| Report | Question ID | Question | Discussion | Answer | Year |
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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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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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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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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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20180009 | Reportability--Head & Neck: Is dentinoameloblastoma reportable, and if so, what is the correct histology code? See Discussion. |
Mixed odontogenic tumor consistent with dentinoameloblastoma, 9.5 cm, See Note: Tumor involves maxillary bone including hard palate, alveolar ridges, nasal cavities and maxillary sinuses bilaterally and buccal soft tissue. Lymphovascular invasion not identified. Perineural invasion not identified. Margins: Tumor involves right posterior bone (alveolar) margin. All other margins negative. Note: This is a rare hybrid tumor showing features of ameloblastoma producing pre-dentin/osteodentin matrix. Submucosal tumor is seen in the nasal cavities and palate. A congo red stain shows that the acellular dentin-like matrix fluoresces similar to collagen after polarization. Immunohistochemistry shows that the tumor cells are diffusely and strongly positive for p63, focally positive for CK19, and negative for CK5/6, SOX10, S100 and calretinin. |
Dentinoameloblastoma is not reportable. It is a variant of ameloblastoma which produces dentin and/or osteoid. It is benign. It can extend locally in a rather aggressive fashion, but is not given a malignant designation unless it metastasizes. |
2018 |
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20091115 | MP/H Rules/Multiple primaries - - Melanoma: How many primaries are reported when a patient presents with a malignant melanoma (NOS) and a separate lentigo maligna, both on right chest? See Discussion. | MP/H rule M5 states that melanomas with ICD-O-3 histology codes that are different at the third number are multiple primaries. However, the 2007 MP/H fundamentals Webcast session on melanoma rules states that this is not two histologic types. Lentigo maligna is a growth pattern, not a histologic type. Will clarification be included in the next MP/H rules revision? |
For cases diagnosed 2007 or later, two primaries are to be reported for this case. Rule M5 applies because there is a difference in the histology codes at the third digit.
Clarifications regarding histologic types of melanoma will be added to the rules when they are revised. |
2009 |
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20091116 | MP/H Rules/Multiple primaries - - Colon: Is a colon tumor reported as "recurrent at the anastomotic junction" just over one year after the diagnosis of a T4 colon tumor to be counted as a new primary? See Discussion. | MP/H rules do not apply to metastasis. However, it has been our experience that pathologists and clinicians tend to use the terms metastatic and recurrence interchangeably. The term "recurrence" is not limited to a tumor recurrence in the same site as a previous malignancy. Sometimes it is obvious that the clinician is using the term recurrence to describe a metastatic lesion. When a "recurrence" is located in tissue that is very different from the original primary site, it is easy to recognize that the intended meaning of the term is metastasis.
Example: Patient with squamous cell carcinoma of the tongue with recurrence in the lung.
However, when the metastatic deposit occurs in similar tissue, it is more difficult to determine the number of primaries.
Example when the term "recurrence" is ambiguous: In April 2008 patient was diagnosed with adenocarcinoma of the ascending colon. At the time of hemicolectomy the tumor was noted to be plastered into the paraduodenal and peripancreatic area. Patient received one dose of adjuvant chemo and then discontinued treatment. In May 2009 the patient was found to have adenocarcinoma in the transverse colon. Per the pathology report the diagnosis for segmental resection at that time showed colonic adenocarcinoma. Tumor location: tumor appears recurrent at anastomotic junction. Abdominal wall mass showed metastatic adenocarcinoma.
One has to wonder if the pathologist found a metastatic nodule at the anastomotic site and called it "recurrent." It is unlikely that the pathologist will compare this specimen to the previous tumor, having already diagnosed it as "recurrent."
