| Report | Question ID | Question | Discussion | Answer | Year |
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20130013 | Reportability--Heme & Lymphoid Neoplasms: Is Mast Cell Activation Syndrome (MCAS) reportable? |
MCAS has been given an ICD-9 code of 202.60 by our medical record coders. In the Progress Notes, the physicians state this is not the same as systemic mastocytosis. There is no listing for MCAS in the Hematopoietic and Lymphoid Database. |
Mast Cell Activation Syndrome (MCAS) is not a reportable neoplasm unless it is specifically stated to be a result of a mast cell proliferative disorder that is reportable. Per our expert pathologist, MCAS is a relatively new term used for conditions in which patients experience the symptoms of mast cell mediators in the absence of an increase/proliferation of mast cells. The diagnosis of this group of disorders is based in part on a complex of symptoms and on the demonstration of no increase in mast cells. Some of these diseases are difficult to separate from mastocytosis (which is reportable). Currently, this group of disorders is not part of the systemic mastocytosis/mast cell leukemia/mast cell sarcoma spectrum. |
2013 |
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20140064 | Reportability--Testis: Is a mature teratoma of the testis reportable? See discussion. |
Mature teratoma is listed as a benign neoplasm (9080/0) in the ICD-O-3. SINQ 20120085 references a NAACCR Webinar that indicated pure mature teratomas of the testis in adults are reportable. We are not aware of any further documentation of this change in reportability. When did mature teratomas of the testis for adults become reportable? What is the defined age range for "adult"? The original SINQ question above lists the 2012 SEER Manual as a Reference, however, no clarification or mention of this change in reportability was found in that manual. |
For testis, mature teratoma in an adult (post-puberty) is reportable because it is malignant (9080/3); however, mature teratoma in a child is benign (9080/0). The 2011 NAACCR webinar introduced this concept and it was documented in the 2012 SINQ question. You may use 2011 or 2012 as the date of this change. The next edition of the SEER manual will include reportability examples. |
2014 |
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20230021 | Histology--Soft Tissue: How is histology coded for malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement diagnosed on left shoulder excision? See Discussion. |
March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation. Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement? |
NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3). WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics. |
2023 |
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20180004 | Reportability/MP/H Rules/Multiple primaries: Is a ganglioneuroblastoma (9490/3) following a melanoma (8720/3) a new primary if the diagnosing pathologist states: "Given the clinical context and patient age, then I believe that this may represent transdifferentiation of metastatic melanoma'? If this is a new primary, what MP/H rule would apply? See Discussion. |
March 2017 lung biopsy showing metastatic melanoma. Subsequent workup shows imaging with additional metastatic involvement of multiple bone sites but no primary tumor is identified. Chemotherapy is started in May 2017. July 2017 biopsy of right lower quadrant mass has a final diagnosis of ganglioneuroblastoma and pathologist's comment states I believe that this may represent transdifferentiation of metastatic melanoma. Later, partial colectomy of transverse colon Gross Description indicates this was centered in the mesentery. |
Abstract two primaries: 1. unknown primary site and 2. peripheral nerves and autonomic nervous system of abdomen, based on Multiple Primaries/Histology for Other Sites Rule M11 (topography codes that differ at the second or third character). While it is possible in rare cases that one tumor transforms into the other, transformations do not factor into the current MP/H rules. |
2018 |
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20071106 | MP/H Rules--Bladder: Does rule M6 mean that any combination of tumors with the histologies 8050, 8120-8124, or 8130-8131 are the same primary regardless of the amount of time between tumor occurrences? See Discussion. |
Many interpret Rule M7 to mean when separate occurrences of TCC of the bladder are diagnosed more than 3 years apart, it is reportable as a second primary. However, doesn't Rule M6 mean that if the histology is any combination of 8050, 8120-8124 or 8130-8131 for tumors diagnosed more than 3 years apart, they are reported as a single primary? |
For cases diagnosed 2007 or later: Papillary, transitional cell and/or papillary transitional cell carcinomas of the bladder are a single primary using Rule M6. Rule M6 includes diagnoses within 3 years of each other AND diagnoses more than three years apart for the histologies listed. If rule M6 applies to your case, stop. Do not continue on to Rule M7. |
2007 |
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20140089 | Multiple primaries--Heme & Lymphoid Neoplasms: Should the 2014 diagnosis be abstracted as a new primary since it is not mantle cell lymphoma and all of the types listed in the differential diagnosis would be a new primary? See discussion. |
Mantle cell lymphoma diagnosed in 1997 which was treated with chemotherapy. Now in 2014 a 'relapse' of non-Hodgkin lymphoma. They do a biopsy of the pericardium, which is called low grade B cell non Hodgkin lymphoma. See comment. The comment says histochemical stains are reviewed and findings are consistent with involvement by a CD5 positive low grade B cell lymphoma. Lack of cyclin D1 and SOX-11 positivity as well as negative IGH-CCND1 FISH analysis essentially rule out mantle cell lymphoma. The morphologic and immunophenotypic features of this disorder are not specific for any lymphoma subtype. The differential includes CLL, marginal zone lymphoma, and lymphoplasmacytic lymphoma. If this is coded NHL, NOS (9591) it is the same primary as seq. 1 and would not be abstracted. |
This is the same primary, the mantle cell lymphoma.
