| Report | Question ID | Question | Discussion | Answer | Year |
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20200040 | Reportability--Skin: Is pseudomyogenic hemangioendothelioma (PMH) reportable with morphology code 9133/3? See Discussion. |
According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis. 12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen. Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note. NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma. 12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note. Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma. |
Do not report PMH. The WHO Classification of Skin Tumors lists pseudomyogenic hemangioendothelioma as a borderline malignancy (9138/1). Borderline malignancies of the skin are not reportable. |
2020 |
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20200005 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what M rule applies when a patient is diagnosed with both plasmablastic lymphoma and at least one plasmacytoma? See Discussion. |
The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019. In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma. Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma. Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process. |
Abstract multiple primaries using the Heme and Lymphoid Rule M15. The Multiple Primaries Calculator shows that the plasmablastic lymphoma (9735/3) and extraosseus plasmacytoma (9734/3) are separate primaries. We also checked with our expert pathologist who concurs as the spinal lesion was not reviewed to prove that it is plasmablastic lymphoma, therefore, the diagnosis as per pathology remains plasmacytoma. |
2020 |
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20200004 | Solid Tumor Rules (2018)/Multiple primaries--Lung: How are Primary Site and EOD Primary Tumor coded when a patient is diagnosed with four invasive tumors in the right lung that represent three separate primaries, but the not otherwise specified (NOS) tumor and one of the specific subtype/variants are in separate lobes? See Discussion. |
There are four invasive tumors in the right lung: Large cell undifferentiated carcinoma in the right lower lobe (8012/3, C343); Adenocarcinoma, acinar-predominant in the right lower lobe (8551/3, C343) that was 0.7 cm in size and limited to the lung; Mucinous adenocarcinoma in the right upper lobe (8253/3, C341) that was 0.9 cm and limited to the lung; Adenocarcinoma, NOS also in the right upper lobe (8140/3, C341) that was 1 cm and limited to the lung. The Lung M Rules confirm the large cell undifferentiated carcinoma is a separate primary from the three adenocarcinoma tumors (Rule M8). The acinar adenocarcinoma and mucinous adenocarcinoma tumors are separate primaries (Rule M6). The adenocarcinoma, NOS tumor is the same primary as both the acinar and mucinous are adenocarcinomas (Rule M7). How is Primary Site coded for both the acinar and mucinous adenocarcinomas if they represent multiple tumors reported as a single primary (when compared to the adenocarcinoma, NOS tumor)? Should the adenocarcinoma, NOS tumor also be included when coding EOD Primary Tumor for both the right lower lobe acinar adenocarcinoma and right upper lobe mucinous adenocarcinoma primaries? Further follow-up with the physician is not possible. |
Abstract three primaries using 2018 Lung Solid Tumor Rules, Rule M6 and M8 as these are multiple synchronous tumors. M6 (Subtypes in Column 3 of Table 3): Adenocarcinoma, acinar predominant: Primary Site: C343 (RLL) EOD Primary Tumor: 300 Mucinous adenocarcinoma Primary Site: C341 (RUL) EOD Primary Tumor: 300 M8 (Separate rows in Table 3): Large cell undifferentiated carcinoma: Primary Site: C343 (RLL) EOD Primary Tumor: 300 Note: The adenocarcinoma, NOS, along with the other subtypes, is on a different row than the large cell undifferentiated carcinoma and is already accounted for in Rule 6 as multiple synchronous tumors. Do not include the adenocarcinoma, NOS in EOD Primary Tumor for the reportable primaries. |
2020 |
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20200049 | Summary Stage 2018/EOD 2018--Lymphoma Orbital Adnexa: What is the correct Summary Stage 2018 (SS2018) for the site/histology Orbit, NOS (C696), 9699/3? In SEER*RSA, Extent of Disease (EOD) Primary Tumor references code 7 (Distant), whereas SS2018 assigns code 2 (Regional)? See Discussion. |
We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses |
To clear this edit of the derived Summary Stage (based on EOD) and the manually assigned Summary Stage (based on Summary Stage 2018), assign the manually assigned Summary Stage to 7. For this particular case, EOD Primary Tumor 700 (which is correct based on the information received) derives Distant; however, for Summary Stage 2018, this description is under Code 2 for Regional by direct extension. This is an error. For 2022, Summary Stage for Lymphoma Ocular Adnexa description under Code 2 (Regional by direct extension) will be moved to Distant. No changes will be done to EOD. |
