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20110056 | Primary site--Heme & Lymphoid Neoplasms: What is the primary site for a post-transplant lymphoproliferative disorder (PTLD) diagnosed on a brain biopsy? See Discussion. | A patient was diagnosed in 6/2010 with PTLD by a brain biopsy. PTLD typically involves lymph nodes. Can the primary site for PTLD be coded to the brain? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule PH30, use the Heme DB to determine the primary site and histology when PH1-PH29 do not apply. Per the Abstractor Notes section in the Heme DB, PTLD commonly involves lymph nodes, GI tract, lungs, and liver. Although CNS involvement is rare, in solid organ recipients the CNS may be the only site of involvement or may be associated with multi-organ involvement. Code the primary site to C719 [brain, NOS] and the histology to 9971/3 [post-transplant lymphoproliferative disorder (PTLD)]
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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20110039 | Multiple primaries/Primary site--Heme & Lymphoid Neoplasms: What are the primary sites and how many primaries are abstracted, when 2004 diagnosis of grade 2 follicular lymphoma of the bilateral breasts is subsequently diagnosed with a 2010 diagnosis of diffuse large B-cell lymphoma (40%) and grade 3a follicular lymphoma (60%) of a left arm nodule? See Discussion. | Follicular lymphoma was diagnosed 7/2004, grade 2 per biopsy of the bilateral breasts. Bone marrow biopsy was positive for lymphoma involving 10% of bone marrow. Imaging showed extensive lymphadenopathy mainly in abdomen/pelvis with an 8 cm mass in the right pelvis. Smaller lymph nodes were noted in the left periaortic region and also some small precarinal lymph nodes. This was a stage IVA lymphoma. The patient had six cycles of CHOP/R with an excellent response. Per the clinician's notes on 12/2005, there was no evidence of recurrence or no sign of active disease. The plan was to follow up with the patient in 6 months.
08/22/06 imaging showed new disease in the bilateral chest wall. 8/2006 bilateral breast nodule biopsies, are positive for grade 1-2 follicular lymphoma. The patient was treated with Rituxan. Per clinician's 3/2007 note, no active disease is noted. Patient was regularly followed with no evidence of disease until 10/2010. At that time, the patient had a left arm nodule biopsy which was positive for diffuse large B cell lymphoma (40%) CD positive and grade 3a follicular lymphoma (60%). RICE was recommended due to "transformation" per oncologist. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M10, this case should be accessioned as two primaries when a neoplasm is originally diagnosed as a chronic neoplasm (is follicular lymphoma (FL), grade 2) AND there is a second diagnosis of an acute neoplasm (diffuse large B-cell lymphoma) more than 21 days after the chronic diagnosis.
Code the histology for the first primary to 9691/3 [follicular lymphoma (FL), grade 2] and the primary site to bilateral C509 [breast, NOS]. FL can start as an extranodal disease; breast is one of the sites in which it originates. It is unlikely that the lymphoma extended from the nodes to the breast, but highly likely that it extended from the breast to the nodes.
Per Rule M4, abstract the 2010 disease process as a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s). Per Rule PH11 the primary site is coded to C779 [lymph nodes, NOS] and the histology is coded to 9680/3 [diffuse large B-cell lymphoma (DLBCL)]. Rule PH11 states one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.
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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20110032 | Primary site--Heme & Lymphoid Neoplasms: What primary site is coded for Langerhans cell histiocytosis (LCH) [9751/3] when it is limited to the skin? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule PH30, use the Heme DB to determine the primary site and histology when PH1-29 do not apply, In this case, code the primary site to C449 [Skin]. According to the Abstractor Notes section in the Heme DB, the solitary form of Langerhans cell histiocytosis (LCH) [9751/3] occurs less commonly than the multisystem form of the disease; but can appear in nodes, skin and lung. This is a solitary form of LCH. Code the primary site to skin [C449].
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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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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20110038 | Reportability/Behavior: Is a "minimally invasive thymoma" a reportable malignancy if the pathology report does not specifically state it is malignant? See Discussion. |
For example, are Types A, B1, B2 and B3 reportable if the pathology report does not state the tumor is a "Malignant Thymoma"? |
For cases diagnosed prior to 2021 According to our expert pathologist consultant, code using the terms in the pathology report. Do not try to second guess the pathologist.
