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20170007 | MP/H Rules/Histology--Urinary System: How should histology be coded when there are multiple bladder, ureter and renal pelvis urothelial tumors including non-invasive papillary urothelial carcinoma in the left ureter, invasive papillary urothelial carcinoma invading the lamina propria in the bladder, and an invasive sarcomatoid urothelial carcinoma of the renal pelvis that invades the muscularis? See Discussion. |
Per Rule M8, this is a single primary as there are multiple urothelial tumors as outlined in Table 1 (papillary urothelial carcinoma [8130] and sarcomatoid urothelial carcinoma [8122]) simultaneously present in multiple urinary organs (bladder, ureter and renal pelvis). As Rule M8 indicates these are a single primary, despite the histologies differing at the third digit (8130 vs 8122), then Rule H14 (Code the histology of the most invasive tumor) seems to be the most applicable histology rule. Following Rule H14 (in the Text version of the MP/H Rules), the histology would be coded as 8122 (sarcomatoid urothelial carcinoma) since the renal pelvis tumor was the most invasive tumor present. However, in both the Matrix and Flowchart versions of the MP/H Rules, Rule H14 contains a note (missing from the Text version) that states that this rule should only be used when the first three numbers of the histology codes are identical (This is a single primary). Rule M8 clearly tells us these are a single primary, despite the differences at the third digit of the histology. Further defaulting to Rule H15 (Code the numerically higher histology code) in this case would ignore the histology of the tumor with the worse prognosis (the most invasive tumor). Was this note included in the Matrix and Flowchart versions in error? |
Code the histology as 8122 according to the MP/H rules for Renal Pelvis, Ureter, Bladder, and Other Urinary, M8 and H14. Rule M8 states urothelial tumors in two or more of urinary sites including bladder and renal pelvis are a single primary. Rule H14 states code the histology of the most invasive tumors for multiple tumors abstracted as a single primary. |
2017 |
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20110017 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a patient originally diagnosed with CLL is subsequently diagnosed several months later on a bone marrow biopsy with Richter's syndrome that transformed into a large cell lymphoma? See Discussion. |
Per reviewed resources, the described condition is rare. Should the histology remain CLL or be changed to large cell lymphoma? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm. Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3]. SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx. |
2011 |
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20130072 | MP/H Rules/Multiple primaries--Lung: How many primaries are accessioned when the right lower lobe lung has two adenocarcinomas, both with lepidic pattern, if the tumor board staged these tumors as separate primaries? See Discussion. |
Per pathology report
The tumor board has staged this as two separate primaries and is treating it as such. They are not considering the second focus metastatic even though it is the same histology. Lepidic is not in the ICD-O-3. Is lepidic a new term for histology? |
For cases diagnosed 2007 and later, accession a single primary, adenocarcinoma [8140/3] of the right lower lobe lung. The steps used to arrive at this decision are: Step 1: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Lung MP rules because site specific rules have been developed for this primary. Step 2: Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. Stop at rule M12. Accession a single primary when the patient has two tumors in the same lung with the same histology. Keep in mind that physicians follow different "rules" to determine the number of primaries. Even though the physicians consider this case to represent two primaries, the MP/H rules instruct you to accession one primary. We have received quite a few questions about the term lepidic. Below is the general definition of lepidic that will be added to the next MP/H revision. "Lepidic" is a growth pattern meaning that tumor cells are growing along the alveolar septa. It is characteristic of bronchioloalveolar carcinoma (BAC), but not diagnostic of it. The diagnosis of BAC also requires no stromal, vascular, or pleural invasion. Lepidic growth may be seen in other adenocarcinomas, including metastases to lung from other sites. It is not a type/subtype of adenocarcinoma. For lepidic lung neoplasms, code the histology indicated, for example BAC. |
2013 |
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20110004 | MP/H Rules/Histology--Breast: Which MP/H rule applies when coding the histology field for a tumor described as a "metaplastic carcinoma, adenosquamous and spindle cell type"? See Discussion. | Per path comment: "The neoplasm is composed of adenosquamous carcinoma which merges with spindle cell carcinoma. The cystic component shows a mixed squamous and ductal epithelial lining which shows cytologic atypia and mitotic activity and can be seen to merge with invasive carcinoma. The features suggest the possibility that the tumor may have arisen from a sclerosing and cystic papilloma with squamous metaplasia, although a clearly benign component is not evident."
