Report | Question ID | Question | Discussion | Answer | Year |
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20100037 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be accessioned for a patient diagnosed with essential thrombocythemia [9962/3] in 2002 who had a 2010 biopsy consistent with the fibrotic stage for a chronic myeloproliferative disorder that "suggests the patient is transforming to an acute myeloid leukemia"? See Discussion. |
Patient had a diagnosis of essential thrombocythemia [9962/3] in 2002 and was treated with Hydroxyurea. In 2010, the patient was admitted with severe bone pain and a diagnosis described as, "The overall features of the biopsy are consistent with a fibrotic stage of a chronic myeloproliferative disorder. The presence of up to 15% CD34+ immature cells seen in the biopsy suggests that the patient is transforming to an acute myeloid leukemia." In addition, cytogenetic studies and molecular testing for JAK2 were ordered. These findings confirmed a myeloproliferative disorder. JAK2 mutation was not detected. The patient died within 2 weeks. Is this a new primary?
Was this patient diagnosed with AML (which requires 20% or more blasts and this is only 15%)? If this is a new primary, is the histology 9861/3 [AML, NOS] or 9895/3 [AML with myelodysplasia-related changes]? Was the second diagnosis of AML definitively diagnosed? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This case is a single primary, essential thrombocythemia [9962/3] in 2002. The 2010 diagnosis is chronic myeloproliferative disorder [9960/3].
According to Rule M15, the Multiple Primaries Calculator is to be used to first determine the number of primaries. Per the calculator, essential thrombocythemia and chronic myeloproliferative disorder are the same primary. (Acute myeloid leukemia is not used as the second histology because it is preceded by a non-reportable ambiguous term, "suggests." "Suggests" is not on the list of reportable ambiguous terms in the Hematopoietic and Lymphoid Neoplasm Coding Manual.
In 2010, this patient was in a late stage of ET. When any of the specific MPN neoplasms such as ET are in the late stage of disease, the characteristics of the specific disease (ET) will no longer be detectable. Accordingly, for this patient the diagnostic testing was positive for MPN, unclassifiable. In this case, do not change the diagnosis from the more specific disease (ET) to the NOS (MPN, unclassifiable).
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. |
2010 |
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20100081 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Should a single primary be accessioned with the histology coded 9732/3 [multiple myeloma] when a patient is diagnosed initially with a plasmacytoma on an excision and a single bone marrow biopsy showed only 4% plasma cells, then the subsequent workup led to a clinical diagnosis of multiple myeloma? See Discussion. | This patient had a plasmacytoma removed from the sphenoid sinus and was started on Dexamethasone. The patient had a bone marrow biopsy with 4% plasma cells. A statement in the hematology notes read, "it can increase the rate of false negative results with a bone marrow biopsy." The bone marrow biopsy was done 15 days after the surgery for the plasmacytoma.
Workup yielded the diagnosis of multiple myeloma. Per a statement in hematology notes, "I found her having 4% blasts, atypical plasma cells in the bone marrow biopsy and also lytic lesions involving the T7 and lucencies involving L4 and L5 vertebral bodies and also the upper sacrum. The PET-CT scan did not show significant metabolic activities in those lesions. The patient had a small amount of Bence-Jones in the urine and also an abnormal kappa to lambda ratio in the serum. The ratio was 12 to 1. The beta 2 microglobulin was 1.4. The albumin in the serum was 3.4. Based on that, the patient has been diagnosed with Durie-Salmon stage III in ISS stage II multiple myeloma."
The abstractor notes for multiple myeloma state that the diagnosis is made when the proportion of plasma cells in the bone marrow is 10% or greater. Should a diagnosis of MM be accessioned and coded when the bone marrow is less than 10% plasma cells, but a clinical diagnosis of MM is made? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accept the physician's diagnosis of multiple myeloma [9732/3]. Code the multiple myeloma as a single primary using rule M8 if there was only ONE positive biopsy. Code as multiple primaries (both the solitary plasmacytoma and multiple myeloma) using Rule M11 if there are TWO positive biopsies, one confirming the chronic neoplasm and the other confirming the acute neoplasm.
Per the Heme DB Abstractor Notes: The registrar DOES NOT CODE plasma cell myeloma based on the percentage of plasma cells. There must be a diagnosis of plasma cell myeloma. In addition, a clinical diagnosis of plasma cell myeloma may be made based on amyloidosis with associated renal impairment, anemia and/or hypercalcemia supported by radiologic evidence of multiple lytic bone lesions. he biopsy confirmed plasma cell malignancy (plasmacytoma) and the clinical workup confirmed myeloma.
