| Report | Question ID | Question | Discussion | Answer | Year |
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20200039 | EOD 2018/Summary Stage 2018--GIST: How should Extent of Disease (EOD) and Summary Stage be coded for a multifocal gastrointestinal stromal tumor (GIST)? See Discussion. |
Example: Patient is found to have a 9.4 cm GIST in the jejunum and 2 cm GIST in the stomach during resection, neither stated to be outright malignant. Similar to the instruction in SINQ 20190041, this case is coded as a malignant jejunal primary due to multifocal tumor. However, it is unclear how to account for the stomach tumor, or any other multifocal tumor for GIST, when coding EOD and Summary Stage. |
For this case, report each GIST diagnosis separately. This differs from SINQ 20190041 because in that case the stomach GIST was incidental and measured only 0.3 cm. Reporting these separately means that each one is no longer a multifocal tumor. If there is no other indication of malignancy for these, they would not be reportable if diagnosed in 2020 or earlier. For cases diagnosed 2021 or later, all GIST are reportable. Report this as two primaries. Use the new GIST schema for EOD and assign EOD Primary Tumor 100 for each. There is no mention of extension outside the primary site. Summary Stage is Localized for each. |
2020 |
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20200045 | Diagnostic confirmation--Heme & Lymphoid Neoplasms: Is Diagnostic Confirmation coded to 5 or 8 based on a patient diagnosed as multiple myeloma by a physician based on a bone marrow biopsy stating plasma cell neoplasm? See Discussion. |
Bone marrow, right iliac crest (aspirate smear, touch preparation, clot section and core biopsy): Hypercellular marrow (40-50%) with plasma cell neoplasm (see Comment): " No evidence of metastatic carcinoma. " Adequate iron storage. Comment: CBC data shows normocytic anemia. Flow cytometric analysis of bone marrow detects a kappa restricted plasma cell population that expresses CD138 and CD38. CD56 is positive. CD19 and CD20 are negative. T lymphocytes are immunophenotypically unremarkable. Polyclonal B lymphocytes are detected. Blast gate is not significantly increased. Immunohistochemical stains are performed on the biopsy core and clot section for greater sensitivity and further architectural assessment with adequate controls. CD138 positive plasma cells comprise > 70% of the total cellularity. AE1/AE3 is negative. Taken together, the morphologic and immunophenotypic findings are consistent with a diagnosis of plasma cell neoplasm. Trilineage hematopoietic activity as are seen. |
This would be a Diagnostic Confirmation of 8 based on the physician's diagnosis. The Pathology report mentions plasma cell neoplasm only. By itself, plasma cell neoplasm is not reportable because it includes a variety of diseases, some that are not reportable, and some that are (See Hematopoietic Database under Plasma Cell Neoplasm.) The physician probably has other information, including imaging, which may show lytic lesions. He/she is probably using clinical findings, plus findings from the bone marrow, and diagnosing this patient with multiple myeloma. |
2020 |
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20210039 | Multiple primaries/Heme & Lymphoid Neoplasms--Lymphoma: Is a 2021 right tongue base biopsy showing diffuse large B-cell lymphoma (DLBCL) (9680/3) a new primary following a prior history of hairy cell leukemia-variant (HCL-v) (9591/3) in 2011? See discussion. |
Patient was diagnosed with low-grade non-Hodgkin lymphoma in 2011, later classified as hairy cell leukemia-variant. Right cervical node biopsy in 2020 proved HCL-v and a subsequent 2021 right tongue base biopsy showed DLBCL. The tongue base biopsy path includes the comment, patient has history of HCL-v, but the morphology and flow cytology features are different from the patient's previous right cervical node biopsy. This DLBCL likely represents a second de novo lymphoma, but cannot exclude an unusual transformation of the prior HCL-v. Per Heme Rule M7, abstract a single primary when a more specific histology is diagnosed after an NOS if the Heme DB confirms the same primary. The histology code for HCL-v, 9591/3 is a non-specific code, but it seems like a specific histology. The Heme Calculator does say 9591 and 9680 are the same primary, but we are unsure if that is correct for this case of HCL-v followed by DLBCL. |
Abstract two primaries. This is a transformation from a chronic disease (the Hairy Cell Variant) to an acute disease (DLBCL). Although this rare situation is not clearly covered in the Hematopoietic rules, the fact that this was originally a Hairy Cell Leukemia variant means that the DLBCL is a new primary. |
2021 |
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20210051 | Primary site/Biliary tract--Ampulla of Vater: What is the correct primary site code for intra-ampullary and periampullary adenocarcinoma, C241 (8144/3) or C249? See Discussion. |
Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation. Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present. Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type. There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber. |
Assign C241. Ampulla (C241) includes both periampullary and intra-ampullary. |
2021 |
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20210062 | Histology/Reportability--Heme and Lymphoid Neoplasms: Is a case that is compatible with low grade myelodysplastic syndrome with multilineage dysplasia (MDS-MLD) reportable, and if so, is the histology plasma cell myeloma or myelodysplastic syndrome (MDS)? See Discussion. |
