Report | Question ID | Question | Discussion | Answer | Year |
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20200008 | Solid Tumor Rules (2018)/Multiple primaries--Corpus uteri: How many primaries are accessioned for patient with a minimally invasive endometrial adenocarcinoma arising in a polyp in 2001, followed by a metastatic poorly differentiated clear cell carcinoma of gynecologic (GYN) origin in 2019? See Discussion. |
The patient has a history of a minimally invasive endometrial adenocarcinoma that was low grade and confined to an endometrial polyp in 2001. The patient underwent a total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) that entirely removed the tumor at that time. Almost 18 years later, the patient had a left inguinal mass excision that was, Carcinoma of gynecologic origin, consistent with clear cell carcinoma. No other disease was found, the physician never indicated whether this was felt to be metastatic from the previous, low grade adenocarcinoma or not. It was only noted as, an unusual malignancy of the left lower quadrant and inguinal region of gynecologic origin. No further information was available in the medical record or from the physician on follow-up. Although neither the Solid Tumor Rules nor the MPH Rules (still in use for the Other Sites schema) apply to metastasis, given the differences in histology and behavior of these two tumors (i.e., minimally invasive, low grade disease diagnosed in 2001 vs. higher grade, more aggressive tumor in 2019) should the current clear cell carcinoma of GYN origin really be the same primary as the 2001 endometrial adenocarcinoma? |
Abstract a multiple primaries using 2018 Other Sites Solid Tumor Rule M10 as these tumors are more than one year apart. This represents endometrioid adenocarcinoma (8380/3 of C541) and 18 years later, clear cell Carcinoma (8310/3 consistent with GYN (C579) primary). |
2020 |
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20230016 | Solid Tumor Rules/Histology--Brain: How is histology coded for an anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III, diagnosed following a right temporal lobe resection in 2021? See Discussion. |
The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation. The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology. |
Assign histology as 9505/3. WHO Classification of Central Nervous System (CNS) Tumors describe ganglioglioma as a well-diffferentiated and slow-growing glioneuronal neoplasm. While WHO does not recognize the histology/behavior combination 9505/3, the 2021 CNS Solid Tumor Rules identify non-malignant tumors that have the potential of transforming to a malignant tumor (new primary). Ganglioglioma (9505/1) is listed with the transformed histology and instructs us to code as anaplastic ganglioglioma (9505/3). |
2023 |
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20180088 | Solid Tumor Rules (2018)/Multiple primaries--Prostate: How many primaries are abstracted and what M Rule applies when a patient is diagnosed with prostate adenocarcinoma in 2014, followed by liver mass biopsy showing neuroendocrine carcinoma, small cell type of the prostate in 2018? See Discussion. |
The patient has a history of prostate adenocarcinoma with lymph node metastases, status post prostatectomy and treatment by Lupron in 2014. The most recent prostate serum antigen measurement (April 2018) was normal. CT scan of the abdomen and pelvis revealed new hypodense liver lesions, a slightly enlarging lung right lower lobe nodule, and enlarging lobular mass in the prostatectomy bed. The core liver biopsy contains areas of metastatic tumor with a differential diagnosis on pathology of high-grade neuroendocrine carcinoma of the prostate (small cell type), which may have been seen in association with prostate adenocarcinoma, or metastatic small cell carcinoma of a different site. Clinically, the physician impression is that this represents metastatic castration-resistant prostate cancer. The Solid Tumor Rules note that the Multiple Primary Rules are not used for tumor(s) described as metastases. However, SINQ 20130221 indicates that, at least historically, these would have been accessioned as multiple primaries (histology 8140 & 8041 per Rule M10). Does the previous SINQ note still apply to these types of cases, and if so how would one know to move beyond the initial note indicating metastases are not new primaries? |
The guidance provided in SINQ 20130221 still applies. Accession two primaries, adenocarcinoma [8140/3] of the prostate [C619], followed by small cell (neuroendocrine) carcinoma [8041/3] of the prostate [C619] for each of the examples given per Rule M10 of the 2018 Solid Tumor Rules, Prostate. In each case, the second histology (because it is not adenocarcinoma) is a new prostate primary. Small cell carcinoma and small cell neuroendocrine carcinoma are not adenocarcinomas. As a result, they are not covered by Rule M3. For the case described in this SINQ submission, based on the findings of a lobular mass in the prostate bed, this is a second primary (there is residual prostatic tissue). This is unchanged from the 2007 Multiple Primaries Rules for Other Sites. |
2018 |
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20110031 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if patient initially diagnosed with granulocytic sarcoma on a vocal cord biopsy is subsequently diagnosed with acute myeloid leukemia more than 21 days later? See Discussion. | The patient has a history of refractory anemia with excess blasts diagnosed in 2008. A vocal cord biopsy performed on 6/2/2010 stated, "in view of a previous history of myelodysplastic syndrome this is indicative of transformation to acute leukemia" and consistent with granulocytic sarcoma. A bone marrow biopsy done on 7/19/2010 stated this was compatible with refractory anemia with excess blasts in transformation.
