Report | Question ID | Question | Discussion | Answer | Year |
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20110039 | Multiple primaries/Primary site--Heme & Lymphoid Neoplasms: What are the primary sites and how many primaries are abstracted, when 2004 diagnosis of grade 2 follicular lymphoma of the bilateral breasts is subsequently diagnosed with a 2010 diagnosis of diffuse large B-cell lymphoma (40%) and grade 3a follicular lymphoma (60%) of a left arm nodule? See Discussion. | Follicular lymphoma was diagnosed 7/2004, grade 2 per biopsy of the bilateral breasts. Bone marrow biopsy was positive for lymphoma involving 10% of bone marrow. Imaging showed extensive lymphadenopathy mainly in abdomen/pelvis with an 8 cm mass in the right pelvis. Smaller lymph nodes were noted in the left periaortic region and also some small precarinal lymph nodes. This was a stage IVA lymphoma. The patient had six cycles of CHOP/R with an excellent response. Per the clinician's notes on 12/2005, there was no evidence of recurrence or no sign of active disease. The plan was to follow up with the patient in 6 months.
08/22/06 imaging showed new disease in the bilateral chest wall. 8/2006 bilateral breast nodule biopsies, are positive for grade 1-2 follicular lymphoma. The patient was treated with Rituxan. Per clinician's 3/2007 note, no active disease is noted. Patient was regularly followed with no evidence of disease until 10/2010. At that time, the patient had a left arm nodule biopsy which was positive for diffuse large B cell lymphoma (40%) CD positive and grade 3a follicular lymphoma (60%). RICE was recommended due to "transformation" per oncologist. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M10, this case should be accessioned as two primaries when a neoplasm is originally diagnosed as a chronic neoplasm (is follicular lymphoma (FL), grade 2) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.
Code the histology for the first primary to 9691/3 [follicular lymphoma (FL), grade 2] and the primary site to bilateral C509 [breast, NOS]. FL can start as an extranodal disease; breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes.
Per Rule M4, abstract the 2010 disease process as a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Per Rule PH11 the primary site is coded to C779 [lymph nodes, NOS] and the histology is coded to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Rule PH11 states one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.
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. |
2011 |
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20200076 | Reportability/Solid Tumor Rules (2018)--Kidney: Should clarification (Notes) be added to Table 1 of the 2018 Kidney Solid Tumor Rules regarding the use of clear cell papillary renal cell carcinoma (8323) and sarcomatoid renal cell carcinoma (8312) as these histologies conflict with the ICD-O-3.2? See Discussion. |
First, reportability of clear cell papillary renal cell carcinoma changed from 8323/3 to 8323/1. Although it does not appear the standard-setters implemented this change, note of the conflict between the ICD-O-3.2 and the Solid Tumor Rules (STR) is not included in the Implementation Guidelines or STR. The current Note for clear cell papillary renal cell carcinoma (8323) was left in Table 1, so this presumably is still reportable. It would be helpful if the conflict with ICD-O-3.2 was addressed, especially since the existing Note refers to changes made back in 2016 (not 2018 or 2021). Second, is the term sarcomatoid renal cell carcinoma still coded as a synonym for renal cell carcinoma (8312) because sarcomatoid is referring to a pattern of differentiation or 8318 (renal cell carcinoma, sarcomatoid)? The STR, Table 1, lists sarcomatoid renal cell carcinoma as 8312, but the ICD-O-3.2 lists this as 8318. The Note in Table 1 still indicates WHO/IARC and College of American Pathologists agree that sarcomatoid carcinoma is a pattern of differentiation, not a specific subtype, of renal cell carcinoma. This appears to conflict with WHO/IARC ICD-O-3.2 Coding Table as it provides a different, specific histology code for this malignancy. How can WHO/IARC classify this both a pattern of renal cell carcinoma and a separate, specific histology? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