HL-7 e-path report, Final Diagnosis High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment. Comments Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation. Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization |
This case is reportable. Assign MDS, NOS (9989/3) based on the information provided for this case. “Compatible with” can be used for reportability; however, it cannot be used for assigning histology. There is no confirmed diagnosis of plasma cell myeloma/neoplasm; the comment specifically addresses the need for further evaluation of this case. |
2021 |
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20210046 | Reportability--Skin: Is dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation synonymous with dermatofibrosarcoma protuberans, fibrosarcomatous, and therefore reportable for diagnosis year 2021 and forward? See Discussion. |
Patient has a 2021 skin excision showing an atypical spindle cell neoplasm, most consistent with dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation. Per the ICD-O-3.2 Coding Table, DFSP, NOS has a behavior code of /1, and DFSP, fibrosarcomatous has a behavior code of /3. There is no code listed for DFSP with fibrosarcomatous transformation. Transformation is not included as a term that can/cannot be used for the Other Sites Schema, but this type of DFSP is often described as DFSP with fibrosarcomatous transformation. How do we code DFSP when transformation is used to describe fibrosarcomatous? |
Report DFSP with fibrosarcomatous transformation as it is synonymous with fibrosarcomatous DFSP (8832/3). According to the WHO Classification of Skin Tumors, 4th edition, fibrosarcomatous DFSP is a variant of DFSP and that fibrosarcomatous transformation is seen in approximately 10% of DFSP cases. It is characterized by an often abrupt transition of DFSP. |
2021 |
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20210076 | Reportability/Brain and CNS: Is a 2021 case of ecchordosis physaliphora (lesion within the prepontine cistern) on brain MRI reportable? |
Ecchordosis physaliphora is not reportable. |
2021 | |
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20210026 | Multiple primaries--Heme & Lymphoid Neoplasms--Lymphoma: Is a case initially submitted as C772 with histology coded 9591/3 (lymphoma, NOS) with a second case submitted as C162 with histology coded 9699/3 (extranodal marginal zone lymphoma of mucosal-associated lymphoid tissue (MALT lymphoma) a single primary or multiple primaries? See Discussion. |
The following cases were submitted to the central registry as separate primaries. First case submitted as C772 with histology coded 9591/3 (Lymphoma, NOS). Second case submitted as C162 with histology coded 9699/3 (MALT Lymphoma). Sequence 01 - 5/2016, Excisional biopsy pancreatic tail lymph node: suspicious for malignant B-cell lymphoma. No treatment recommended or administered. Sequence 02 - 2/2019, Stomach biopsy: MALT Lymphoma. Unknown if treatment was recommended or administered. Biopsy was only at this facility. Using the Hematopoietic and Lymphoid Neoplasm Multiple Primaries/Histology rules, Rule M7 makes this a single primary. Note 4 instructs to change the histology of the initial abstract to the more specific histology (9699/3). If this is done, they would be multiple primaries per the exception within Rule M2. Should the histology on sequence 01 be changed to the MALT lymphoma and the cases would be multiple primaries or is this a single primary? |
Abstract two primaries and assign Primary 1: C772, 9699/3 Primary 2: C162, 9699/3 Per Rule M7, you would change the first case to histology 9699/3 based on Note 4 under Rule M7, Note 4: Change the histology code on the original abstract to the more specific histology when the original diagnosis is in your registry database. Use previous editions of ICD-O (i.e., ICD-O-1, ICD-O-2) or the Hematopoietic Database to assign the code applicable to the year of diagnosis for the more specific histology. Per Rule M2 this would be the same primary based on both being the same histology; however, there is an exception for MALT lymphomas (9699/3), which states: Abstract multiple primaries when a nodal MALT (C770-779, 9699/3) occurs before or after an extranodal MALT (all other sites, 9699/3). |
2021 |
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20210015 | Solid Tumor Rules (2007/2021)/Multiple Primaries--Anus: Have the disease free interval criteria been met for the following case scenario. A patient was diagnosed with anal intraepithelial neoplasia (AIN) III in 7/2018 that was treated with local tumor destruction, followed by Pap smears and biopsies that prove AIN I or AIN II through 2020, before being diagnosed with a reportable AIN II or AIN III in 2021. See Discussion. |
Since AIN I is not reportable and AIN II is not reportable until 2021, we are not sure if we can say the patient was disease free because there was no intervening reportable tumor (AIN III), or was never disease free because there was evidence of related disease (lower grade dysplasia). |
The 2021 AIN III is not a new primary. According to our GI pathology expert, findings of AIN I and/or AIN II following a diagnosis of AIN III indicates the patient was never NED and indicates persistent disease. . |
2021 |
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20210034 | Reportability/Histology--Endometrium: Is endometrial hyperplasia with atypia equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable? |
Endometrial hyperplasia with atypia is equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable for cases diagnosed 2021 and later. Our expert pathologist consultant confirmed this for us. |
2021 |
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