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20210044 | Diagnostic Confirmation--Heme & Lymphoid Neoplasms--Plasma Cell Myeloma: Can serum protein electrophoresis (SPEP) be used as a definitive diagnostic method in the absence of a bone marrow biopsy? Is it appropriate to assign code 5 (Positive laboratory test/marker study) if there is no histological confirmation? See Discussion. |
Patient was diagnosed with lambda myeloma based on the M spike found on serum protein electrophoresis. A bone marrow biopsy was performed, but it was an insufficient sample. SPEP is not listed in the Hematopoietic Database as a lab test that can be used as a definitive diagnostic method. Since the physician did base the diagnosis on the SPEP result, would it be appropriate to assign code 5 (Positive laboratory test/marker study) since there was no histological confirmation? Under code 5, the Hematopoietic Manual states: Laboratory tests are listed under Definitive Diagnostic Methods in the Hematopoietic Database. |
Assign code 5 in Diagnostic Confirmation. We consulted with an expert hematopathologist who stated that SPEP would qualify for a diagnostic confirmation code of 5. He also stated that normally a SPEP is followed by a bone marrow biopsy. SPEP has been added to the Definitive Diagnostic Methods for plasma cell myeloma (9732/3). |
2021 |
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20210003 | Solid Tumor Rules (2018)/Primary Site--Head & Neck: The instructions for Table 9 of the Head and Neck Solid Tumor Rules instruct registrars to code the primary site to C479 (Autonomic nervous system) for paragangliomas that arise in the head and neck region, but the ICD-O-3.2 provides a site-associated code for most of these tumors (C754, Carotid body and C755, Paraganglion). Which primary site is correct? See Discussion. |
While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755. Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755. |
Always code primary site to the site of origin. Look for information about where the neoplasm originated. Primary site should always be coded to reflect the site of origin according to the medical opinion on the case. Always code the primary site based on where the tumor arose / site of origin. Site of origin may be indicated by terms such as "tumor arose from," "tumor originated in," or similar statements. Refer to ICD-O-3.2 and ICD-O-3 for topographty codes that are associated with specific histologies whenthe medical documentation does not specify the primary site. |
2021 |
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20210049 | Histology/Heme & Lymphoid Neoplasms--Leukemia: Is this the correct histology for a case of acute myeloid leukemia (AML) with recurrent genetic abnormalities? If the only information was AML with recurrent genetic abnormalities,"what code would you use: AML, NOS (9861/3) or AML with recurrent genetic abnormalities (9896/3)? See Discussion. |
12/3/2020 Pathology: AML: Blasts 40% of nucleated cells. CD45 positive, CD34 negative, CD 117+, CD13 positive, CD33 positive in 59.6% and HLA-DR was dim and myeloperoxidase was dim. Cytogenetics normal karyotype. The next generation sequencing detected IDH 2p.(R172K)c515>A. Because this was AML NOS, we consulted with the physician. The physician stated the patient had AML with recurrent genetic abnormalities"and the basis for the diagnosis was the IDH-2 mutation identified on Next Generation Sequencing. We assigned 9896/3, based on the physician's interpretation of the pathology. This histology is being questioned. |
We found that the term AML with recurrent genetic abnormalities, NOS"was incorrectly included as an alternate name with code 9896/3. We followed back with our expert hematopathologist and he stated that this should have been coded to 9861/3 (AML, NOS), for AML with recurrent genetic abnormalities, NOS. This alternate name has been added to 9861/3. (Note: The same alternate name has been removed from 9896/3). IDH-2 is not listed as a genetic abnormality for any of the histologies listed in the database. It could be that this is a new genetic marker for one of the AML with recurrent genetic abnormalities that we are not aware of. Without further clarification on which histology the IDH-2 would indicate, you would have to default to 9861/3. There are several histologies that are grouped as AML with recurrent genetic abnormalities."All of these have specific genetics listed as part of the ICD-O-3 histology name. 9865: Acute myeloid leukemia with t(6;9)(p23;q34.1) DEK-NUP214 9866: Acute promyelocytic leukemia with PML-RARA 9869: Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM 9871: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11 9877: Acute myeloid leukemia with mutated NPM1 (2021+) 9878: Acute myeloid leukemia with biallelic mutation of CEBPA (2021+) 9879: Acute myeloid leukemia with mutated RUNX1 (2021+) 9896: Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1 9897: Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3 9911: Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1 9912: Acute myeloid leukemia with BCR-ABL1 (2021)+ Of note, for the above histologies, since these are diagnosed solely based on genetics, diagnostic confirmation will always be 3. This instruction will be added to the Hematopoietic database for the 2022 update. |
