Report | Question ID | Question | Discussion | Answer | Year |
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20240048 | Solid Tumor Rules/Histology--Breast: What is histology code of a breast tumor with ductal carcinoma, lymphoepithelioma-like carcinoma type? See Discussion. |
Example: 12/2023 Breast lumpectomy final diagnosis is Invasive ductal carcinoma, lymphoepithelioma-like carcinoma type. This is a single tumor with no in situ carcinoma present. Lymphoepithelioma-like carcinoma is not listed as a subtype/variant or synonym for breast carcinoma in the Solid Tumor Rules histology tables. |
Lymphoepithelial carcinoma is a subtype of SCC usually seen in skin or H&N sites and often associated with EBV. CPC SME review determined 8082/3 invalid for breast but did not recommend a substitute code. There were only 45 cases coded 8082 2001 to 2019. For this case, it's possible the lesion originated in the breast skin and progressed to breast tissue. SCC is a subtype of metaplastic breast carcinoma so one could argue it code be coded either 8575 or 8070. For this case, we recommend assigning 8500/3. Use text fields to record the details. |
2024 |
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20240017 | EOD/Prostate Pathologic Extension--Prostate: Is a pathology report from a prostate biopsy/transurethral resection of the prostate that states "with intraductal spread" extraprostatic/extracapsular extension or localized? |
Code as a localized, intracapsular tumor as ductal carcinoma in situ does not invade. Intraductal spread is describing the neoplasm spreading through the acinar/ductal cells in the prostate specimen. It is an in-situ type of spread and not invasive but almost always presents with an invasive tumor. |
2024 | |
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20240050 | Solid Tumor Rules/Multiple Primaries--Vulva: Why is there no M Rule in the Other Sites Multiple Primary Rules related to extramammary Paget disease of the vulva? See Discussion. |
The only Other Sites H Rule related to extramammary Paget disease is included in the Multiple Tumors Abstracted as a Single Primary module. Rule H28 instructs one to code the histology of the underlying tumor when there is extramammary Paget disease and an underlying tumor of the anus, perianal region, or vulva. Therefore, a vulvar extramammary Paget disease with underlying adenocarcinoma is coded as adenocarcinoma (8140/3), and not extramammary Paget disease (8542/3). However, there is no M Rule confirming extramammary Paget disease and the underlying adenocarcinoma are a single primary (i.e., multiple tumors abstracted as a single primary) making it difficult for one to use the Multiple Tumors Abstracted as a Single Primary H rules module. We recognize this is a longstanding histology coding rule, but how are registrars supposed to arrive at Rule H28 when the M Rules must be applied first and do not instruct one to accession a single primary? Moreover, if this is to be a single primary (per rule M2), why is there no H Rule in the Single Tumor module? |
In sites other than breast (see Breast Solid Tumor Rules M8/M9), Paget disease with underlying invasive disease is a single primary and the underlying histology is coded. Primary Paget disease of the vulva is uncommon, and we cannot create rules for all possible situations in the Other Sites module. A GYN specific module is in development, and we will look into adding a Paget-related rule. It will differ because Paget in breast is a different situation while Paget in the vulva is always adenocarcinoma. Paget disease of the vulva is an in-situ adenocarcinoma of vulvar skin with or without an underlying adenocarcinoma (WHO Classification of Female Genital Tumors, 5th ed.). When there is a statement of “underlying” adenocarcinoma, it is a single primary as with Breast Solid Tumor Rule M8. |
2024 |
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20240049 | First Course Treatment/Neoadjuvant Therapy--Breast: When are pre-operative therapies given as part of a clinical trial coded as neoadjuvant treatment versus limited systemic exposure in the Neoadjuvant Therapy data item? See Discussion. |
The SEER Manual seems to give somewhat conflicting instructions for clinical trial therapies under the Neoadjuvant Therapy data item. One section states that limited systemic therapy may occur in clinical trials to impact the biology of a cancer, but is not a full course of neoadjuvant therapy with the intent to impact extent of surgical resection or other outcomes (organ preservation, function or quality of life); do not code as neoadjuvant therapy for the purposes of this data item. Then another section states for purposes of this data item, the criteria for neoadjuvant therapy include that treatment must follow recommended guidelines for the type and duration of treatment for that particular cancer site and/or histology, and that neoadjuvant therapy may be given as part of a clinical trial. For example, a patient was diagnosed with invasive ductal carcinoma of the breast, 6 cm in size; treatment planning conference recommended neoadjuvant chemotherapy. The patient elected to participate in a clinical trial and was assigned to a group given the antibody drug conjugate datopotamab deruxtecan (Dato-DXd) plus durvalumab for 12 weeks. There was no physician documentation of intent or expected outcomes, nor yC staging or statement of clinical response. Post-therapy imaging showed no residual mass, and post-therapy mastectomy path report showed only residual ductal carcinoma in situ, stating "Treatment Effect (after neoadjuvant): Residual Cancer Burden - pCR, In the breast - complete response." The medical oncologist stated post-therapy stage was ypTis ypN0 cM0. The trial drugs this patient were given do not appear to be approved or standard neoadjuvant/pre-operative drugs in SEER*Rx or NCCN guidelines for this type of cancer; however, the duration of treatment was fairly substantial, and although we don't have clear documentation from physicians as recommended in the SEER manual (which is usually not stated, in our experience), it seems like they may be considering it as neoadjuvant therapy. How should the Neoadjuvant Therapy data item be coded for cases like this? What is the best way to differentiate between clinical trial therapies that are "limited systemic exposure" (code 3) versus true neoadjuvant therapy (code 1)? |