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2011 |
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20110129 | MP/H Rules/Histology--Lung: How many primaries are accessioned if a pathology report for a right upper lobectomy with a chest wall resection describes the disease as 1) two foci of poorly differentiated non-small cell carcinoma, 2) mixed adenocarcinoma and non-mucinous bronchioalveolar carcinoma, each present as a separate focus? See Discussion. |
This case was abstracted as two primaries, adenocarcinoma, acinar and papillary types [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3] per Rules M5 and M10. If this is reported as only two primaries, what is the stage for each tumor? The non-small cell tumors were the most invasive, but they were not a separate primary per Rule M10. Final pathology diagnosis for a RUL lobectomy and chest wall resection: Carcinoma of the lung with the following features: 1. Non-small cell carcinoma, poorly differentiated (see comment). Two foci in same lobe: 10 cm and 3 cm (largest dimensions of each tumor). Invades pleura (PL3), main bronchus and chest wall invasion present. 2. Adenocarcinoma and bronchioloalveolar carcinoma (see comment). Histologic subtype: Acinar and papillary (adenocarcinoma); non-mucinous (BAC). Two foci in same lobe: up to 1.0 cm. Pleural invasion absent, chest wall invasion absent. 3. Metastatic carcinoma in 5/7 peribronchial LN's. Two histologically distinct neoplasms identified in the lobectomy/chest wall resection specimen: Poorly differentiated non-small cell carcinoma, present as two foci; and adenocarcinoma and non-mucinous bronchioloalveolar carcinoma, each present as a separate focus. |
SEER will answer the question about the number of primaries to accession. Submit questions about stage to the CoC CAnswer Forum. For cases diagnosed 2007 or later: Accession two primaries: a mixed adenocarcinoma, acinar and papillary types [8255/3] and a bronchioloalveolar carcinoma, non-mucinous [8252/3]. The steps used to arrive at this decision are: Determine the histology code for each tumor prior to applying the Multiple Primary Rules to determine the number of primaries to accession. There are two non-small cell carcinomas, NOS; the histology code for these two tumors will be 8046/3. There is a single adenocarcinoma with acinar and papillary subtypes tumor, the histology for this tumor will be 8255. There is a single bronchioloalveolar carcinoma, non-mucinous subtype tumor; the histology for this tumor will be 8252/3. Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Lung Multiple Primary rules to determine the number of primaries. Start at the MULTIPLE TUMORS module, Rule M3, because this patient has multiple tumors. The rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3] are multiple primaries. Perform a second pass through the Multiple Primary rules to determine whether the two non-small cell carcinomas [8046/3] are multiple primaries or manifestations of the same primaries identified in Step 3. Start at Rule M3 again because this patient has multiple tumors. Again, these rules are intended to be reviewed in consecutive order within the module (from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient's non-small cell carcinomas, NOS [8046/3] are a single primary when compared to the adenocarcinoma with acinar and papillary subtypes [8255/3] and non-mucinous bronchioloalveolar carcinoma [8252/3]. Both of these histologies are more specific types of non-small cell carcinoma per the Lung Histology Groups and Specific Types Chart (Chart 1). You can also apply Rule M10 for both non-small cell carcinoma, NOS [8046/3] compared to adenocarcinoma with acinar and papillary subtypes [8255/3] and non-small cell carcinoma, NOS [8046/3] compared to non-mucinous bronchioloalveolar carcinoma [8252/3]. |
2011 |
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20110016 | Behavior--Brain and CNS: Can hemangioblastomas occurring in the CNS be coded as /3 (malignant) based on a radiologic or clinical diagnosis by the physician? See Discussion. | Hemangioblastomas are borderline (/1) according to ICD-O. The standard matrix rule in ICD-O directs registrars to change the behavior code to malignant when a malignant (/3) behavior is stated by a physician for a morphology code that appears in ICD-O with a non-malignant behavior code. The "malignant" hemangioblastomas we see are not pathologically confirmed; they are radiological or clinical diagnoses confirmed when renal cell carcinoma is a disease process listed in the malignant differential diagnoses. | The behavior code for hemangioblastoma can be coded to /3 when a pathologist indicates that the behavior is malignant. The behavior code should be based on a pathologist's opinion. It is usually not possible for a radiologist or patient care physician to make this determination clinically.
