Discussion. | The bone marrow biopsy diagnosis was, "Combined bone marrow morphologic, flow cytometric, immunohistochemical, molecular and cytogenetic findings are most consistent with early or evolving essential thrombocythemia with low level JAK2 V617F mutation documented on molecular testing." The physician is calling this a benign process. Is this reportable as essential thrombocythemia? Are the terms early or evolving ignored? Does the presence of a JAK2 mutation make this reportable? Without JAK2 testing is this case reportable? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Yes, this is a reportable case. The histology is coded to 9962/3 [essential thrombocythemia]. The positive JAK2 mutation testing and bone marrow biopsy results taken together support the diagnosis of essential thrombocythemia in this case.
In the Abstractor Notes section of the Heme DB, it indicates that only 50-60 percent of patients with essential thrombocythemia will have a positive JAK2 mutation. A diagnosis of essential thrombocythemia can still be made in the absence of a JAK2 mutation. For example, if the bone marrow biopsy final diagnosis or a physician's clinical diagnosis is essential thrombocythemia, despite a negative JAK2 mutation test, the neoplasm is still 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. |
2013 |
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20130115 | Histology--Heme & Lymphoid Neoplasms: How is histology coded when the biopsy final diagnosis is "low grade B-cell lymphoma of unclear subtype (splenic marginal zone lymphoma?)" and the hematologist clinically diagnoses this as splenic marginal zone lymphoma? See Discussion. | This patient has massive splenomegaly. The biopsy final diagnosis was "low grade B lymphoma of unclear subtype (splenic marginal zone lymphoma?)." The pathologist's comment states, "Because of the clinical context (lymphocytosis and splenomegaly) a splenic marginal zone lymphoma is a possibility." There are no other histologic diagnoses. All the flow cytometry reports are as unclear as the biopsy.
The hematologist, after seeing the pathology report, states, "The bone marrow biopsy shows a significant infiltration by mature lymphocytes; their markers strongly suggest a marginal zone lymphoma, probably of splenic origin The final diagnosis is a splenic marginal zone lymphoma."
Should the clinical diagnosis of splenic marginal zone lymphoma [9689/3] be coded when a clinical diagnosis is not listed as a definitive diagnostic method for this neoplasm? Or should the histology be coded as low grade B-cell lymphoma [9591/3]? The clinicians will expect the case to be coded as a splenic marginal zone lymphoma when there's no doubt about the diagnosis. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9689/3 [splenic marginal zone lymphoma] per Rule PH29 and Case Reportability Instruction #6 in the Heme Manual. Case Reportability Instruction #6 indicates, "Report the case when there is a (physician's statement) of reportable hematopoietic or lymphoid neoplasm."
The pathology gave an NOS diagnosis, low grade B-cell lymphoma [9591/3]. The physician clinically stated this was a splenic marginal zone lymphoma [9689/3]. Rule PH 29 states to code the specific histology when the diagnosis is one non-specific histology AND one specific histology AND the Heme DB MP Calculator indicates they are the same primary. Per the Multiple Primaries Calculator, these two histologies indicate the same primary.
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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20130079 | Reportability--Heme & Lymphoid Neoplasms: Is plasma cell dyscrasia reportable and synonymous with multiple myeloma? See Discussion. |
Bone marrow biopsy and aspirate: Plasma cell dyscrasia with IgG kappa expression with FISH (+) for the following abnormalities: 3 copies of 1q21 (25/30 plasma cells) and an extra CCND1 signal (25/34 plasma cells) which is indicative of the presence of other chromosome 11 abnormalities possibly trisomy 11, a change known to occur in plasma cell neoplasms. Flow cytometry: A monoclonal plasma cell population is present, co-expressing cIgG, cKappa, CD56, & CD117 (up to 14% of analyzed cells). |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Plasma cell dyscrasia and multiple myeloma are not synonymous terms. Plasma cell dyscrasia is not listed in the Alternate Names section of the Heme DB for plasma cell myeloma (multiple myeloma). Plasma cell dyscrasia is listed in the Alternate Names section of the Heme DB for MGUS [9765/1], which is not a reportable disease. Plasma cell dyscrasia (PCD) is not reportable. PCD is a diverse group of neoplastic diseases that produces a serum M component (monoclonal immunoglobulin). Usually these patients have a plasma cell morphology such as multiple myeloma or heavy chain disease. However, the registrar cannot diagnose multiple myeloma or heavy chain disease (or any other plasma cell neoplasm). There must be a physician statement and/or a positive biopsy to confirm a reportable 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. |
2013 |
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20130070 | Reportability--Is "intraductal papillary mucinous neoplasm with low grade dysplasia" (also called IPMN adenoma) reportable? See Discussion. |
According to the ICD-O-3, the histology for IPMN adenoma is 8453/0 is non-reportable. However, per SINQ 20021099, this is reportable. |
Intraductal papillary mucinous neoplasm (IPMN) of the pancreas with low grade dysplasia, also referred to as IPMN adenoma, is not reportable. IPMN of the pancreas is reportable when stated as "IPMN with high-grade dysplasia," or "IPMN with an associated invasive carcinoma," or "IPMN with an associated in situ carcinoma." The case in SINQ 20021099 is stated to have "multifocal high grade dysplasia (so-called borderline tumor and carcinoma in-situ)" and is reportable because there is an explicit statement of carcinoma in situ, not because of the reference to the presence of high grade dysplasia. It is coded 8453/2 [Intraductal papillary-mucinous carcinoma, non-invasive]. |
2013 |
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20130088 | Grade--Heme & Lymphoid Neoplasms: Should Grade be coded to 5 [T-cell] or 9 [cell type not determined, not stated, not applicable] for anaplastic large cell lymphoma, NOS [9714/3]? See Discussion. | Under the Grade section in the Heme DB for anaplastic large cell lymphoma, NOS it indicates the following:
"Grade - Code grade specified by pathologist. If no grade specified, code 9."
