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20190064 | Multiple Primaries--Heme & Lymphoid Neoplasms: Patient is diagnosed with myelodysplastic syndrome (MDS) with an early/evolving acute myeloid leukemia (AML) thought to be treatment related. Does rule M11 apply since there are two biopsies within 21 days, and therefore, two primaries, or one primary (9920/3)? See Discussion. |
Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation. On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm. MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given. On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts. |
Code as one primary (9920/3). This case does not fit the rules very well, since it is a treatment-related neoplasm and involves a transformation of MDS to AML during the clinical workup. Per the abstractor notes for 9920/3, code 9920/3 when the physician comments that the neoplasm is treatment related. This can be for the MDS or the AML. Use text fields to document that it was first referred to as MDS and then transformed to AML. If you followed the rules strictly and coded this as two primaries (the MDS and AML), you would lose the information that this was treatment related, which is more important. |
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20190094 | Reportability/Heme & Lymphoid Neoplasms--Skin: Is elephantiasis nostras verrucosa (ENV) reportable as a lymphoma? See Discussion. |
The autopsy report indicated a diagnosis of: Skin: Hyperkeratosis and pseudoepitheliomatous hyperplasia as well as reactive angioendotheliomatosis indicating Elephantiasis Nostras Verrucosa. |
Elephantiasis nostras verrucosa (ENV) is not reportable. ENV is a rare form of chronic lymphedema caused by any number of conditions including neoplasms, trauma, radiation treatment, congestive heart failure, obesity, hypothyroidism, chronic venous stasis, and parasitic infection. |
2019 |
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20190104 | Histology--Corpus uteri: Is 8020/3 used for a predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma diagnosed in 2018? See Discussion. |
After a little research, it appears as though Endometrial Dedifferentiated carcinoma is a relatively new term and is set to be included in ICD-O-3.2: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577 If you look at the link on that page for All Additions, Changes, and Revisions to the ICD-O-3, 1st Revision for ICDO-3.2, there is 8020/3 Dedifferentiated carcinoma. Currently, 8020/3 is Carcinoma, undifferentiated, NOS. For 2018 diagnosis, would you use 8020/3 for a predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma as stated in the pathology: Uterus, bilateral ovaries and fallopian tubes; supracervical hysterectomy/BSO: Predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma in the endometrium, FIGO grade 1 Portion of omentum, omental/anterior abdominal wall/ round ligament/uterine/small bowel mesenteric tumor nodules all involved by dedifferentiated carcinoma. Synoptic reads as follows: Histological Type: Endometrioid carcinoma, NOS Dedifferentiated carcinoma predominantly Histological Grade: Endometrioid carcinoma, FIGO grade 1. |
Assign code 8380/3 for endometrioid carcinoma, NOS as this is listed as the histological type in the synoptic report. |
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20190046 | Tumor Size/Bladder: The 2018 SEER Coding and Staging Manual says to use imaging over physical exam as priority for determining tumor size. If a bladder tumor is 4 cm visualized on cystoscopy, and is 2.8 cm on CT scan, which should be used as the clinical size? Is cystoscopy (endoscopy) a clinical exam or imaging? |
For the case described here, use the size from the CT scan. Physical exam includes what can be seen by a clinician either directly or through a scope. A tumor size obtained visually via cystoscopy is part of a physical exam. Therefore, the imaging (CT) tumor size is preferred. Use text fields to describe the details. |
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20190006 | MP/H Rules/Multiple primaries--Breast: Please confirm Multiple Primaries/Histology Breast Rule M8 applies in this 2017 case. The surgical resection is >60 days past the biopsy date but is it possible treatment plans for breast could span >60 days and this is one primary? See Discussion. |
7/25/17 Part A: Left breast at 8:00, 5 CFN: Specimen type: Stereotactic biopsy. Tumor type: Ductal carcinoma in situ (DCIS), cribriform type. Tumor size: The largest focus of DCIS measures 1 mm in greatest dimension as measured on the slide. Nuclear grade: 2 (Intermediate grade). Microcalcifications: Present. Other findings: Stromal fibrosis, microcalcification and fat necrosis. 11/1/17 A. Sentinel lymph node, left: One lymph node, negative for metastatic tumor on three levels of routine H\T\E and pan cytokeratin immunohistochemical stains. B. Left breast: Procedure: Total mastectomy with skin and nipple. Specimen Laterality: Left. Lymph Node Sampling: Yes, portion A. Specimen Integrity: Intact. Histologic Type: Extensive ductal carcinoma in situ and one focus of Invasive ductal carcinoma with mucinous features. Histologic Grade (Nottingham Histologic Score): Glandular Differentiation: Score 3 Nuclear Grade: Score 2. Mitotic Count: Score 1. Total Nottingham score 6 (grade 2, moderately differentiated). Tumor Size: 3.3 x 2 mm (0.33 x 0.2 cm) measured on slide (B3). Tumor Site: Lower inner quadrant of left breast. Tumor Focality: Unifocal. Ductal Carcinoma In Situ (DCIS): Present, cribriform, solid and micropapillary types with focal necrosis and calcifications. Size of DCIS: Number of blocks examined: Thirty (30). Number of blocks with DCIS: Thirteen (13). Lobular Carcinoma In Situ (LCIS): Not identified, Lymphovascular Invasion: Present. Perineural Invasion: Not identified. Other Findings: Changes consistent with previous biopsy site. Cysts, foci of atypical ductal hyperplasia, focal ductal hyperplasia, adenosis, stromal fibrosis and microcalcifications. Skin (epidermis): Uninvolved. Nipple: Uninvolved. Margins: 1 mm from DCIS to the closest deep margin (slide B12). At least 10 mm (1 cm) from invasive carcinoma to deep margin. Estrogen receptor (ER, clone 1D5) by immunohistochemistry performed on this material: Positive (invasive and in situ carcinoma), high intensity, in greater than 95% of carcinoma cells. Progesterone receptor (PR, clone 16) by immunohistochemistry performed on this material: Positive (invasive and in situ carcinoma), moderate intensity in about 80% of the carcinoma cells. Her 2 by FISH performed on this material: Pending, an addendum to follow. Pathologic staging: pT1aN0(sn)MX (AJCC 7th edition). Dictated by: (Pathologist), MD Intradepartmental review. |