2020 |
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20200035 | Reportability/Ambiguous Terminology--Brain and CNS: Is the expression differential considerations a synonym for differential diagnoses? See Discussion. |
Example: An MRI Spine showed a large expansile mass arising from the sella turcica and extending into the suprasellar cistern, but the radiologist only noted: The leading differential considerations include pituitary macroadenoma or a large suprasellar base meningioma. The patient was subsequently pathologically diagnosed with a pituitary adenoma. It is unclear if the diagnosis date should be coded to the MRI date. There are two existing SINQ questions regarding the term consider. SINQ 20061094 confirms a diagnosis that is considered to be is reportable because it is unambiguous, but SINQ 20081033 states the phrase malignancy is highly considered is not a reportable ambiguous term. How should we interpret differential considerations? If differential considerations is equivalent to a differential diagnosis, then this patient was clinically diagnosed on imaging. However, if differential considerations is not reportable, then there was no diagnosis prior to the resection. |
In an ideal situation, the radiologist should be consulted to determine what he/she meant by "differental considerations." If that is not possible, given the context and usage, "differential considerations" in this case can be interpreted as differential diagnoses. And since the two differential considerations are both reportable, this case is reportable as of the date of the MRI. |
2020 |
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20200067 | Summary Stage 2018/Extension--Colon: What is the Summary Stage for adenocarcinoma of cecum where the tumor extends into the proximal portion of attached vermiform appendix? See Discussion. |
2020 Diagnosis: Patient had a right hemicolectomy showing adenocarcinoma of cecum, tumor extends into proximal portion of attached vermiform appendix. Tumor invades through muscularis propria into pericolorectal tissues (NOS). Regional lymph nodes: 06/39. Primary Tumor EOD: Where does the appendix involvement come into coding or will this be based on the pericolorectal tissue (NOS) invasion? What is my Summary Stage? I know it is at least 3 due to regional ln involvement, but the appendix involvement is making me question 3 vs 4. |
Assign code 4, Regional by BOTH direct extension AND regional lymph node(s) involved. In this case, the Regional component for Summary Stage 2018 is based on Note 6, under Colon and Rectum where Regional is defined as: -Mesentery -Peritonealized pericolic/perirectal tissues invaded [Ascending Colon/Descending Colon/Hepatic Flexure/Splenic Flexure/Upper third of rectum: anterior and lateral surfaces; Cecum; Sigmoid Colon; Transverse Colon; Rectosigmoid; Rectum: middle third anterior surface] -Pericolic/Perirectal fat |
2020 |
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20200070 | Solid Tumor Rules (2018)/Multiple Primaries--Breast: The December 2020 revision to 2018 Breast Solid Tumor Rules, Rule M10, using behavior rather than timing to determine the number of primaries, has caused synchronous separate/non-contiguous tumors reported as invasive carcinoma, NST (8500/3) and lobular carcinoma in situ (8520/2) (or vice versa) to be reported as separate primaries per Rule M14. Should an invasive carcinoma NST and a synchronous, separate lobular carcinoma in situ be separate primaries per M14? See Discussion. |
Recognizing the addition of the behavior requirement into this rule is an attempt to stop non-synchronous ductal carcinoma and lobular carcinomas from being accessioned as a single primary (SINQ 20200022), the issue with using behavior rather than timing is that now, synchronous separate/non-contiguous tumors that are invasive carcinoma NST (8500/3) and lobular carcinoma in situ (8520/2) (or vice versa) are separate primaries per M14. Lobular and carcinoma, NST are separate rows in Table 3, so we cannot stop at M10 and code the mixed histology because there are two separate histologies with different behaviors. There is no rule that states we can just ignore the in situ tumors for the purpose of applying the M Rules. (We are instructed to ignore the in situ when coding histology only in certain circumstances.) The problem with Rule M10 appears to be related to timing. This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
The original issue with M10 was with registrars being instructed that multiple in situ and invasive tumors were a single primary and then coding 8522/3 when one tumor was in situ and one was invasive. This incorrectly identified both components as being malignant (/3). Our effort to correct this misconception apparently did not work. M10 has been revised to state that yes, an in situ lobular or duct plus an invasive lobular or duct is a single primary with a new note that states: When a mixture of behaviors is present in carcinoma, NST, and lobular carcinoma, follow the H rules to determine the correct histology code. They will stop at H8 which instructs them to code the invasive histology. 8522/3 should only be used when both components are invasive. |
