Report | Question ID | Question | Discussion | Answer | Year |
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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). |
2019 |
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20190095 | Reportability/Histology--Thymus: Is a thymoma a malignancy if described as having separate tumor nodules within peri-thymic adipose tissue? See Discussion. |
Patient had a thymectomy including pericardial fat for a mediastinal mass found incidentally during lung screening. Final diagnosis is WHO B3 thymoma. Staging Summary lists transcapsular invasion: "Present, as separate tumor nodules within peri-thymic adipose tissue." Tumor extension is stated to be "Confined to thymus, including peri-thymic adipose tissue." The pathologist staged this resection as pT1a pNX with no mention of mets. Clinically, there are no noted metastatic sites and no further treatment is planned. |
Report this case as a malignant thymoma. Our expert pathologist consultant reviewed this case and in his opinion, the "separate tumor nodules within peri-thymic adipose tissue" fit registry reporting criteria for separate tumor nodules making this a malignant thymoma. |
2019 |
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20190019 | Solid Tumor Rules 2018/Histology--Brain and CNS: How is histology coded for a single meningioma tumor when the histology is a meningioma comprised of multiple specific subtypes/variants? See Discussion. |
Example: Patient has a left cerebral meningioma that is meningothelial meningioma (9531) and two right-sided cerebral meningiomas: one that is transitional meningioma (9537) and the other that is meningioma, transitional and angiomatous, WHO Grade I. If the histology for the mixed tumor is 9534 (angiomatous meningioma), then there are three primaries. If the histology is 9537 (transitional meningioma), then there are two primaries. Per Table 6, angiomatous meningioma is 9534/0 and transitional meningioma is 9537/0. There is no mixed histology coding rule, or mixed histology meningioma code. There is also no default rule that would instruct registrars to code the numerically higher ICD-O code or to default to a meningioma (NOS) histology code. |
Code the histology for the meningioma, transitional and angiomatous, WHO Grade I to Meningioma, NOS (9530/0). Since a mixed meningioma ICD-O code has not been proposed by WHO, we consulted with our expert neuropathologist. The other option is to follow back with the pathologist and code what they feel is the predominant type. A new histology rule for coding mixed meningiomas will be added in a future update of CNS rules. |
2019 |
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20190049 | Lymph nodes/Melanoma: Is a single axillary lymph node regional or distant for a patient diagnosed in 2018 with metastatic melanoma to the brain found via imaging. The staging procedure was an single axillary lymph node excision that was positive for metastatic melanoma. The exact site of the primary was never determined; the primary site is coded to C449. See Discussion. |
The patient was diagnosed in 2018 with met melanoma to the brain found via imaging. The staging procedure was a single axillary lymph node excision which was positive for metastatic melanoma. The exact site of the primary was never determined and the site code is C449. Is the axillary lymph node regional or distant? This affects how I code regional lymph nodes positive, regional lymph nodes examined, and scope of regional lymph node surgery or surgical procedure other site. Similar question was asked in the past (question # 20091101) but I have not found this question restated since the 2018 changes and just want to verify this is still what we are to do. |
Lymph node mets from a melanoma of unknown primary site are presumed to be regional if the lymph node mets are confined to one area, as they are in this case. We are assuming there are no previous melanoma diagnoses for this patient. The workup should include examination of the skin areas that drain to the axillary area. |
2019 |
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20190048 | Reportability/Histology--Skin: Is malignant hidroacanthoma simplex of the scalp reportable? If so, what is the histology? |
Malignant hidroacanthoma simplex of the scalp is reportable. Malignant hidroacanthoma simplex is a synonym for porocarcinoma, 8409/3. |
2019 | |
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20190102 | Solid Tumor Rules/Histology--Head & Neck: What is the histology code of an external ear lesion when the dermatopathology report is the only available information (follow-up with the physician or pathologist is not possible) and the final diagnosis is malignant spindle cell neoplasm, most consistent with atypical fibroxanthoma? See Discussion. |
