Report | Question ID | Question | Discussion | Answer | Year |
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20210046 | Reportability--Skin: Is dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation synonymous with dermatofibrosarcoma protuberans, fibrosarcomatous, and therefore reportable for diagnosis year 2021 and forward? See Discussion. |
Patient has a 2021 skin excision showing an atypical spindle cell neoplasm, most consistent with dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation. Per the ICD-O-3.2 Coding Table, DFSP, NOS has a behavior code of /1, and DFSP, fibrosarcomatous has a behavior code of /3. There is no code listed for DFSP with fibrosarcomatous transformation. Transformation is not included as a term that can/cannot be used for the Other Sites Schema, but this type of DFSP is often described as DFSP with fibrosarcomatous transformation. How do we code DFSP when transformation is used to describe fibrosarcomatous? |
Report DFSP with fibrosarcomatous transformation as it is synonymous with fibrosarcomatous DFSP (8832/3). According to the WHO Classification of Skin Tumors, 4th edition, fibrosarcomatous DFSP is a variant of DFSP and that fibrosarcomatous transformation is seen in approximately 10% of DFSP cases. It is characterized by an often abrupt transition of DFSP. |
2021 |
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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 |
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20210040 | Solid Tumor Rules (2018, 2021)/Histology--Breast: How is histology coded for a diagnosis ofmixed mucinous carcinoma? See Discussion. |
Patient was diagnosed with invasive ductal carcinoma with mucinous features in February 2021, left breast biopsies at 2:00 (3 cm from nipple) and 3:00 (8 cm from nipple) positions. Intraductal component was absent in those specimens. Subsequent left breast total mastectomy in March 2021, provided a final diagnosis of multifocal mixed mucinous carcinoma, grade 1, 27 and 8 mm and ductal carcinoma in situ (DCIS), low to intermediate grade, with focal mucinous features. The Staging Summary lists Histologic Type as mixed mucinous carcinomafor both foci of invasive carcinoma. DCIS is also noted as present negative for extensive intraductal component. There does not appear to be a clear instruction or code for this histology. As a central registry, we are unable to follow-up with the pathologist regarding this diagnosis. Just to be clear, there are 2 foci of invasive mixed mucinous carcinoma in this case measuring 27 mm and 8 mm. The DCIS component measured 56 mm. |
If the DCIS is separate from the mucinous, apply the breast M rules and abstract TWO primaries per M14. Since all we know of the “mixed mucinous” is there is mucinous present, code 8480/3. |
2021 |
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20210057 | Reportability/Histology--Kidney: Is an oncocytic renal neoplasm of low malignant potential (ORNLMP) reportable? See Discussion. |
Kidney, right interpolar neoplasm, partial nephrectomy: Oncocytic renal neoplasm of low malignant potential (ORNLMP). Within part B, right interpolar kidney neoplasm, the neoplasm shows oncocytic features, with abundant granular eosinophilic cytoplasm and enlarged vesicular nuclei with prominent central nucleoli. The cells are arranged in small nests and tubules with hypocellular fibrous stroma identified within the background. Scattered binucleated cells are present, and rare cells with irregular nuclear membranes are present. No perinuclear halos or prominent cell membranes are present. Given the histologic features, the neoplasm is best classified as an oncocytic renal neoplasm of low malignant potential (ORNLMP). |
Oncocytic renal neoplasm of low malignant potential is not reportable. |
2021 |
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20210041 | Reportability/Behavior--Paraganglia: Is a 2021+ diagnosis of paraganglioma reportable if the grading of adrenal pheochromocytoma and paraganglioma (GAPP) score falls outside the stated requirements for malignancy? See Discussion. |
Patient was diagnosed with a retroperitoneal paraganglioma on April 2021 mass resection. Final diagnosis included the comment: Based on the modified grading of adrenal pheochromocytoma and paraganglioma (GAPP), the GAPP score is 1. Scores greater than or equal to 3 are malignant. We are aware that paraganglioma is classified as malignant for cases diagnosed in 2021+, however it is unclear how the pathologist's interpretation of the GAPP score may affect the behavior of this case. |
Report retroperitoneal paraganglioma based on ICD-O-3.2 histology/behavior that lists paraganglioma, NOS as 8680/3 for cases diagnosed 2021 and forward. While GAPP is a predictor of metastatic potential, it does not factor into behavior, thus reportability. |
2021 |
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20210073 | Solid Tumor Rules (2018/2021)/Multiple Primaries--Corpus Uteri: How many primaries should be reported when a hysterectomy identifies primary endometrial carcinosarcoma (8980/3) and the endometrium has a background of endometrioid intraepithelial neoplasia (EIN) (8380/2)? A tumor size is provided for the carcinosarcoma, but not the background EIN. |
Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN). |
Abstract this case as a single primary and code histology as carcinosarcoma (8980/3). The carcinosarcoma is intermixed with the EIN making this a single primary coded to the invasive histology. EIN is a precursor of endometrial carcinoma in the WHO Classification of Female Genital Tumors, 5th edition. Carcinosarcoma of the uterus is described in the literature as an aggressive variant of endometrial carcinoma characterized by unusual histologic features including discrete malignant epithelial and mesenchymal components (carcinoma and sarcoma). |
2021 |
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20210008 | Solid Tumor Rules (2018, 2021)/Histology--Melanoma: In what situation will Rule H6 be used to code the histology to lentigo maligna melanoma? See Discussion. |
Rule H6 states: Code 8742/3 (Lentigo maligna melanoma) when the diagnosis is lentigo maligna melanoma with no other histologic types. However, if the diagnosis was strictly lentigo maligna or lentigo maligna melanoma, the first rule that applies is Rule H1 because lentigo maligna 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 H6. Should the H Rules be reordered? Or should an example of when one would use Rule H6 be added to clarify when to use this rule? |
Solid Tumor rule H6 is the same as MP/H rule H8. We found registrars have problems understanding reportable terminology and the corresponding ICD-O-3 histology code for lentigo maligna melanoma. It is included in H6 to capture cases where the registrar may not stop at H1. We will add another note to H1 instructing users to continue through the rules if the diagnosis is lentigo maligna melanoma. |
2021 |
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20210028 | Histology/Biliary tract--Ampulla of Vater: What is the histology code for Intra ampullary papillary-tubular neoplasm in association with microinvasion? See discussion. |
Patient was diagnosed on 01/2020, and primary site on the pathology report is Ampulla of Vater (C241). Synoptic Report states histology as: Intra ampullary papillary-tubular neoplasm in association with microinvasion. I have reviewed the ICD-O-3 coding table and found histology Intraductal tubulopapillary neoplasm (C25_) code 8503/2. Based on the Matrix principle (Rule F on the ICD-O-3), I will change the behavior to 3 and code as 8503/3. If I look in ICDO-3, Tubulopapillary adenocarcinoma is coded 8263/3. |
Assign code 8163/3. Based on the microinvasion, the correct term for this neoplasm is pancreatobiliary-type carcinoma. Unfortunately, WHO did not provide all synonyms or related terms for some of the new ICD-O codes. Pathologists may continue using non-preferred terminology as well. |
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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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 |