Would MP/H rule H19 apply based on the pathology report comment resulting in histology for the case being coded to 8255 [adenocarcinoma with mixed subtypes]? Or, would MP/H rule H14 apply based on the final diagnosis resulting in histology for the case being coded to 8575 [metaplastic carcinoma] because adenosquamous and spindle cell are not specific types of metaplastic carcinoma? |
This is a metaplastic carcinoma as stated in the path diagnosis. Rule H14 applies. Assign code 8575/3. According to the WHO Classification, metaplastic carcinoma is a general term for a group of neoplasms characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous, and/or mesenchymal differentiation.
Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the histology for this case. Code histology to 8575/3 [metaplastic carcinoma] as stated in the pathology diagnosis.
Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Breast Histo rules determine histology for the case.
Go to the SINGLE TUMOR: INVASIVE CARCINOMA ONLY module. The rules are intended to be reviewed in consecutive order within the module from Rule H10 to Rule H19. You stop at the first rule that applies to the case you are processing.
Code the histology when only one histologic type is identified. According to the WHO Classification, metaplastic carcinoma is a general term for a group of neoplasms characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous, and/or mesenchymal differentiation. |
2011 |
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20021162 | Chemotherapy: Should radiosensitizing chemotherapy agents (i.e., drugs typically coded as treatment for cancer) be coded as treatment when they are given in combination with radiation therapy with the intention of enhancing that treatment? See discussion. |
Per our consultant, these drugs are given at a lower dose than that typically given to treat cancer patients. |
Do not code radiosensitizers and radioprotectants as cancer-directed therapy. Drugs typically classified as chemotherapy agents would be "ancillary drugs" for the purpose of coding cancer-directed therapy because the drugs are given at a much lower dosage than that typically given to treat cancer patients. Per Book 8, ancillary drugs are not to be coded as cancer-directed therapy. Radiosensitizers and radioprotectants do not work directly on the cancer and are not coded under any of the systemic therapy fields. |
2002 |
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20100034 | MP/H Rules/Multiple primaries--Esophagus: Should two separate nodules of adenocarcinoma with one at the GE junction [C160] and one arising in Barretts esophagus of the distal esophagus [C155] be accessioned as a single primary because these sites are now grouped together in the same stage grouping per the AJCC 7th Edition? See Discussion. | Per notes included in CSv2, the cardia/EGJ, and the proximal 5cm of the fundus and body of the stomach [C16.0-C16.2] have been moved from the Stomach chapter and added to the Esophagus chapter effective with AJCC TNM 7th Edition. A new schema, EG Junction, was created in CSv2 to accommodate this change. Tumors arising at the EGJ, or arising in the stomach within 5 cm of the EGJ and crossing the EGJ are staged using the schema for EG Junction. MP/H Rule M11 states that tumors with ICD-O-3 topography codes that are different at the second (Cxxx) and/or third characters (Cxxx) are multiple primaries.
