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20120058 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned when the patient is diagnosed with an acute neoplasm (diffuse large B-cell lymphoma) per a pathology report and is subsequently diagnosed clinically with a chronic neoplasm (chronic lymphocytic leukemia/small lymphocytic lymphoma) less than 21 days later? See Discussion. | The patient was diagnosed with an extranodal DLBCL on a biopsy of the stomach. A bone marrow biopsy performed 16 days later showed no DLBCL, but demonstrated an abnormal CD5-positive B-cell population that was subsequently referred to as CLL/SLL by the physician. The peripheral blood was negative and showed only moderate thrombocytopenia.
Does rule M10 apply in this case? Abstract the acute neoplasm as a single primary (DLBCL) as there was only one pathology specimen (stomach biopsy) proving DLBCL and the bone marrow did not definitively identify CLL/SLL. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case should be accessioned as two primaries per Rule M11. Code the histology of one primary to 9680/3 [diffuse large B-cell lymphoma], the acute neoplasm. Code the histology for the second primary to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma], the chronic neoplasm.
Per Rule M11, abstract as multiple primaries when both a chronic and acute neoplasm are diagnosed simultaneously or less than or equal to 21 days apart AND there is documentation of two pathology specimens, one confirming the chronic neoplasm (bone marrow biopsy) and one confirming the acute neoplasm (stomach biopsy).
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. |
2012 |
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20120011 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Is there a timing rule used to recode histology should a more specific diagnosis of refractory anemia with excess blasts (RAEB) be confirmed after an initial diagnosis of myelodysplastic syndrome (MDS)? How many primaries are abstracted if RAEB subsequently evolves toward an acute myeloid leukemia? See Discussion. |
Facility A: 4/8/2010 Bone Marrow biopsy: Features most compatible with MDS. (No treatment administered.) 7/2/2010 Peripherial Blood: Transforming Myelodysplastic Syndrome (MDS). COMMENT: Clonal abnormality compatible with MDS/acute myeloid leukemia (AML) in all metaphases examined. (Still no treatment administered.) Facility B: 10/6/2010 Patient now presents for evaluation and treatment. Patient started on Vidaza. 10/07/10 Bone Marrow biopsy: Refractory anemia with excess blasts (RAEB-2) COMMENT: Evolution towards AML with myelodysplasia related changes considered; cytogenetic analysis reveals abnormalities most compatible with MDS and/or AML. Based on the Heme Manual and DB, the 4/8/2010 diagnosis of MDS, NOS (9989/3) is the first primary. Should the 7/2/2010 diagnosis of transforming MDS to AML (9861/3) be a new, second primary? Based on the Abstractor Note for MDS in the Heme DB for MDS, "If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis." Based on this note, should the MDS histology code [9989/3] be changed to refractory anemia with excess blasts (RAEB-2) [9983/3] from the biopsy taken on 10/7/2010 (one day after treatment began) that revealed RAEB-2 with evolution towards AML? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. There is no time limit set to update histology to a more specific disease process if a patient has an initial NOS histology identified. Unlike solid tumors, hematopoietic and lymphoid neoplasms may take a year or more to manifest the specific disease. This is simply a part of the "disease characteristics." Abstract a single primary per M2, a single histology represents a single primary. Code the histology to 9983/3 [MDS/RAEB-2.] The Heme DB guidelines were interpreted correctly. MDS/RAEB can transform to AML and would be two separate primaries there had also been a reportable diagnosis of AML. The 7/2/2010 peripheral blood showed MDS and a clonal abnormality that was "compatible with MDS/AML." The 10/7/2010 bone marrow biopsy showed only RAEB-2 with "evolution towards AML with myelodysplasia related changes." Ambiguous terminology is only used to help determine reportability; it not used to code a more specific histology. In this case, there was only ambiguous terminology used to describe the AML. It is important to understand the implication of incorrectly assigning histology codes for hematopoietic and lymphoid neoplasm using ambiguous terminology. Using this case as an example, the patient was not treated until three months after the 7/2/2010 peripheral blood diagnosis of MDS compatible with MDS/AML. The medical literature indicates that AML, if left untreated, is usually fatal within 1-3 months. The treatment given 10/6/2010, 3 months after the "compatible with" diagnosis, was a drug used to treat MDS and not AML. The other issue with this case is that the bone marrow examination, which is more reliable than peripheral blood, showed only "evolution towards AML." This means that the bone marrow is exhibiting the changes seen in the final stages of MDS prior to progression to AML. Wait for a definitive diagnosis of AML and/or treatment for AML before abstracting the second primary. 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. |
2012 |
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20120067 | MP/H Rules/Histology--Thyroid: How is the histology coded for a poorly differentiated thyroid carcinoma with rhabdoid phenotype arising in a papillary carcinoma? | For cases diagnosed 2007 or later, code the histology as papillary carcinoma, poorly differentiated [8260/33].