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. |
2010 |
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20100002 | Reportability/Histology--Colon: Is a colon tumor reportable if the pathology report final diagnosis is high grade dysplasia but CAP protocol histologic type designation is adenocarcinoma in situ? See Discussion. | The microscopic description and the final diagnosis on the pathology report indicate the tumor is a large tubulovillous adenoma of the cecum with focal surface high grade dysplasia. The CAP protocol histologic type designation is adenocarcinoma in situ and pT designation is pTis. Which has priority? Is the case reportable? | The case is reportable because carcinoma in situ is stated. Carcinoma in situ has higher priority than severe dysplasia or high grade dysplasia. Per AJCC 6th edition colon chapter, the terms "high grade dysplasia" or "severe dysplasia" may be synonymous with carcinoma in situ. Because the pathologist gave carcinoma in situ information within the CAP, (s)he is apparently defining the dysplasia as in situ carcinoma. |
2010 |
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20100107 | MP/H Rules/Histology--Kidney, renal pelvis: How is histology coded for a tumor described as "renal cell carcinoma, clear cell with rhabdoid features"? See Discussion. | Is the statement "with __ features" indicative of a specific type of renal cell carcinoma (that is not represented by a specific histology code) or a second histologic type? Per ICD-O, "malignant rhabdoid tumor" is coded 8963/3. "Rhabdoid" is not listed in Table 1 in the MP/H rules as a specific type of renal cell carcinoma. |
Rhabdoid features occur in about 5% of all renal cell cancers and indicate a more aggressive tumor. Per WHO, these tumors comprise approximately 2% of all pediatric tumors with a median diagnosis age of 1-2 years old. This diagnosis is highly suspect in patients over age 3. Most previously reported rhabdoid tumors over age 5 have subsequently proven to be renal medullary carcinomas.
For cases diagnosed 2007 or later, if the patient in this case is a child, apply Kidney Rule H7 and code histology to 8963/3 [malignant rhaboid tumor]. Otherwise, we strongly suggest you consult with the pathologist to determine if this is truly a rhabdoid rather than a medullary tumor. |
2010 |
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20100024 | Histology: How is this field coded for a perivascular epithelioid cell neoplasm (PEComa) of uncertain malignant potential that is malignant based on the presence of metastases? See Discussion. |
In 11/2006 the patient had surgery for a 6cm mass in the RUQ arising in the falciform ligament. The pathologic final diagnosis was: Perivascular epithelioid cell neoplasm (PEComa) of uncertain malignant potential. In 10/2009 a liver biopsy showed metastatic perivascular epithelioid cell neoplasm. |
Assign histology code 8005/3 [malignant clear cell tumor]. According to our expert pathology consultant, this is the best histology code available at this time for the occasional tumor which is designated as malignant. The appearance of metastatic disease clearly defines this case as malignant. |
2010 |
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20100053 | Primary site--Heme & Lymphoid Neoplasms: How is primary site coded for a myeloid sarcoma (granulocytic sarcoma) arising in the chest wall in a patient that has a negative bone marrow biopsy? See Discussion. | Patient was diagnosed with Myeloid Sarcoma (granulocytic sarcoma) by chest wall biopsy. This is an extramedullary manifestation of acute leukemia and is not in the bone marrow (bone marrow is negative).
How should primary site be coded? The Heme DB states that almost any part of the body can be involved. It also states to not code primary site to C421. In this case the only involvement is the chest wall [C493]. However, use of the primary site code C493 triggers an edit error questioning this site/histology combination. If the primary site is coded to C421 [bone marrow], there is no edit error. Please explain the site code and rationale. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Unless there are scans showing involvement of a lymph node or tissue other than the chest wall, the histology should be coded myeloid sarcoma [9930/3] and the primary site to C493 [soft tissue of chest wall]. Per Rule PH 30, use the Heme DB to determine primary site and histology when rules PH1-PH29 to not apply. Override the edit.
Per the Abstractor Notes section in Heme DB, for myeloid sarcoma [9930/3] the most frequently affected sites are skin, lymph nodes, gastrointestinal tract, soft tissue, and testis.