For cases diagnosed 2021 or later, use ICD-O-3.2 to determine reportability. Use the Solid Tumor Rules to determine the number of primaries to report and the histology to code for tumors that are reportable. Do not use the Solid Tumor Rules to determine reportability. ICD-O-3.2 was implemented by the North American standard setters as of 1/1/2021 and it is the basis for reportability for cases diagnosed as of 1/1/21. See 1.a on page 6 in the 2021 SEER manual, https://seer.cancer.gov/manuals/2021/SPCSM_2021_MainDoc.pdf WHO 4th edition Tumors of the Urinary System has proposed ICD-O code 8323/1 for clear cell papillary renal cell carcinoma. This has not been approved for implementation by the standard setters. Continue assigning 8323/3 for clear cell papillary renal cell carcinoma. Sarcomatoid RCC is listed as a synonym for RCC 8312/3. This is correct per WHO and our SME. Do NOT code sarcomatoid RCC to 8318/3. |
2020 |
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20061037 | Multiple Primaries/Histology--Lymphoma: If a gastric biopsy demonstrates large B cell lymphoma arising in a low grade MALT lymphoma, how many tumors should be abstracted and how should the histology field(s) be coded? See Discussion. | Final path for gastric biopsy on 12/2005 is "consistent with malignant lymphoma" and Micro says "morphologic findings consistent with MALT lymphoma and an increased proportion of large atypical cells is concerning for large cell transformation. However, since the large cells are present only focally, a definitive diagnosis of large cell lymphoma cannot be rendered" A second gastric biopsy a week later said: Final Path: Diffuse large B cell lymphoma arising in low grade MALT lymphoma. Micro says: "Compared to patient's previous biopsy...the current specimen contains a higher percentage of large atypical cells which stain positively for CD79a, a B cell marker. The morphologic and immunohistochemical findings are consistent with a large B cell lymphoma arising in a low grade MALT lymphoma." These are different primaries according to the table of single versus subsequent primaries of lymphatic and hematopoietic diseases. |
For cases diagnosed prior to 1/1/2010: This is one primary. Code as 9699 [Marginal zone B-cell lymphoma, NOS]. The first biopsy was not conclusive. The biopsy one week later was more definitive. The reports are describing a difference between specimens, not a difference in disease. According to the WHO classification, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is an extranodal lymphoma with B-cells, cells resembling monocytoid cells, small lymphocytes and scattered immunoblast and centroblast-like cells. For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ. |
2006 |
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20031035 | Reportability/Histology--Hematopoietic, NOS: Does the presence of sideroblasts on a bone marrow biopsy confirm a diagnosis of refractory anemia with sideroblasts? | Final path diagnosis of bone marrow biopsy:
I. Hypercellular marrow for age with trilinear hyperplasia. II. Decreased iron stores with decreased sideroblasts.
Comment: Although the overall picture is not diagnostic of a specific entity, it is most consistent with an early stage myelodysplastic syndrome which would best be considered refractory anemia at this point.
In this case the percentage of sideroblasts is not stated. Would the path diagnosis of "decreased sideroblasts" along with the path comment of "refractory anemia" indicate that this case should be coded to 9982/3 [Refractory anemia with sideroblasts]? |
For cases diagnosed prior to 1/1/2010:
For the hematologic diseases, do not accession the case unless there is a definite positive diagnosis. A positive diagnosis, such as "Refractory anemia" must be stated in order to code that diagnosis. Other words associated with the positive diagnosis, such as "sideroblasts" are NOT to be used alone to assume a diagnosis.
Decreased sideroblasts does not make a diagnosis of Refractory anemia with sideroblasts. The sideroblasts for 9982/3 [Refractory anemia with sideroblasts] are characteristic in rings, and are INCREASED to make the diagnosis.
Based on the information provided, this case is not reportable. The final path diagnosis is not a reportable disease. The comment further states that the overall picture is not diagnostic of a specific entity. Therefore, it should not be reported at this point.