In light of the fact that tumors of the GE junction are now included with tumors of the esophagus in AJCC 7th Edition, will the MP/H rules also be adjusted to reflect that change? |
For cases diagnosed 2007 or later, use the multiple primary rules to determine the number of primaries. Use staging resources for staging. Abstract two primaries for the case example using Rule M11. | 2010 |
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20051086 | CS Site Specific Factor 4--Prostate: For apex involvement at prostatectomy, is only apical involvement found at prostatectomy included or is all histologically proven apical involvement documented in the second digit of Site Specific Factor 4? See Discussion. | Per note 1 for Site Specific Factor 3 - Pathologic Extension all histologic information is used. Biopsy information would be included when coding path extension. Would all histologic information be used for coding prostatectomy apex involvement in Site Specific Factor 4? Example 1: Prostate biopsies of the right and left apex and right and left mid gland show adenocarcinoma. Prostatectomy shows bilateral adenocarcinoma. Apex negative for tumor. Example 2: Prostate biopsies of right apex and mid gland show adenocarcinoma. There is no mention of apex on prostatectomy path. How is CS Site Specific Factor 4 Prostate Apex Involvement coded? |
This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2. Assign the second digit of CS SSF 4 based on prostatectomy only, do not include biopsy or other histologic information in the second digit. According to the CS Steering Committee, the clinical or biopsy of the prostate is included in the first number of the code and should not be combined with the prostatectomy code which is the second number. These were separated purposely. Example 1: Code the second digit of SSF 4 based on the prostatectomy, 1 [no involvement of prostatic apex]. Example 2: Code the second digit of SSF 4 based on the prostatectomy, 5 [apex extension unknown]. |
2005 |
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20110013 | MP/H Rules/Histology--Testis: Which MP/H rule applies in coding the histology described as a "malignant mixed germ cell tumor with the following features: Histologic type: embryonal carcinoma (97%) and yolk sac tumor (3%)"? See Discussion. |
Per MP/H rule H16, code the appropriate combination/mixed code (Table 2) when there are multiple specific histologies or when there is a non-specific histology with multiple specific histologies. The combination embryonal carcinoma and yolk sac tumor is not listed in Table 2, even though the pathology report indicates this is a mixed germ cell tumor.
Should rule H17 be applied and the numerically higher histology code be used? |
As of 2016: Code histology to 9085/3 [mixed germ cell tumor]. Combine 9065 and 9085 for analysis purposes. |
2011 |
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20200057 | Histology--Lung: Is there a better code for SMARCA4-deficient malignant neoplasms than 8000/3 that could be used especially given its aggressive nature? This term is not included in the Lung Solid Tumor Rules or ICD-O-3.1 and 3.2. See Discussion. |
Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32. |
Answer updated August 2025 Assign code 8044/3. WHO Classification of Thoracic Tumors, 5th edition, classifies SMARCA4-deficient malignant neoplasm as Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT). |
2020 |
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20110155 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient shows evidence of "MDS as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera" 18 years after a diagnosis of "thrombocytosis and probable polycythemia that progressed to probable myelofibrosis"? See Discussion | Per consultation: an 83 year old patient started on hydroxurea 18 years ago following a diagnosis of thrombocytosis and probable polycythemia. It appears the polycythemia progressed to probable myelofibrosis. The possibility of an MDS needs to be considered.
Problem list: Polycythemia with probable progression to myelofibrosis or MDS.
Bone marrow biopsy two weeks later shows some progression of dysmegakaryocytopoiesis. Patient has evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera.
On follow-up visit six weeks later: Continue to manage patient with hydroxyurea.
An additional six months later: Diagnosis is polycythemia with thrombocytosis. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as a single primary. Code the histology to 9920/3 [therapy-related myelodysplastic syndrome].
The reportable diagnoses must first be separated from the non-reportable diagnoses mentioned in the consult. Thrombocytosis (NOS), polycythemia (NOS), and myelofibrosis (NOS) are not reportable terms. To verify this, look up each term in the Heme DB. No database matches list the preferred name or the alternative names as any of these NOS terms.
The reportable diagnoses are all from the post-bone marrow biopsy consult, "evidence of MDS, as well as essential thrombocytosis and JAK2 mutation positive polycythemia vera." The subsequent notes in the consult again only refer to this as non-reportable polycythemia (NOS) or thrombocytosis (NOS). Keep in mind that this patient has been undergoing treatment with chemotherapy (hydroxyurea) for many years for polycythemia (NOS); the patient was diagnosed with polycythemia, "about 18 years ago."
According to the Subject Matter Experts, as MDS progresses, it may manifest as several different subtypes, this is a part of the disease process and abstracting each subtype would result in over-reporting this disease. This patient has a complicated history. The consult information does not adequately document whether this patient's initial diagnosis of "polycythemia" was primary polycythemia (reportable) or a secondary polycythemia (not reportable). If the patient was initially diagnosed with a primary polycythemia 18 years ago the current diagnosis of "JAK2 mutation positive polycythemia vera" would not be a new primary. The manifestation of ET may be due to the progression of MDS. In either case, this patient does have a therapy-related myelodysplastic syndrome which is the same primary as both PV and ET.
SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx. |
2011 |
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