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Report Produced: 03/26/2023 08:38 AM

Report Question ID (Ascending) Question Discussion Answer

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.


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.


Solid Tumor Rules (2018)/Multiple primaries--Colon: Is a colorectal anastomotic site recurrence reportable, that is, a second primary, per Rule M7, third bullet, if there is no mention of mucosa but the tumor is seen on colonoscopy? See Discussion.

Colon, Rectosigmoid, and Rectum Multiple Primary Rule M7 states, Abstract multiple primaries when a subsequent tumor arises at the anastomotic site AND the subsequent tumor arises in the mucosa.

We identified tumors at the anastomotic site of previous colon primaries with no mention of mucosa in any of the available documentation. Are there any other indicators that would imply a tumor arising in the mucosa, or do we need this specific statement to apply rule M7?

Example: Patient has a history of invasive ascending colon adenocarcinoma diagnosed in October 2017 status post hemicolectomy followed by adjuvant chemo. There is no documentation of disease until August 2019 colonoscopy which shows a mass in the ileocolic anastomosis. Biopsy of the anastomotic site is positive for adenocarcinoma consistent with recurrence of the patient's colonic adenocarcinoma. There is no mention of mucosa found on the pathology report.

Abstract a single primary using 2018 Colon Solid Tumor Rule M8 in the example provided as there is a subsequent tumor occurring less than 24 months in the anastomotic site, with the same histology and no mention of mucosa.

The new tumor would be a new primary when it meets any one of the criteria noted in M7. The tumor does not have to be stated to have arisen in the mucosa. M8 also has three options to determine if a single primary is present.


Solid Tumor Rules (2018)/Multiple primaries--Lung: How many primaries are there and what M rules apply for multiple lung histologies in the left lower lobe (LLL) and right upper lobe (RUL) of the lungs? See Discussion.

There is one tumor in the left lung that is acinar adenocarcinoma, 8551/3, and two tumors in the right lung, one of which is 8551/3 and a separate one that is mucinous adenocarcinoma 8253/3.

3/21/18- left robotic video assisted thoracoscopy with left lower lobe lobectomy: 2.5 cm adenocarcinoma, acinar predominant, margins negative

11/3/18- right upper lobe lobectomy: invasive mucinous adenocarcinoma, 1.7 cm, invasive adenocarcinoma, acinar predominant, 0.6 cm, margins negative

If you start by comparing the 8551/3 left lung tumor to the 8253/3 right lung tumor, M6 applies and these would be separate primaries (seq 01 and seq 02). How would we handle the third tumor, 8551/3, in the right lung? Seq 01: 3/21/18- left lung primary 8551/3 Seq 02: 11/3/18- right lung primary 8253/3 Is the right lung tumor 8551/3 a third primary, and if so, which M rule applies? I cannot find a rule that seems to fit completely. Rule M6 may apply if you were comparing the right 8551/3 tumor to the seq 02 8253/3 tumor. But how would you know to use the seq 02 histology code 8253/3 or seq 01 histology code 8551/3 for the comparison? I think M9 was designed for situations where you have multiple tumors involving both lungs but they didn't biopsy all of them. Is that correct? If so, then we would be able to bypass M9. Would M11 apply since we already took care of two of the tumors with rule M6? If M11 doesn't apply, it seems like you would get to M14.

Abstract two primaries applying Rules M6 and M9 s follows.

First, assign a histology for each tumor.

--LLL adenocarcinoma, acinar predominant 8551/3

--RUL invasive mucinous adenocarcinoma 8253/3

--RUL invasive adenocarcinoma, acinar predominant 8551/3

For the RUL, this is two primaries according to Rule M6, to subtypes in Column 3 of the histology table.

For the LLL and RUL, this represents the same primary as these are the same histology according to Rule M9.


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.


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.


Solid Tumor Rules/Multiple primaries--Brain and CNS: What M rule applies to a clinically diagnosed right-sided parietal meningioma undergoing active surveillance, followed by a left-sided frontal anaplastic oligodendroglioma? See Discussion.

The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma.

The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor.

Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states:

--Patient had a resection of the non-malignant tumor (not the same tumor) OR

--It is unknown/not documented if the patient had a resection.

This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor?

Apply 2018 Malignant CNS Solid Tumor Rule M5 and abstract multiple primaries when there are multiple CNS tumors, one of which is malignant /3 and the other is non-malignant /0 or /1. According to Note 3, a non-malignant CNS tumor and a malignant CNS tumor are always multiple primaries (timing and primary sites are irrelevant). Prepare two abstracts; one for the non-malignant and another for the malignant tumor.


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:

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.


Histology--Brain and CNS: What morphology code should be assigned to a low-grade glial/glioneuronal neoplasm? See Discussion.

Pathology Diagnosis: Left temporal lesion - Low grade glial/glioneuronal neoplasm BRAF mutant. Pathologist Comment: The histopathological appearance of this lesion does not allow for a definitive diagnosis. However, the low-grade appearance, fibrillary nature, immunohistochemical profile, and the presence of a BRAF V600E mutation allow this to be categorized as a low-grade glial or possibly glioneuronal tumor. Despite the lack of exact classification this neoplasm can be expected to behave in a very indolent manner consistent with a WHO grade I classification.

Assign 9413/0 for glioneuronal neoplasm.

We consulted with our expert neuropathologist about the histology "glioneuronal neoplasm." This term is relatively new and has not yet been recognized by WHO or assigned an ICD-O code. Until such time that WHO determines a code for this neoplasm, our expert instructed us to use 9413/0. Since this is not a recognized neoplasm it is not included in the solid tumor rules.


Tumor Size--Esophagus: Can information from the endoscopy procedure that implies a size of 3 cm for Tumor Size--Clinical be used for Esophagus? See Discussion.

1-28-2018 CT Scan: 2.4 cm mass

2-15-2018 Endoscopy: Mass was present 22 to 25 cm. Biopsies were taken with cold forceps for histology; biopsy positive.

For the case you describe, we would record the clinical tumor size stated on the CT report.

The priority order for clinical tumor size is as follows.

1. Biopsy or operative (surgical exploration) report

2. Imaging

3. Physical exam

We do not recommend coding tumor size based on an inferred tumor size from a description such as "Mass was present 22 to 25 cm." Look for an actual measurement of the mass, or a stated tumor size. Use text fields to record details.