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. |
2010 |
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20100010 | MP/H Rules/Multiple primaries--Ovary: How many primaries are to be abstracted when a patient is diagnosed with serous cystadenocarcinoma [8441] of the right ovary and clear cell adenocarcinoma [8310] of the left ovary? See Discussion. |
Patient had bilateral ovarian tumors. The right ovary had serous cystadenocarcinoma and left ovary had clear cell adenocarcinoma. The pathology COMMENT section stated, "Based on the histologic differences of the tumors within each ovary, feel these represent two distinct separate primaries. Lymph node metastases are clearly serous ca." The physician staged the right ovary as T2a N1 M0 and left ovary as T1c N0 M0. Do we accession one primary per rule M7 [Bilateral epithelial tumors (8000-8799) of the ovary within 60 days are a single primary]? What is intention of Rule M7? If the histology in each ovary is different but within the range (8000-8799), is that supposed to be accessioned as one primary? Or is the intention of Rule M7 that tumors in both ovaries must have the SAME histology within that histology range to be a single primary? |
For cases diagnosed 2007 or later, apply rule M8 and abstract this case as multiple primaries. Rule M7 does not apply when each ovary has a distinctly different histology, even when both histologies are with the specified code range. This clarification will be added to the next version of the rules. |
2010 |
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20100006 | MP/H Rules/Multiple primaries--Kidney: In a patient with a history of renal cell carcinoma, would a new primary be accessioned per Rule M10 for a soft tissue mass in the renal fossa not stated to be a metastasis but that was referred to as recurrent renal cell carcinoma, clear cell per the excision pathology report? See Discussion. |
This patient was diagnosed with clear cell carcinoma of the right kidney in 2003, treated with nephrectomy. The tumor was limited to the kidney. An FNA of the pancreas in 11/07 was consistent with metastatic renal cell carcinoma. In 2009 the patient was diagnosed with a right renal fossa mass by CT. The mass was excised on 8/26/09 and showed, "recurrent renal cell ca, clear cell." The path specimen was labeled as, "soft tissue, rt renal fossa." The original 2003 slides were not reviewed and the renal fossa mass was not described as being metastatic. If the renal fossa soft tissue mass is a new tumor, the MP/H rules for Other Sites directs you to code it as a new primary per rule M10 [Tumors diagnosed more than one (1) year apart are multiple primaries]. Would this be a new soft tissue tumor per rule M10? Or would this be a recurrence of the original kidney primary? |
For cases diagnosed 2007 or later: This is not a new primary. The patient has metastatic disease from the 2003 kidney primary. Clear cell carcinoma metastasized to the pancreas in 2007 and to the right renal fossa in 2009. |
2010 |
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20100105 | Surgery of Primary Site--Brain and CNS: Is "debulking" of a primary brain tumor coded to 21 [subtotal resection of tumor] or 30 [gross resection of tumor]? | Assign code 21 [subtotal resection of tumor, lesion, or mass]. Debulking removes as much of the tumor volume as possible in cases where it is not possible to remove the entire tumor. Debulking should improve the effectiveness of subsequent radiation therapy and/or chemotherapy. | 2010 | |
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20100071 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed in February 2010 with a plasmacytoma of the frontal skull followed by a diagnosis of smoldering myeloma by bone marrow biopsy? See Discussion. | The patient had a diagnosis of solitary plasmacytoma of the right frontal skull in 2/2010 that was totally resected (the cranial specimen final diagnosis was plasmacytoma). The patient received radiation. While undergoing radiation, the patient was seen by a medical oncologist who did a bone marrow biopsy that revealed 10-15% plasma cells, and was called smoldering myeloma. Watchful waiting was recommended. In 8/2010, the patient had multiple lytic lesions and began systemic treatment.
Per rule M15 and the Multiple primary calculator, 9731/3 [plasmacytoma] and 9732/3 [smoldering myeloma] is accessioned as two primaries. When the manual states, "Use the Hematopoietic Database to determine the primary site and histology when PH1-PH29 do not apply," does this mean to use the calculator not the database itself? By the old rules this was one primary. Did this change for cases diagnosed 1/1/10 and later? Which M rule is the correct rule to apply? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
The smoldering myeloma is a second primary per Rule M10. Accession as multiple primaries because this case was originally diagnosed as a chronic neoplasm (plasmacytoma)phase and there was a second diagnosis of an acute neoplasm (multiple myeloma) more than 21 days after chronic diagnosis. See note 1 which indicates, "This is a change from previous rules." Note that the MP rules and the MP calculator in the Heme DB agree.
When the rules tell you to go to the DB to determine the histology and primary site, you use the DB information. (Don't forget to check the Abstractor Notes). The multiple primaries calculator is used to determine the number of primaries to abstract. Always use the M rules before using the MP calculator.
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. |
2010 |