There are two histologies provided in the final diagnosis, malignant spindle cell neoplasm (8004/3) and atypical fibroxanthoma (8830/3). There is a definitive diagnosis of the non-specific histology, but the more specific histology is only described using ambiguous terminology. The external ear (C442) is included in the Head and Neck schema for diagnosis year 2018 and later. The Head and Neck Histology Rules indicate ambiguous terminology cannot be used to code a more specific histology. So ignoring the atypical fibroxanthoma, because it is modified by ambiguous terminology, we are left with a non-reportable site and histology combination (C442, 8004/3). Diagnoses of malignant atypical fibroxanthomas are regularly diagnosed using the syntax above in our area. Follow-up with the physician or pathologist is generally not possible as these cases are received from dermatopathology clinics only. The pathology report is the only information that will be received. If the reportable diagnosis of malignant atypical fibroxanthoma is ignored per the current Solid Tumor Rules, incidence cases will be lost. |
By definition, atypical fibroxanthoma (AFX) is a diagnosis of exclusion. Markers of specific differentiation must be negative. As written in your example, neither histology is reportable for skin. If possible, clarify the behavior of the AFX (8830/1) with the pathologist to determine reportability of the case. |
2019 |
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20190042 | Solid Tumor Rules (2018)/Multiple Primaries--Breast: Is a breast resection showing invasive mucinous carcinoma in a single tumor with associated ductal carcinoma in situ and additional findings of a background of lobular carcinoma in situ single or multiple primaries and which M rule applies? See Discussion |
Example: Right breast core biopsy found ductal carcinoma in situ in the upper outer quadrant. Subsequent resection has a final diagnosis of invasive mucinous carcinoma, grade 1, measuring approximately 7 mm, with close margins. See staging summary. Gross description mentions only the primary tumor with associated marker clip from previous biopsy. Breast Cancer Staging Summary lists (testing and margins removed for brevity): Procedure type: Lumpectomy. Specimen laterality: Right. Tumor size: 7mm. Histologic type: Invasive mucinous carcinoma. Histologic grade (Nottingham histologic score): Grade 1, (score 5/9). Tumor focality: Single focus. Lymph-vascular invasion: Not identified. Treatment effect: No known therapy. Ductal carcinoma in situ (DCIS): Present. Architectural pattern: Cribriform. Nuclear grade: Grade 1. Necrosis: Not identified. Calcifications: Not identified. Estimated size/extent of DCIS: Spanning an area measuring 15mm. Pathologic stage: pT1b, pNx. (AJCC 8th ed). Distant metastasis: Not applicable. Additional findings: Background lobular carcinoma in situ (LCIS), flat epithelial atypia (FEA), and atypical ductal hyperplasia (ADH). |
Apply Breast Solid Tumor Rule M3, abstract a single tumor when there is a single tumor, as there is reference to the primary, single 7 mm tumor. Apply Rule H7 and code the invasive histology only, mucinous carcinoma, when both invasive and in situ components are present. The rules state: Do not use Table 2 Histology Combination Codes for tumors with both invasive and in situ behavior. |
2019 |
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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. |
2019 | |
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20190077 | Summary Stage 2018/EOD 2018: How should SEER Summary Stage 2018 be coded for a 2018 thymus primary which has mediastinal fat invasion without mediastinal pleural involvement? See Discussion. |
The Extent of Disease (EOD) manual states that "Confined to thymus WITH mediastinal or pleural involvement" should be coded as regional by direct extension. I have EOD primary tumor coded as 200 and based on SEER*RSA, this is localized. |
Code 200 derives Regional Extension (RE) for Summary Stage; however, based on the information you provided, thymus primary with mediastinal fat invasion without mediastinal pleural involvement, EOD Primary Tumor would be coded to 100: Confined to thymus (encapsulated tumor), which includes extension into the mediastinal fat; No mediastinal or pleura involvement. This derives "Localized" for Summary Stage. Per AJCC T1, extension into the mediastinal fat is separate from involvement of the mediastinal pleura. For Summary Stage 2018, this would be code 1, Localized only (localized, NOS): Confined to thymus, NOS; No mediastinal or pleura involvement or UNKNOWN if involved. We will note that "extension into the mediastinal fat" is included in code 100 for the next release (September 2020). |
2019 |
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20190098 | Solid Tumor Rules (2018)/Multiple primaries--Breast: How many primaries are there and how is histology coded for a breast primary showing encapsulated papillary carcinoma and Paget disease of the nipple? See Discussion. |
Patient has a 1.7 cm encapsulated papillary carcinoma staged as pTis located 2 cm from the nipple and Paget disease of the nipple on mastectomy pathology. There is no indication in Table 3: Specific Histologies, NOS/NST, and Subtypes/Variants that encapsulated papillary carcinoma is a subtype of ductal carcinoma. Rule M8 notes that if the histology of the underlying tumor is any histology OTHER THAN duct or subtypes of duct, one should continue through the rules. But if M9 applies to this case, then incidence reporting will be increased in comparison to prior years. |
Abstract multiple primaries when there is Paget disease (8540/3) and an underlying tumor that is not duct, in this case, encapsulated papillary carcinoma (8504/2) using Rule M9 of the 2018 Breast Solid Tumor Rules. |
2019 |