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For cases diagnosed 2007 or later, Rule M4 applies to the example of adenocarcinoma of ascending colon diagnosed in 2008 followed by adenocarcinoma of transverse colon diagnosed in 2009. When a colon resection has taken place, the original primary site is no longer present. A colon resection usually includes a portion of uninvolved colon on either side of the tumor. A tumor diagnosed at the anastomotic junction cannot be located in the same site as the previous tumor. Use of the term "recurrent" in this case is not synonymous with "metastatic." Apply the MP/H rules. | 2009 |
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20120088 | MP/H Rules/Multiple primaries--Head & Neck: How many primaries are accessioned and what rule applies if a patient has an extensive tumor in the left ethmoid sinus and a separate tumor in the right maxillary sinus? See Discussion. |
MRI and CT Neck Impression: Extensive tumor mass which likely originated within the left ethmoid sinus and extends intracranially via the cribriform plate into the anterior cranial fossa. There is involvement of the left orbit and extension into the superior aspect of the left maxillary sinuses as well as the nose. Second enhancing lesion within the right maxillary sinus measures almost 2 cm. The second mass within the floor of the right maxillary sinus, with similar imaging characteristics, is consistent with malignant involvement. The patient has an extensive ethmoid sinus tumor, biopsy showed squamous cell carcinoma. The ethmoid sinus is not a paired organ. The patient also has a small maxillary tumor with no histologic confirmation, Hem/Oncology chart notes state the right maxillary sinus mass is carcinoma. The maxillary sinus is a paired organ. Per the AJCC Manual (AJCC Manual for Staging, 7th edition, page 70), both the ethmoid and maxillary sinuses are further identified by their laterality (left and right). Why aren't the ethmoid sinuses a paired organ for the MP/H Rules? What MP rule applies to this case? |
For cases diagnosed 2007 or later, accession a single primary. The steps used to arrive at this decision are: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Head and Neck MP rules after determining the histology of each tumor - (8070/3 [squamous cell carcinoma] and 8010/3 [carcinoma, NOS]) because site specific rules have been developed for this primary. Start at the MULTIPLE TUMORS module, Rule M3. The rules are intended to be reviewed in consecutive order within a module. Abstract a single when one tumor is carcinoma, NOS [8010] and another tumor is a specific carcinoma, squamous cell carcinoma [8070] because the ethmoid sinus (site of origin) is not a paired site per the MP/H rules. We will review the list of paired organs for the next edition of the MP/H Rules. |
2012 |
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20130055 | Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a lymphoma with multifocal bone and epidural involvement but no lymph node involvement if the physician does not clearly state the primary site? See Discussion. | MRI Lumbar spine: Bony metastatic disease most evident at L5, L3 and T10. There is marrow tumor in the posterior elements of T12 and T10. The 14 mm epidural mass represents epidural tumor, likely metastatic, extending into the left intervertebral foramen at T12-L1.
PET scan: Hypermetabolic activity corresponding to epidural mass at the level of T12 and L1 concerning for malignancy. Other small areas of hypermetabolic activity in the left mandible and both femoral necks. There is no hypermetabolic activity corresponding to the areas of abnormal marrow edema in the vertebral bodies which enhanced on MRI scan in the lumbar and lower thoracic spine. No lymph nodes mentioned.
Biopsy epidural mass: Diffuse large B-cell lymphoma with a background of follicular lymphoma, consistent with a large cell transformation. Flow cytometry confirms a mixed large and small cell population of lymphoma (55% large cells).
T12/L1 Bone Biopsy: Bone and marrow with atypical paratrabecular lymphoid infiltrates, suspicious for involvement by follicular lymphoma. Negative for large cell lymphoma. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site of the diffuse large B-cell lymphoma [9680/3] to C809 [unknown primary site] per Rule PH27. The patient has involvement of multiple bones and an epidural mass with no evidence of nodal involvement. Code the primary site to unknown [C809] when multiple organs are involved without any lymph node involvement, even when there is no statement from the physician regarding primary site.
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. |
2013 |
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20150036 | Reportability/MP/H--Kidney: "Multilocular clear cell renal cell carcinoma." Would this be coded 8310? See discussion. |
Multilocular clear cell renal cell carcinoma is a specifc histologic type listed in the CAP cancer protocol for kidney, but not in the ICD-O-3 and it is not on the list of specific types of renal cell carcinomas in Table 1 of the kidney equivalent terms and definitions in the MP/H manual. There is a malignant multilocular cystic nephroma 8959 in Table 1, but I can't tell if this the same histology as what is stated in this path report. |
Apply Kidney rule H5 and code the clear cell (8310/3) which is the specific type of renal cell. Multilocular is a variant of clear cell which is a variant of renal cell carcinoma. As of yet, no new ICD-O morphology code as been proposed for this specific histology. It will be addressed in the revised rules. |
2015 |
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