The WHO classification lists grade III papillary carcinoma as one of the synonyms for poorly differentiated thyroid carcinoma.
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 Other Sites Histo rules because site specific rules have not been developed for this primary. Start with the SINGLE TUMOR: INVASIVE ONLY module, rule H8. The rules are intended to be reviewed in consecutive order within a module. Per rule H13 "phenotype" is not a term used to code a more specific histology. Moving to Rule H14 the histology is coded 8260/3 [papillary adenocarcinoma]. |
2012 | |
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20120002 | Histology/Diagnostic confirmation--Heme & Lymphoid Neoplasms: How are histology and diagnostic confirmation coded when a patient has a clinical diagnosis of lymphoma but a pathologic diagnosis of malignant neoplasm, NOS? See Discussion. |
This patient had CT scans showing extensive bilateral retroperitoneal lymphadenopathy suspicious for lymphoma and left axillary lymphadenopathy. Thin core biopsies were done of the left axillary lymph nodes and immunohistology pathology was read as malignant neoplasm with extensive necrosis. Flow cytometry analysis of the sample shows no definitive or sufficient CD45+ events for informative analysis. Karyotype analysis could not be performed on this specimen due to inadequate sample. FISH analysis using IGH break apart probe showed no evidence of clonal rearrangement in limited number of cells available for analysis. The physician's diagnosis is probable lymphoma, no further workup felt necessary because patient would not tolerate chemotherapy anyway and hospice was felt most appropriate care for patient.
The definitive diagnostic method for lymphoma, NOS is histologic confirmation, but the only histologic confirmation was of "malignant neoplasm with extensive necrosis." Should the histology and diagnostic confirmation be coded as lymphoma, NOS [9590/3] and imaging without microscopic confirmation [7] or malignancy, NOS [8000/3] and positive histology [1]? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the histology to 9590/3 [malignant lymphoma, NOS] and the diagnostic confirmation to 7 [radiology and other imaging techniques without microscopic confirmation]. Per the Diagnostic Confirmation Coding Instructions for Heme and Lymphatic Neoplasms, use code 1 when ONLY the biopsy was used to diagnose the specific histology. The biopsy only confirmed a malignancy; the scan confirmed the specific diagnosis of lymphoma.
Note that a clinical diagnosis can be a definitive diagnostic method for malignant lymphoma, NOS. In this case, the biopsy was inadequate and a more specific diagnosis could not be made by histology. Because no further work-up was pursued, this NOS diagnosis of malignant lymphoma was a clinical diagnosis only.
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. |
2012 |
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20120025 | MP/H Rules/Multiple Primaries--Brain and CNS: How many primaries are abstracted if a patient was diagnosed with metastatic malignant melanoma to the brain in 2003 and subsequently was diagnosed with meningeal melanomatosis? See Discussion. | Meningeal melanomatosis has a separate ICD-O-3 code, but is also a very rare form of melanoma. | This is a single primary coded to the site of the original melanoma. The brain and meninges are both metastatic sites. The MP/H Rules do not apply to metastases.
This case was sent to the melanoma physician specialists. The physician stated that, in this case, the meningeal involvement is secondary to the brain involvement (metastatic spread). Whenever brain metastases are diagnosed, the meningeal spread is metastatic. |
2012 |
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20120051 | MP/H Rules/Histology--Breast: What histology code for a diagnosis of pleomorphic lobular carcinoma in situ? | For cases diagnosed 2007 or later, code the histology as lobular carcinoma, in situ [8520/2].
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 Breast Histo rules because site specific rules exist for this primary.
Start at the SINGLE TUMOR: IN SITU CARCINOMA ONLY module, Rule H1. The rules are intended to be reviewed in consecutive order. Stop at the first rule that applies to the case you are processing. Code the histology to lobular carcinoma in situ [8520/2] because this is the only histologic type identified.
