Report | Question ID | Question | Discussion | Answer | Year |
---|---|---|---|---|---|
|
20210024 | Primary Site--Vulva: What is the primary site of patient with an excision of a left vulvar cystic mass showing focal mammary-type ductal carcinoma in situ (DCIS) on 11/06/2020? See Discussion. |
Final Pathologic Diagnosis: Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment) Size of DCIS: 0.7 CM. Margins: Negative. Comment Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised. 12/01/2020 post op visit with surgeon: Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion. PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management. |
Code the primary site to vulva. Use text fields to record the details. According to the WHO classification, several types of primary vulvar mammary-like carcinoma have been reported. It is rare and is thought to arise from specialized anogenital mammary-like glands within the vulva. It does not arise from ectopic breast tissue and is does not represent metastatic breast carcinoma. |
2021 |
|
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 |
|
20210061 | First course treatment/Update to current manual: Should the instruction regarding expectant management in the 2021 (and 2022) SEER Manual include how to code for the patient’s decision to proceed with expectant management? See Discussion. |
Currently, First Course Therapy instruction for expectant management (also referred to as active surveillance, watchful waiting, etc.) instructs one to code 0 or 00 (not done) for all data items when the physician opts for expectant management. We find that the treatment decisions can be driven by the patient, physician, or combination of both patient and physician depending on the options presented. |
Instructions for First Course of Therapy include using the documented first course of therapy (treatment plan) from the medical record. While a patient may weigh in on the treatment decision, the physician is responsible for developing and managing the treatment plan including closely watching a patient’s condition but not giving treatment unless symptoms appear or change. We can add language to a future manual to clarify. |
2021 |
|
20210064 | Solid Tumor Rules (2018/2021)/Multiple primaries--Ovary: 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 |
|
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 |
|
20210058 | Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion. |
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored. 4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH. 5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia. 6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma. |
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3). The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern. In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis. We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns. |
2021 |
|
20210006 | Behavior/Summary Stage 2018--Colon: What is the correct behavior and Summary Stage for a case of intramucosal adenocarcinoma arising in tubular adenoma? AJCC states this is Tis, though SEER Summary Stagie states this is Localized (code 1). The histology is 8140/2 (adenocarcinoma in situ), but the SEER Summary Stage is Locallized. |
Intramucosal carcinoma of the colon is assigned behavior code of /3. Intramucosal is not the same as in situ in terms of behavior. Behavior and staging are separate concepts, although there is some overlap. Use the instructions for coding behavior to code this field. Do not use stage to determine behavior in this case. For purposes of Summary Stage, intramucosal carcinoma is a localized lesion; however, for purposes of AJCC staging, assign Tis for the stage. |
2021 | |
|
20210036 | Update to current manual/Lymphovascular invasion: Are lymphvascular invasion and lymphvascular space invasion on a pathology report the same thing or do they describe different things? |
We confirmed with our expert pathologist consultant that lymphovascular invasion and lymphovascular space invasion are synonymous. |
2021 | |
|
20210012 | Solid Tumor Rules (2018, 2021/Multiple Primaries/)--Lung: How many primaries should be reported and what M rule applies when a diagnosis of presumed adenocarcinoma in situ (AIS) of the left lung follows a known diagnosis of progressive multifocal malignant adenocarcinoma in the right lung? See Discussion. |
Patient was initially diagnosed with a right lower lobe (RLL) lung adenocarcinoma in 2014 followed by subsequent right upper lobe (RUL) lung adenocarcinoma in 2016 (single primary). Both were treated with radiation and the nodules were seen as stable on surveillance. There was subsequent growth in the RUL nodule in 2019 and RLL nodule in 2020 as well as a new right middle lobe (RML) nodule in 2020. All left sided nodules were noted to be stable and/or ground glass opacities. There was no documented diagnosis of malignancy in the left lung until June 2020 when the physician noted that if there was a response in the left lung to systemic treatment, then this was probably multifocal AIS. However, only one tumor in the left lung responded to treatment. While it seems somewhat unlikely that only a single AIS in the contralateral lung should be metastasis from the right lung malignancy, it is difficult to apply the multiple tumors rules to this case. |
Abstract a single primary using 2018 Lung Solid Tumor Rule M9. The 2014 and 2016 R lung tumors were pathologically confirmed; it is not stated if they were resected. Follow up after XRT noted stable disease but no indication of NED. Subsequent right lung tumor is also the same primary. The issue is the assumed left lung adenocarcinoma in situ. It is not clear how long the left lung nodules were present, but they appeared to be stable as well and only diagnosed as a malignancy based on treatment response. At this time M9 applies and the left lung AIS is not a separate primary. We have discussed at length with lung pathology experts the issue of determining multiple primaries. Identifying and diagnosing lung tumors has become easier with new technology and the result is patients are being diagnosed with multiple lung tumors. Some lung experts feel we are under-reporting lung primaries but all noted the many issues with creating rules for consistency. |
2021 |
|
20210009 | Solid Tumor Rules (2018, 2021)/Histology--Melanoma: In what situation will Rule H4 be used to code the histology to regressing melanoma? See Discussion. |
Rule H4 states: Code 8723/3 (malignant melanoma, regressing) when the diagnosis is regressing melanoma. However, if the diagnosis was strictly regressing melanoma or malignant melanoma, regressing, the first rule that applies is Rule H1 because regressing melanoma is a single, specific histologic type and Rule H1 states: Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H4. Should the H Rules be reordered? Or should an example of when one would use Rule H4 be added to clarify when to use this rule? |
Coding regressing melanoma has been an issue as registrars may not realize it is a reportable histology. Hence, H4 was written to reinforce correct histology. A note will be added to H1 instructing registrars to continue thru rules when the diagnosis is regressing melanoma. |
2021 |