There is no reference in the Grade section that we should look at the Abstractor Notes or a specific Module in the Heme DB for additional information. However, in the Abstractor Notes section it states, "Grade is T-cell (5) unless pathologist specifically designates as a B-cell (see G2 rule)." These two statements are conflicting. Which is the correct grade? |
Assign code 5 [T-cell] for anaplastic large cell lymphoma [9714/3] unless the pathologist specifies that the histology is a B-cell disease process. See Grade rule G2, Note 2.
In the Heme DB, there is a default value in the Grade field for histologies that do not have a grade specified. However, this particular histology does not default to code 9. There was an error in the Grade section of the 2010 and 2012 versions of Heme DB that has now been corrected in the latest release. |
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20130009 | Grade--Pancreas: Can the grade be coded when a biopsy is taken from the part of a primary tumor that has contiguously extended into an adjacent organ or structure? See Discussion. | The grade rule states to code grade from tissue removed from the primary tumor only, never from a metastatic site or a site of recurrence. There is no mention of whether the grade can be coded if only the contiguous site of involvement is biopsied when a single tumor directly extends to an adjacent tissue or organ. For example, is grade coded to 2 when a pancreatic tumor extends into the duodenum, and the duodenal biopsy confirms moderately differentiated adenocarcinoma consistent with a pancreatic primary? Or does the primary organ/site have to be biopsied in order to be able to code grade? | For one tumor involving a contiguous site, when there is no tissue specimen available from the primary site, you may code the grade based on the tissue from the tumor in the contiguous site.
This instruction is included in the upcoming grade instruction document. |
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20130155 | Diagnostic confirmation--Heme & Lymphoid Neoplasms: How do we code diagnostic confirmation if the pathology report states the diagnosis of a skin biopsy is "low-grade B cell lymphoma, most compatible with marginal zone lymphoma," genetic data includes positive rearrangement for immunoglobulin heavy chain gene favor a diagnosis of "B cell lymphoma," and the physician's clinical diagnosis is "cutaneous marginal zone lymphoma"? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code diagnostic confirmation to 3 [positive histology AND positive immunophenotyping studies (9590/3 - 9992/3)].
Immunoglobulin heavy and light chain genes rearranged is listed under Genetics Data in the Heme DB for 9699/3 [extranodal marginal zone lymphoma]. Given the documentation of this positive genetic finding and the positive bone marrow, code diagnostic confirmation to 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. |
2013 | |
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20130054 | MP/H Rules/Multiple primaries--Lung: How many primaries are accessioned if a lobectomy has two tumors that are both stated to be adenocarcinoma but the pathologist states they are synchronous primaries? See Discussion. | Left upper lung lobectomy: Adenocarcinoma, poorly-differentiated (grade 3), tumor size 1.2 cm, confined to lung. Second primary lung tumor: adenocarcinoma, well-differentiated (grade 1), tumor size 0.9 cm, confined to lung. Diagnosis COMMENT: The two tumors, although both adenocarcinoma, show markedly different histologies and thus are classified as synchronous primaries. Multiple synchronous primaries are staged separately according to the 7th edition of AJCC.
The AJCC Staging Manual 7th ed states, "Multiple tumors may be considered to be synchronous primaries if they are of different histological cell types. When multiple tumors are of the same cell type, they should only be considered to be synchronous primary tumors if, in the opinion of the pathologist, based on features such as ..., they represent different subtypes of the same histologic cell type..."
In this case, the pathologist insists these are two synchronous primaries, although different subtypes are not given, because the tumors have different grades and look completely different under the microscope. The MP/H rules indicate this is a single primary. How many primaries are accessioned? |
For cases diagnosed 2007 or later, accession a single primary, adenocarcinoma [8140/3] of the left upper lobe lung [C341]. 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 Lung MP rules because site specific rules exist for this primary.
Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. The patient has two tumors in the same lung with the same histology.
Do not use the AJCC Manual to make multiple primary decisions. Use the MP/H Rules to determine the number of primaries to accession. |
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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. |
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20130050 | Multiple Primaries/Primary site/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the primary site and histology for each if a 6/12/12 left shoulder mass specimen suspicious for large B-cell lymphoma is followed on 7/10/12 with three skin nodules excised from the back with a diagnosis of "composite lymphoma? See Discussion. | 6/12/12 Excisional biopsy left shoulder soft tissue mass: Suspicious for large B-cell lymphoma.
7/10/12 Excisional biopsy three skin nodules of back: "Composite lymphoma" - primary cutaneous anaplastic large cell lymphoma (CD3 pos, CD4 pos, CD30 pos, ALK neg with partial loss of CD5) and CONCURRENT cutaneous follicular center lymphoma (CD20 pos, PAX5 pos, BCL-6 pos, partially CD10 pos) and flow cytometry revealed results compatible with involvement by a lymphoproliferative disorder of T-cell lineage.
Per imaging performed, there was no involvement of lymph nodes or other organs.
Is the primary site C449 Skin, NOS and histology 9718/3 [Lymphoma, primary cutaneous anaplastic large cell] be correct? |
Code primary site to C445 [skin, back] and histology to 9718/3 [cutaneous anaplastic large cell lymphoma] .
Per Rule M6, abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location. For this case, there is cutaneous follicular (follicle) center lymphoma (9597/3) and cutaneous anaplastic large cell lymphoma (9718/3).
Per Rule PH22, code the primary site to the site or origin (skin, back) and the histology to the NHL with the numerically highest ICD-O-3 code. In this case, that would be 9718/3. |
2013 |
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