2021 |
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20210017 | Update to current manual/Mets at diagnosis fields--Lymphoma: Are distant metastases possible for a lymphoma with a primary site of lymph nodes? The instructions in the SEER manual tell us to assign code 8 in each of the Mets at Dx fields for a lymphoma originating in lymph nodes. |
This is a correction to the SEER manual. Lymphomas originating in lymph nodes (C77) could have distant metastases to any site except lymph nodes. The following corrections to the manual apply now and will appear in the next version of the manual. Remove C770-C779 from the instruction for assigning code 8 on the following pages. Page 135 Mets at Dx--Bone Page 137 Mets at Dx--Brain Page 139 Mets at Dx--Liver Page 141 Mets at Dx--Lung Page 145 Mets at Dx--Other Example Biopsy of axillary lymph node: Diffuse Large B-Cell lymphoma. Lymph nodes involved above and below the diaphragm, multiple nodules seen in lung, lesions in liver. Bone marrow biopsy positive for DLBLC. Per Hematopoietic manual, primary site would be C778 for multiple lymph node regions involved. Mets at Dx--Bone-0 Mets at Dx--Brain-0 Mets at Dx--Liver-1 Mets at Dx--Lung-1 Mets at Dx--Distant Lymph Nodes-8 Mets at Dx--Other-1 |
2021 | |
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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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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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20210066 | 2021 SEER Manual/Surgery of Primary Site--Lung: What is the correct surgery code for a left upper lobe (LUL) wedge resection (confirming adenocarcinoma) followed by a lingular-sparing LUL lobectomy and mediastinal lymph node dissection? Is the correct Surgery Code 22 since the lingula was not resected (not the whole LUL Lung)? Or should the appropriate surgery code be 33 (this surgery suffices to code to a lobectomy with the mediastinal lymph node dissection)? |
Assign code 22 for LUL wedge resection followed by a lingular-sparing LUL lobectomy and mediastinal lymph node dissection. Code the lymph node surgery in Scope of Regional Lymph Node Surgery. We obtained input from an expert who agrees with this code. He states a lingula-sparing lobectomy is best coded as a segmentectomy because it is the same as an apical trisegmentectomy. |
2021 | |
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20210069 | EOD 2018/Summary Stage 2018--Intrahepatic Bile Duct: How should Extent of Disease (EOD) Primary Tumor (PT) be coded for invasion of or into (but not through) the visceral peritoneum for an intrahepatic bile duct primary? See Discussion. |
Invasion of the visceral peritoneum is Regional (code 2) in Summary Stage. EOD PT code 500 is for invasion BEYOND the visceral peritoneum into adjacent connective tissues, and maps to T3 and Regional Summary Stage, but that code seems too extensive. All lower EOD codes map to Localized Summary Stage. |
Assign code 500 for EOD Primary Tumor for now. We have confirmed with AJCC that "invasion of" but not "through" the visceral peritoneum maps to a T2 and not T3. Involvement of the visceral peritoneum for Summary Stage is Regional and does not make a distinction between "invasion of" or "invasion through." Any involvement of the visceral peritoneum is regional. To correct this situation would require a new code, which would derive a T2/RE. That code will be added to the updates for 2023. Code 500 will derive the appropriate Summary Stage of 2 (Regional). We are aware that this will derive the incorrect T; however, there is no work around at this time that will derive the correct T and Summary Stage, so we are defaulting to deriving the correct Summary Stage. |
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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20210064 | Solid Tumor Rules (2018/2021)/Multiple primaries--Ovary, Fallopian Tube: How many primaries should be reported when patient has right fallopian tube high-grade serous carcinoma and bilateral serous tubal intraepithelial carcinoma (STIC)? See Discussion. |
Patient is diagnosed March 2021, with malignant pleural effusion, clinical impression supports either endometrial or tubo-ovarian primary and neoadjuvant chemotherapy is given. Subsequent total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) in July, shows high-grade serous carcinoma involving the right fallopian tube and bilateral ovaries, as well as bilateral STIC. Summary Stage lists tumor site as right fallopian tube, with the serous tubal intraepithelial carcinoma (STIC) noted under “additional findings.” Should the contralateral (left-sided) STIC be accessioned as an additional primary, per MP/H Rule M8, the since fallopian tubes are listed in Table 1 as Paired Organs with Laterality? |
Abstract as multiple primaries per rule M8. There are bilateral fallopian tube primaries. It sounds like the "primary" tumor was identified in the right fallopian tube with bilateral spread of disease. Incidental STIC was also identifed in the left fallopian tube. Do not record the STIC as another primary. |
2021 |
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