The histologic appearance of hemangioblastoma may resemble metastatic renal cell carcinoma; therefore, one will often see renal cell carcinoma listed as a possible diagnosis. This does not indicate that the hemangioblastoma is malignant. Do not code the behavior as /3 based on a differential diagnosis of renal cell carcinoma. |
2011 |
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20110110 | MP/H Rules/Multiple primaries--Head & Neck: If a 1991 neuroesthesioblastoma [9522/3] of the nasal cavity has subsequent recurrences of the same histology but later "recurs" in 2008 with "sarcoma, NOS, high grade" on a biopsy and a "high grade fibrosarcomatous transformation of esthesioneuroblastoma" [8810/3] on resection, should the subsequent tumor be reported as a new primary if the clinician continues to refer to the tumor as a "recurrence"? See Discussion. |
Are histologic transformations always recurrences of the original tumor? |
Assuming the same primary site for the 2008 lesion, according to the current MP/H rules the high grade fibrosarcoma [8810/3] is a new primary per Head & Neck MPH rule 11 because it is a different histology. The revised MP/H rules will include tables to define tumors that de-differentiate (transform) and recur with what is seemingly a different histology. Although the rules will be changed in the future, we must use the rules in place at this time for this case. |
2011 |
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20110060 | Reportability--Heme & Lymphoid Neoplasms: In the absence of any additional information regarding the disease process, is a diagnosis of "polycythemia" reportable if a patient is treated with phlebotomy? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
No. Polycythemia, NOS is not reportable.
Polycythemia (also known as polycythaemia or erythrocytosis) is a disease state in which the proportion of blood volume that is occupied by red blood cells increases. Blood volume proportions can be measured as hematocrit level. It can be due to an increase in the mass of red blood cells, "absolute polycythemia"; or to a decrease in the volume of plasma, "relative polycythemia".
The phlebotomy is a treatment for the excessive blood volume; therefore, a diagnosis of "polycythemia" without one of the modifying terms listed in the Heme DB under Alternative Names is not reportable.
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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20110068 | MP/H Rules/Multiple primaries--Bladder: Which multiple primary rule is used to determine the number of primaries to accession when a patient has a papillary transitional cell carcinoma of the bladder diagnosed in 2009 followed by a high grade invasive urothelial carcinoma with neuroendocrine features per immunohistochemistry diagnosed in 2010? See Discussion. | A patient has papillary transitional cell carcinoma of the bladder in March of 2009. In June of 2010 the patient has another TURBT that demonstrates a flat in situ and invasive high grade urothelial carcinoma. The path addendum indicates, "Genzyme IHC show results consistent with high grade invasive urothelial carcinoma with neuroendocrine features." Two months later a liver biopsy shows poorly differentiated malignant tumor. The path addendum indicates, "Genzyme IHC results show metastatic poorly differentiated carcinoma with neuroendocrine features, favor bladder primary."
Is the latter a second bladder primary with histology code 8246/3 [neuroendocrine carcinoma]?
NOTE: Neuroendocrine is not listed as an urothelial tumor in Table 1 of MP/H Rules. |
Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the number of primaries. This is a single primary. The 2010 diagnosis is urothelial carcinoma. The presence of "neuroendocrine features" does not change the histologic category.
The steps used to arrive at this decision are:
Open the Multiple Primary and Histology Coding Rules manual. Once in the manual, locate the Urinary MP rules under one of the three formats (i.e., flowchart, matrix or text). The rules are intended to be reviewed in consecutive order within the module. You stop at the first rule that applies to the case you are processing.
Start at the MULTIPLE TUMORS module start at rule M3.
. Bladder tumors with any combination of transitional cell carcinoma and papillary transitional carcinoma are a single primary. |
2011 |