Granulocytic sarcoma is a solid manifestation of AML. When these diagnoses occur more than 21 days apart, are they separate primaries?
According to the WHO definition, this is acute myeloid leukemia complicating myelodysplasia. Which rule applies for this case? |
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. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.
As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.
In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.
Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.
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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20230078 | Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: Should the primary site be coded C421 (bone marrow) for a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) when the managing physician provides a Rai stage? See Discussion. |
The patient has adenopathy and a lymph node biopsy proved CLL/SLL. The patient underwent a peripheral blood smear, but the final diagnosis only indicated there is an abnormal CLL panel, positive for monoallelic or biallelic deletion of 13q. The pathologist noted a CLL related clone was detected, but there was no definitive diagnosis of CLL on the peripheral blood. No bone marrow biopsy was performed. However, the managing physician noted this was Rai Stage I CLL/SLL with adenopathy in the neck. The SSDI Manual notes, “Rai stage is only applicable for CLL, in which the bone marrow and/or peripheral blood are involved (primary site C421 for bone marrow, see Hematopoietic Manual, Module 3: PH 5, 6).” Should primary site default to C421 if the physician provides a Rai Stage in the absence of definitive peripheral blood or bone marrow involvement documented in the medical record? |
Assign primary site C421. The Site-Specific Data item (SSDI) Manual, Rai Classification section, states: Per confirmation from medical oncologists, Rai stage is only recorded for patients who have bone marrow and/or peripheral blood involvement. Per the Hematopoietic Rules, primary site would be C421 (See Hematopoietic Manual, Module 3: Rules PH 5, 6). A new code has been added to the 5 SSDIs (code 5) to use when primary site is not C421. |
2023 |
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20230077 | EOD 2018/ Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: How are Primary Site and Extent of Disease (EOD) Primary Tumor coded when a lymph node biopsy proved chronic lymphocytic leukemia (CLL), and the peripheral blood is involved with an “abnormal CD5-positive B-cell population”? See Discussion. |
The patient has adenopathy in multiple lymph node regions above and below the diaphragm and a lymph node biopsy pathology proved CLL/small lymphocytic lymphoma (SLL). Further work-up with peripheral blood proved an abnormal CD5-positive B-cell population comprising only a small percentage of the white blood cells (WBCs). The pathologist noted this neoplastic B-cell population comprises “3.5% of white blood cells and has an immunophenotype characteristic of CLL/SLL and is similar to the recent lymph node biopsy in this patient.” The managing physician indicated this was a Lugano Stage III SLL. The registrar coded the peripheral blood involvement in EOD Primary Tumor. If this small percentage of WBCs with an abnormal B-cell population is included in EOD Primary Tumor as peripheral blood involvement, then this would indicate peripheral blood/bone marrow involvement and primary site would need to be coded to C421 per Rule PH5. Rules PH5 and PH6 confirm primary site must be coded C421 if peripheral blood or bone marrow are involved. Is there a cutoff value for these abnormal B-cell populations in the peripheral blood? Or should these abnormal B-cell populations be ignored unless the pathologist states the abnormal B-cell population is consistent with CLL/SLL (not just immunophenotypically characteristic of CLL/SLL)? |
Primary site would be C421 based on Hematopoietic and Lymphoid Neoplasm Manual, Module 3, Rule PH 5. Assign EOD Primary Tumor to code 800 (peripheral blood involvement WITH other involvement). Per consultation with an expert hematologist oncologist, this is a Stage IV CLL/SLL since the peripheral blood is involved. There is no cutoff value for the abnormal B-cell populations in the peripheral blood when the cells are consistent with CLL/SLL. If the peripheral blood is involved, even only slightly, it is a Stage IV CLL/SLL. Our expert stated that the physician's staging was wrong (this is not a Lugano, Stage III). |
2023 |
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20170050 | First course of treatment/Other therapy--How do you code medical marijuana when given as "treatment?" See Discussion. |
The patient has gastric cancer and the physician prescribed medical marijuana as treatment. SEER*Rx says marijuana is ancillary as a psychoactive cannabinoid and antiemetic and advises not to code it. The physician specifically wrote "treatment with" in the record. Should it be coded as Other (Code 1) under Other Therapy? |
Do not code as treatment. Enter the information regarding the use of marijuana in a text field. There have been some early clinical trials of cannabinoids in treating cancer in humans and more studies are planned. While the studies so far have shown that cannabinoids can be safe in treating cancer, they do not show that they help control or cure the disease. At this time, marijuana is used to treat side-effects (such as nausea, vomiting, and pain) and to help increase appetite which helps patients tolerate standard therapies. |