2020 |
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20200006 | Reportability--Retina: Is a diagnosis of retinal astrocytoma reportable? See Discussion. |
There is no specific ICD-O-3 code for a which resulted in abstractors assigning the malignant astrocytoma, NOS code. These lesions were previously called but we are seeing the new terminology more frequently. |
Report retinal astrocytoma. The WHO Classification of Tumors of the Eye, 4th edition, lists astrocytoma, NOS as 9400/3 with astrocytic hamartoma of the retina as a synonym. You may receive a site/type edit (IF25) which can be overridden. The changes in terminology, codes, etc. proposed in WHO 4th Ed Eye book were implemented for cases diagnosed 1/1/2018 forward. Apply this to retina astrocytomas and do not accession cases diagnosed with this histology prior to 1/1/2018. |
2020 |
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20200081 | Solid Tumor Rules (2018)/Histology--Pancreas: How is the histology coded, and what H Rule applies, for a 2021 diagnosis when the pathological diagnosis is neuroendocrine tumor (NET) G1 or NET G2, but clinically, the tumor is stated to be insulinoma? See Discussion. |
Insulinoma, NOS is reportable for cases diagnosed 2021 and later. However, the diagnosis of insulinoma is most frequently made with clinical correlation of the patient's clinical syndrome and serum hormone levels. Despite a pathological diagnosis of NET, this will clinically be stated as insulinoma based on the functional type of tumor. At the largest facility in our area, all pathology reports with a diagnosis of insulinoma over the last year only provide a pathological Final Diagnosis of NET (either G1 or G2), but elsewhere specify, Functional Type: Pancreatic neuroendocrine tumor, functional. Correlation with Clinical Syndrome and Elevated Serum Levels of Hormone Product: Insulin-producing (Insulinoma). For 2021 and later, it seems this should be accessioned as insulinoma (8151/3), but one cannot arrive at that histology using the current Other Sites (MP/H) H Rules. Following the existing rules, one would code the histology to NET, G1 or NET, G2 (8240 or 8249) per Rule H6. There are technically two specific histologies to consider: NET (either 8240 or 8249) and insulinoma, NOS (8151). Following the H Rules, Rule H6 instructs one to code the histology with the numerically higher ICD-O-3 code (8240 or 8249). Coding this histology to NET (8240 or 8249) does not seem to reflect the most accurate classification of this tumor, but applying the current rules, this is the only histology that can be coded. There is no current guideline in the Other Sites schema or the ICD-O-3.2 Implementation Guidelines instructing us to ignore the pathological diagnosis of a NET for these tumors (even though insulinomas are NETs). The only SINQ that currently exists (SINQ 20150019) states the histology can be coded as either a NET or an insulinoma in these cases. How are registrars to consistently code histology for these tumors without a rule clarification? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
Code the tissue/pathology histology over the clinical diagnosis. Because of implementation timelines, a comprehensive revision to Other Sites rules will not be available 2022. A limited revision is planned and histology tables will be added for select sites. The General Instructions will also be revised for Other Sites. |
2020 |
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20200085 | Solid Tumor Rules (2018)/Histology--Head and Neck: What is the histology of paraganglioma, NOS arising outside of the adrenal gland (for example, in the bladder) for cases diagnosed 1/1/2021 and later? See Discussion. |
Should histology be coded as paraganglioma, NOS (8680/3) or as extra-adrenal paraganglioma, NOS (8693/3) for a diagnosis of paraganglioma in the bladder? Does the pathologist have to specifically diagnose the tumor as extra-adrenal paraganglioma, NOS to use histology code 8693/3? Or, does any diagnosis of paraganglioma (NOS) arising outside of the adrenal gland, carotid body, middle ear, or aortic body (the specified sites for other types of paragangliomas) qualify as an extra-adrenal paraganglioma, NOS? The ICD-O-3.2 Implementation Guidelines (Tables 6 and 7) provide an associated site of C755 for histology 8680/3 (paraganglioma, NOS), but no associated site code is provided for histology 8693/3 (extra-adrenal paraganglioma, NOS). If the preferred site for paraganglioma, NOS is the paraganglia, would a paraganglioma in the bladder be an extra-adrenal paraganglioma? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
Code the histology stated by the pathologist: paraganglioma, NOS 8680/3. |
2020 |
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