Pleomorphic lobular carcinoma is a variant of lobular carcinoma which does not have an ICD-O-3 code. It is still a lobular carcinoma. The identification of the variants of lobular carcinoma was a relatively recent discovery and the information was not available when the 2007 MP/H Rules were written. All of the lobular variants will be included in the next revision of the MP/H Rules. |
2012 | |
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20120063 | Reportability--Pancreas: Are neuroendocrine "tumors" reportable and are they synonymous with neuroendocrine "carcinoma"? See Discussion. | Example: Pancreatic mass that probably represents a neuroendocrine tumor is staged as cT2N0M0. | According to the World Health Organization (WHO) pancreatic neuroendocrine tumors (NET) are malignant. They are reportable.
For pancreas primaries, code NET, G1 (well differentiated) to 8240/3; NET G2 (moderately differentiated) to 8249/3; and nonfunctional NET, GI or G2 to 8150/3. The histology code for neuroendocrine carcinoma (NEC) is 8246/3, large cell NEC is 8013/3 and small cell NEC is 8041/3. |
2012 |
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20120059 | Primary site/Reportability--Breast: Is a "right nipple skin" biopsy that demonstrates squamous cell carcinoma reportable using a primary site of C500? See Discussion. | In the 2011 SEER Manual Reportability Examples, example 3, it states a "biopsy-proven squamous cell carcinoma of the nipple" is reportable when the subsequent resection shows "no evidence of residual malignancy in the nipple epidermis." However, this example does not specify the biopsy is from the nipple skin and the ICD-O-3 does not list nipple skin as a synonym for code C500. | Because the site is specifically stated to "skin" of nipple [C44.5], this case is not reportable.
If possible, you may wish to confirm the type of biopsy performed. If the biopsy was done by FNA or needle biopsy, the biopsy tissue should contain a full-thickness of skin and subcutaneous breast (nipple) tissue. If that is the case, this tumor would likely be a reportable squamous cell carcinoma of nipple [C50.0]. If, however, this was a punch biopsy it is more likely a non-reportable squamous cell carcinoma of the skin [C44.5]. |
2012 |
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20120041 | Primary site/Heme & Lymphoid Neoplasms: How is the primary site coded if the patient presents with diffuse B cell lymphoma involving the nasopharynx and right maxillary sinus with bilateral cervical, right supraclavicular and axillary lymph nodes? See Discussion. | There is one mass in the nasopharynx and right maxillary sinus and the site of origin cannot be determined for this diffuse B-cell lymphoma. The patient also has bilateral cervical, right supraclavicular and axillary lymph nodes.
Should the primary site be coded per Module 7 Rule PH25 because regional nodes are involved or Rule PH22 because both regional and distant nodes are involved? If rule PH22 is used, what is the primary site? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site to C119 [nasopharynx] per Rule PH25.
Per our subject matter expert, use Module 7 Rule PH25 to code the primary site to an organ (nasopharynx and maxillary sinus) because an organ(s) and its regional lymph nodes are involved. The distant lymph nodes are simply part of the staging (the lymphoma has progressed to another lymph node region).
Diffuse large B cell lymphoma originating in the oral cavity and maxillofacial region is rare, but documented. The most common sites for this rare neoplasm are Waldeyer ring, tonsils, nasopharynx, base of tongue, and palatine tonsil. There are also rare cases of diffuse large B cell lymphoma originating in the maxillary sinus. The percentage of cases arising in the nasopharynx is greater than those originating in the maxillary sinus.
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. |
2012 |
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20120017 | Reportability: Is a low-grade neuroendocrine neoplasm with gastrin expression found in a periportal lymph node reportable if the clinical impression is compatible with a gastrinoma? See Discussion. |
SINQ 20110095 states that "low-grade neuroendocrine neoplasm/carcinoid tumor with expression of gastrin" is reportable. However, in this case "carcinoid tumor" is not mentioned. Is this case reportable if the expression "carcinoid tumor" is missing in the diagnosis of the pathology report? Also, does the fact that the gastrinoma was found in a lymph node affect reportability? |
This is a reportable case. Code the histology as malignant gastrinoma [8153/3]. Gastrinomas are usually malignant. This one is apparently present in a metastatic site (periportal lymph node) which confirms the malignancy. |
2012 |
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