2017 |
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20180087 | Solid Tumor Rules (2018)/Multiple Primaries--Brain: How many primaries are there and what M Rule applies when two tumors identified in the brain are pathologically proven to be glioblastoma, IDH-wild type and anaplastic astrocytoma per the pathology report final diagnosis, but the diagnosis comment and tumor board indicates multifocal glioblastoma is favored? See Discussion. |
The patient has one tumor each in the left parietal and left medial temporal lobe. The tumors were excised. The final diagnosis for the left parietal tumor is glioblastoma, IDH-wild type. he final diagnosis of the left medial temporal tumor is, at least anaplastic astrocytoma, WHO grade III; see comment. The comment states: There is a single focus of vascular hyperplasia, separate from neoplastic cells. No necrosis is identified. These findings on their own would warrant a diagnosis of anaplastic astrocytoma, WHO grade III. However, in the context of the patient's glioblastoma in the left parietal lobe, and imaging showing ring-enhancing lesions of the parietal and temporal lobes, this specimen is favored to be an un-sampled glioblastoma, WHO grade IV. The Solid Tumor Rules indicate we may no longer use terms like favor(s) to code the histology, leaving the final diagnosis as the priority source for coding histology per the Histology coding rules. The tumor board review confirmed that, despite the anaplastic astrocytoma on pathology, they felt strongly that this is a multifocal glioblastoma and not an anaplastic astrocytoma. Both the pathologist's comment and the tumor board's assessment indicate this patient does not have two primaries. However, the Solid Tumor Rules do not give priority to the tumor board's assessment over the pathology, and registrars are not to use ambiguous terms to code histology thus leaving the two histologies to consider. Per the Solid Tumor Rules, one tumor that is glioblastoma and one tumor that is anaplastic astrocytoma are multiple primaries per M11 (Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant). As a central registry, we cannot ask the pathologist or attending physician for clarification as suggested in Section 3 of the Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions. We can only follow the current Solid Tumor Rules. In doing so, we would have to ignore both the pathologist's and tumor board's assessment that this patient has multifocal glioblastoma. Is there any concern that this will lead to over-reporting? |
Abstract separate primaries based on the two histology codes as these are separate tumors on different rows in Table 3 of the 2018 Solid Tumor Rules for Malignant CNS, Rule M11. The priority order for using documentation to identify histology for Malignant CNS is to use pathology/tissue from the resection over the tumor board. |
2018 |
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20180039 | Solid Tumor Rules 2018/Histology--Testis: What is the histology code for a 2018 diagnosis of left testis tumor diagnosed as mixed germ cell tumor with secondary malignant components: primitive neuroectodermal tumor (PNET) and rhabdomyosarcoma? See Discussion. |
The patient has testicular cancer with bilateral lung metastases and possible liver metastasis. The left orchiectomy final diagnosis was The Summary describes a single tumor that is, Germ cell neoplasia in situ (GCNIS) is also present. Although there is mixed germ cell tumor present, the PNET component of the tumor is locally invasive extending into the epididymis, hilar soft tissues, spermatic cord, and tunica vaginalis. The mixed germ cell tumor is limited to the testis only. We are instructed not to use to the term to code histology in the MP/H Rules General Instructions (Other Site Rules not updated for 2018), however the PNET comprises the majority of this tumor and represents the most extensive disease. Should the PNET histology be ignored in this case as its a ? |
Assign code 9084/3. According to our expert pathologist consultant, this is a teratoma with a somatic-type malignancy. This code is the best choice even though it does not capture the mixed germ cell elements of the tumor, or the character of the somatic component (rhadomyosarcoma, PNET).There aren't enough histology code numbers to cover all of the possibilities. Use text fields to describe the specifics of this case. |
2018 |
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20190103 | Solid Tumor Rules/Multiple primaries--Brain and CNS: What M rule applies to a clinically diagnosed right-sided parietal meningioma undergoing active surveillance, followed by a left-sided frontal anaplastic oligodendroglioma? See Discussion. |
The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma. The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor. Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states: --Patient had a resection of the non-malignant tumor (not the same tumor) OR --It is unknown/not documented if the patient had a resection. This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor? |
Apply 2018 Malignant CNS Solid Tumor Rule M5 and abstract multiple primaries when there are multiple CNS tumors, one of which is malignant /3 and the other is non-malignant /0 or /1. According to Note 3, a non-malignant CNS tumor and a malignant CNS tumor are always multiple primaries (timing and primary sites are irrelevant). Prepare two abstracts; one for the non-malignant and another for the malignant tumor. |
2019 |