Differential diagnoses cannot be used to assign histology. For the 2014 diagnosis, the only histology that can be assigned is 9591/3 for non-Hodgkin lymphoma, NOS. (CLL, mantle cell lymphoma and lymphoplasmacytic lymphoma are all NHL's.)
Compare the 1997 diganosis of mantle cell lymphoma with the 2014 diagnosis of non-Hodgkin lymphoma. Start with Rule M1. The first rule that applies is Rule M15, which instructs you to use the multiple primaries calculator. Enter 9673/3 and then 9591/3 and then calculate. The result is same primary.
If at a later time one of the differential diagnoses is confirmed, apply the rules again.
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2014 |
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20240038 | Solid Tumor Rules/Multiple Primaries--Brain and CNS: How many primaries are accessioned, and what M Rule applies to a 2023 diagnosis of pituitary macroadenoma followed by a 2024 diagnosis of pituitary neuroendocrine tumor (PitNET) when the patient did not undergo surgery, but did undergo hormone therapy with Cabergoline? See Discussion. |
Malignant Central Nervous System (CNS) Rule M5 instructs us to abstract a single primary (as malignant) when a single tumor is originally diagnosed as non-malignant, the “First course treatment was active surveillance (no tumor resection),” and the subsequent resection pathology is malignant. This patient’s first course of treatment was not active surveillance. While the patient did not have first course tumor resection, the tumor was treated with Cabergoline. Should Rule M5 apply because there was no tumor resection? If so, should Rule M5 clearly state no tumor resection is the criteria (not active surveillance)? SINQ 20230023 does indicate a PitNET diagnosis following a diagnosis of pituitary adenoma does not fall into standard rules, but in the previous SINQ the first course treatment was a partial resection. It is unclear whether other types of treatment could result in a new malignant PitNET, following a previously treated non-malignant pituitary tumor. |
Abstract a single primary as 8272/3 (pituitary adenoma/PitNET) using the Malignant CNS and Peripheral Nerves Solid Tumor Rules, Rule M2, a single tumor is always a single tumor. Change the histology of the 2023 diagnosis to 8272/3. This scenario does not meet the criteria in the current rules for M5 in that it requires a resection as part of the criteria. Since the patient did not undergo resection for either diagnosis, the 2024 diagnosis may indicate recurrence or progression. A diagnosis of pituitary adenoma only is still coded 8272/0 (this code is still valid). A diagnosis of pituitary adenoma/PitNET, PitNET, or pituitary neuroendorine tumor is coded 8272/3. Cabergoline is used to treat prolactinoma or high levels of prolactin but does not impact the PitNET. |
2024 |
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20220034 | First Course Treatment--Lymphoma: Is the first round of systemic therapy coded as first course of therapy or is it all the therapy given to achieve remission for a lymphoma case with multiple treatments? See Discussion. |
Lymphoma case diagnosed in 2021: The patient had first round of systemic therapy as documented in the treatment plan and a post-chemotherapy PET scan that showed residual disease. The patient then had a different combination of systemic therapy and still had some residual disease. The patient was given a third round of different combination of systemic therapy in preparation for stem cell transplant. According to the physician post-stem cell transplant note, the patient achieved complete remission. Is the first course of therapy the first round of systemic therapy only or is it all the therapy given to achieve remission? It seems like only the first round of systemic therapy is first course of therapy for both leukemia and lymphoma in the hematopoietic manual. I thought all treatment for all hematopoietic cases was first course until remission achieved or progression was evident. |
Code all treatments the patient received as first course of treatment. For lymphoma and leukemia, first course of treatment may include first-line, second-line, consolidation, maintenance, salvage, etc., any treatment to achieve remission. We have added this to the agenda for the 2024 updates to the Hematopoietic Manual and Database. |
2022 |
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20220042 | First Course Treatment/Radiation Therapy: How should Lutathera be coded? CoC states XRT- Radioisotopes and SEER states Other Treatment. |
Lutathera is a radioconjugate consisting of the tyrosine-containing somatostatin analog Tyr3-octreotate (TATE) conjugated with the bifunctional, macrocyclic chelating agent tetra-azacyclododecanetetra-acetic acid (DOTA) and radiolabeled with the beta-emitting radioisotope lutetium Lu 177 with potential antineoplastic activities. |
Update to the current manual: Code Lutathera as radiation (isotopes NOS code 13). We will make this change in the next version of the SEER manual. |
2022 |
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20200048 | Solid Tumor Rules/Multiple Primaries--Lung: How many primaries are accessioned when a patient is diagnosed with right lower lobe invasive acinar adenocarcinoma (8551/3) in 2018 and treated with lobectomy, followed by a 2019 right middle lobe cancer (NOS, 8000/3) diagnosed as new stage 1 primary by cancer conference? See Discussion. |
Lung Rule M14 appears to be the first rule that applies to this case and instructs the user to abstract a single primary. However, we were hoping for confirmation that a cancer (NOS) or malignancy (NOS) would not be a distinctly different histology that may qualify for Lung Rule M8. Currently, these histologic terms are not included in the Table 3 options or mentioned in the preceding notes. |
Use M14 and code a single primary. Per our SME, carcinoma or cancer, NOS is not an acceptable diagnosis which is why 8000 and 8010 were not included in the tables or rules. We assume there was no tissue diagnosis for the 2019 diagnosis. We recommend searching for more information or better documentation on this case. |
2020 |
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