Abstract a single breast primary. Apply MP/H Rule M3 as this is a single tumor identified in the biopsy at 8 o'clock and at the same location in the mastectomy specimen. Code the behavior as invasive according to rule H9. The first course of therapy ends when the documented treatment plan is completed, no matter how long, unless there is progression, recurrence, or treatment failure. |
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20190043 | Diagnostic Confirmation: How is Diagnostic Confirmation coded for malignancies diagnosed by a FoundationOne Liquid biopsy/assay involving circulating tumor DNA in blood only? See Discussion. |
Example: FoundationAct assay of circulating tumor DNA in blood sample results: Tumor type = non-small cell lung carcinoma, NOS, with 3 genomic alterations identified: NRAS Q61H, IDH2 R140Q and TP53 V172F. The tumor was identified on imaging and the imaging findings were not clearly what one would expect to see with a SCLC. |
Code Diagnostic Confirmation as 7, Radiology and other imaging techniques without microscopic confirmation for this case. Results of a FoundationOne Liquid biopsy/assay are not specific enough to diagnose this lung malignancy. |
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20190010 | Reportability/Histology--Bladder: Is papillary urothelial neoplasm of low malignant potential (PUNLMP) (8130/1) reportable when also referred to as papillary transitional cell carcinoma, grade 1, no invasion (8130/2) previously? See Discussion. |
The pathology report reads: Urinary bladder, tumor over right ureteral orifice, biopsy: Urinary bladder mucosa (urothelium) and submucosa (lamina propria), with papillary urothelial neoplasm of low malignant potential (previously known as papillary transitional cell carcinoma, grade 1 of 3), no invasion identified. |
This case is not reportable. PUNLMP (8130/1) is the diagnosis stated by the pathologist for this case and PUNLMP is not reportable. The information in parentheses is informational in this case and does not change the pathologist's diagnosis. According to WHO Classification of Tumors of the Urinary System and Male Genital Organs, 4th edition, there is variation of architectural and cytological features between PUNLMP and papillary urothelial carcinoma, low grade, reflecting grading changes from an older classification system. |
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20190090 | Update to current manual/EOD 2018/Summary Stage 2018--Fallopian Tube: How are behavior, EOD Primary Tumor, and Summary Stage 2018 coded for a diagnosis of serous tubal intraepithelial carcinoma (STIC) of the fallopian tube? See Discussion. |
The 2018 ICD-O-3 Histology Updates table lists serous tubal intraepithelial carcinoma (C57.0) with a behavior code of 2. The EOD Primary Tumor schema for Fallopian Tube shows STIC has an extension code of 100. It also maps code 100 to Summary Stage 2018 L (localized). Summary Stage 2018 for fallopian tube only documents that intraepithelial tumors are summary stage 0 (in situ). |
We are aware of the issue and have been in discussion with standard setters (SEER, NPCR, AJCC, and NAACCR). At this time, we recommend coding: Histology: 8441/2 Extent of Disease (EOD) Primary Tumor: 000 Summary Stage: 0 AJCC Clin/Path T would be 88, since all in situ lesions are not applicable. Edits will not allow you to have a 8441/2 with a T1. Also, EOD is not currently set up to derive the correct T value, unless you code 100. The change to address the issue will take effect in 2021. |
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20190053 | Solid Tumor Rules (2018)/Histology--Brain and CNS: What is the histology code for a central nervous system (CNS) Ewing sarcoma family tumor with CIC alteration of the right parietal lobe? See Discussion. |
Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors. While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin. |
Code as 9364/3. WHO Classification of Tumors of the CNS, 4th edition, refers to Ewing sarcoma/peripheral primitive neuroectodermal tumor as a tumor of neuroectodermal origin involving the CNS either as a primary dural neoplasm or by direct extension from contiguous bone or soft tissues (such as skull, vertebra, or paraspinal soft tissue). |
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20190016 | Update to current manual/SS2018--Breast: Should Code 3 of the Summary Stage 2018 (SS2018) for Breast designate the intramammary and infraclavicular lymph nodes as being ipsilateral? Similarly, should Code 7 designate infraclavicular lymph nodes as contralateral/bilateral? Laterality (ipsilateral, contralateral/bilateral) is included for axillary and internal mammary nodes in the respective codes. |
Based on your question, a review of the AJCC manual was done to clarify how these nodes would be coded. A review of Extent of Disease (EOD) Regional Nodes and EOD Mets was also done. That information is correct and in line with AJCC 8th edition. We apologize that SS2018 was not updated accordingly and thank you for bringing this issue to our attention. Per AJCC, infraclavicular and intramammary nodes are ipsilateral for the N category. Contralateral or bilateral involvement are included in the M category. The following will be applied to the planned 2020 update of the SS2018 manual. Code 3 Ipsilateral will be added to Infraclavicular and Intramammary Infraclavicular (subclavicular) (ipsilateral) Intramammary (ipsilateral) Code 7 The following will be added under Distant lymph nodes Infraclavicular (subclavicular) (contralateral or bilateral) Intramammary (contralateral or bilateral) |
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