For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ. |
2003 |
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20210024 | Primary Site--Vulva: What is the primary site of patient with an excision of a left vulvar cystic mass showing focal mammary-type ductal carcinoma in situ (DCIS) on 11/06/2020? See Discussion. |
Final Pathologic Diagnosis: Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment) Size of DCIS: 0.7 CM. Margins: Negative. Comment Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised. 12/01/2020 post op visit with surgeon: Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion. PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management. |
Code the primary site to vulva. Use text fields to record the details. According to the WHO classification, several types of primary vulvar mammary-like carcinoma have been reported. It is rare and is thought to arise from specialized anogenital mammary-like glands within the vulva. It does not arise from ectopic breast tissue and is does not represent metastatic breast carcinoma. |
2021 |
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20051055 | CS Lymph Nodes/CS Mets at Dx--Lung: In which CS field is a focus of squamous cell carcinoma in the soft tissue coded for a lung primary? See Discussion. | Final Pathologic Diagnosis: 1. Right upper lobe mass, lobectomy: Extensive well differentiated squamous cell carcinoma 2. Right hilar lymph nodes: No tumor identified in nine hilar lymph nodes. A focus of squamous carcinoma is present in soft tissue |
This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.Code a separate focus of squamous cell carcinoma in soft tissue in the CS Mets at DX field. Use this field to capture discontinuous metastasis. Code CS Mets at DX as 40 [Distant mets except distant lymph nodes] for the case described above. |
2005 |
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20120011 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Is there a timing rule used to recode histology should a more specific diagnosis of refractory anemia with excess blasts (RAEB) be confirmed after an initial diagnosis of myelodysplastic syndrome (MDS)? How many primaries are abstracted if RAEB subsequently evolves toward an acute myeloid leukemia? See Discussion. |
Facility A: 4/8/2010 Bone Marrow biopsy: Features most compatible with MDS. (No treatment administered.) 7/2/2010 Peripherial Blood: Transforming Myelodysplastic Syndrome (MDS). COMMENT: Clonal abnormality compatible with MDS/acute myeloid leukemia (AML) in all metaphases examined. (Still no treatment administered.) Facility B: 10/6/2010 Patient now presents for evaluation and treatment. Patient started on Vidaza. 10/07/10 Bone Marrow biopsy: Refractory anemia with excess blasts (RAEB-2) COMMENT: Evolution towards AML with myelodysplasia related changes considered; cytogenetic analysis reveals abnormalities most compatible with MDS and/or AML. Based on the Heme Manual and DB, the 4/8/2010 diagnosis of MDS, NOS (9989/3) is the first primary. Should the 7/2/2010 diagnosis of transforming MDS to AML (9861/3) be a new, second primary? Based on the Abstractor Note for MDS in the Heme DB for MDS, "If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis." Based on this note, should the MDS histology code [9989/3] be changed to refractory anemia with excess blasts (RAEB-2) [9983/3] from the biopsy taken on 10/7/2010 (one day after treatment began) that revealed RAEB-2 with evolution towards AML? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics." Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.] The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML. It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML. The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second 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. |
2012 |
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20130186 | Grade: Can the FIGO grade be used to code the morphologic grade? See discussion. |
FIGO Grade is coded in CS SSF 7 in the Corpus Uteri schema. The SEER Manual does not address using FIGO grade for coding grade in morphology. |
Do not use FIGO grade to code the grade field. See the sentence below the table in Instruction #6 in the Grade Coding Instructions for cases diagnosed 2014 and later, http://seer.cancer.gov/tools/grade/ |
2013 |
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20041080 | Behavior Code/CS Extension--Brain and CNS: How are these fields coded when the final diagnosis on pathology indicates that an atypical meningioma invades the brain and the bone flap specimen indicates extensive invasion through the full thickness of the calvarium? See Discussion. |
FDx on the path is: A. Rt frontotemporal brain tumor: Atypical meningioma, WHO grade II (out of III). B. Arachnoid tissue: Atypical meningioma with small focus of invasion into superficial brain and focal perivascular spread. C. Bone flap: Atypical meningioma with extensive invasion through full thickness of the calvarium. Comment: Although this tumor shows a small focus of brain invasion, it should be considered a grade II (out of III) meningioma based on its histologic atypia (cellularity, sheeting of tumor cells and prominent nucleoli), elevated Ki-67 index and low mitotic rate. |
This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.For tumors diagnosed prior to 2004, the example above is a benign meningioma and not reportable to SEER. For tumors diagnosed 2004 or later, code the behavior as 1 [Borderline malignancy]. Code CS Extension as 05 [Benign or borderline brain tumors]. According to expert consultant, meningiomas are in the lining cells for the inner table of the skull and as such have an affinity for bone that allows them to penetrate adjacent bone without being "malignant. |
2004 |
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20061045 | CS Lymph Nodes/CS Mets at Dx--Melanoma: How are these fields coded for a melanoma primary when melanoma is identified in lymph nodes but no primary skin tumor is found? See Discussion. | Excisional biopsy of an inguinal lymph node revealed metastatic melanoma. Multiple skin biopsies did not reveal the primary site. Subsequent lymph node dissection of superficial inguinal nodes showed microscopic focus of malignant melanoma in subcutaneous fat adjacent to previous procedure site. No evidence of metastatic melanoma in 7 lymph nodes. Dissection of external iliac lymph nodes showed no evidence of melanoma in 5 lymph nodes. |
This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2. Code CS Lymph Nodes 80 [Lymph nodes, NOS]. Code CS Mets at DX 00 [None]. Since it cannot be determined whether the lymph nodes are regional or distant, code CS Lymph Nodes to lymph nodes, NOS. |
2006 |