When pre-operative therapies are given as part of a clinical trial, code as neoadjuvant treatment in the Neoadjuvant Therapy data item when the intent is neoadjuvant and/or when surgical resection follows the clinical trial therapies. In the example, neoadjuvant chemotherapy was recommended in the treatment planning and the patient had the planned resection after neoadjuvant treatment. The treatment effect outcome is based on imaging that reported no mass and as documented by the physician, pathologist in this case as complete response to the neoadjuvant therapy based on the resection. Use code 3 (limited systemic exposure) when treatment does not meet the definition of neoadjuvant therapy in the data item, Neoadjuvant Therapy. Limited exposure occurs when the patient receives some therapy prior to surgical resection, but the treatment is not enough to qualify for a full course of neoadjuvant therapy with the intent to impact extent of surgical resection or other outcomes. While this type of treatment may given as part of a clinical trial, it mostly refers to short term treatments such as hormone therapy. When neoadjuvant therapy is given prior to surgical resection that is planned (intended) or performed to improve outcomes, use Code 1 or 2. Because a clinical trial is a type of research study that tests new methods of screening, prevention, diagnosis, or treatment of a disease, the treatment regimens likely will not be incorporated in recommended guidelines until all phases of the trial are completed and approved by the U.S. Food and Drug Administration. ClinicalTrials.gov is available to learn more about clinical studies around the world. |
2024 |
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20240041 | Reportability--Brain and CNS: Is an optic nerve meningioma reportable if stated to arise in the “intraorbital segment” of the optic nerve meninges? See Discussion. |
Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma. Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location. It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable. |
Report optic nerve sheath meningioma arising in the intraorbital segment. The optic nerve contains four segments, of which intraorbital is one. The WHO Classification of Eye Tumors, 4th edition, defines meningioma as a neoplasm originating from the meningothelial cells of the optic nerve leptomeninges. According to the Table 3 of the Non-malignant Solid Tumor Rules, all portions of the optic are reportable and meningiomas arising in the dura/meninges of an intracranial nerve are coded to cerebral meninges C700. |
2024 |
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20240028 | 2024 SEER Manual/Primary Site--Breast: Is Primary Site coded as C504 or C501 based on the Solid Tumor Rules and the SEER Manual Breast Coding Guidelines? The pathology report reads "Right Breast 10:00 1 cm from the nipple." Codes C502-C505 take priority over code C501. The description for C501 in the Solid Tumor Rules has "Area extending 1 cm around areolar complex." |
Assign Primary Site code C504 based on the location in the upper outer quadrant of the right breast, 10 o’clock, as opposed to code C501, around the areolar complex. The 2024 SEER Manual Breast Coding Guidelines advise that C502 - C505 are generally preferred over C501 when there is no other way to determine the subsite. |
2024 | |
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20240002 | First Course Treatment--Heme & Lymphoid Neoplasms: How should treatment data items be coded for a diagnosis of myelodysplastic syndrome (MDS) and symptomatic anemia treated with Reblozyl (Luspatercept)? See Discussion. |
Example: Patient has a 04/2023 diagnosis of symptomatic anemia not responsive to Retacrit. Further testing includes diagnostic bone marrow biopsy 10/2023 proving MDS with low blasts and SF3B1 mutation, treated with Relozyl (Luspatercept). There is no SEER*Rx listing for Reblozyl or Luspatercept. Per web search, Luspatercept, sold under the brand name Reblozyl, is a medication used for the treatment of anemia in beta thalassemia and myelodysplastic syndromes. Is this non-cancer directed treatment since it is given to address the anemia rather than the MDS? If cancer-directed treatment, how should it be coded? |
Do not code Reblozyl (luspatercept) as treatment. Luspatercept is an ancillary drug approved to treat anemia associated with MDS but not the malignancy. |
2024 |
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20240009 | Solid Tumor Rules/Histology --Brain and CNS: Why is high grade astrocytoma with piloid features (HGAP) not grouped together with the other astrocytoma histologies as a subtype/variant of astrocytoma? See Discussion. |
It appears there was some confusion about finding this new malignant HGAP tumor (2023+) code. If this is not a specific subtype/variant of astrocytoma, can clarification be added to the “New for 2023” entry for HGAP? |
HGAP is listed as a separate classification and is not a subtype of the diffuse gliomas. WHO Classification of Tumors of the Central Nervous System, 5th edition, has two categories dealing with non-pediatric astrocytic tumors: Adult-type diffuse gliomas Circumscribed astrocytic tumors HGAP falls into the second category as a result of updates to the 4th edition WHO classification in 2016 with advances in the role of molecular diagnostics with the 5th edition. All astrocytic tumors were previously grouped together whereas not all diffuse gliomas (astrocytic or not) are grouped together on the basis of growth pattern and behaviors, and shared IDH1 and IDH2 genetic status. The new classification separates astrocytomas that have a more circumscribed growth pattern, lack IDH gene alterations, and sometimes have BRAF mutations (i.e., pilocytic astrocytoma). The impact of molecular advances has driven classification changes as described in the 5th edition. Review of site/histologiy combinations for CNS neoplasms is currently being performed by Cancer PathCHART experts. It's possible they will recommend HGAP be moved to a subtype/variant of astrocytoma, NOS. |
2024 |
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20240052 | Reportability/Histology--Heme & Lymphoid Neoplasms: Should a non-Langerhans cell histiocytosis involving the hypothalamus and pituitary gland be accessioned as a reportable, behavior /1, CNS neoplasm? See Discussion. |
Imaging identified a mass involving the hypothalamus and pituitary gland and excision of the mass proved “histiocytosis.” The case was extensively reviewed, and the physician notes this patient has a pituitary tumor that is a “non-Langerhans cell histiocytosis,” or a “non-LCH histiocytic neoplasm.” There is no histology for histiocytosis (NOS) or non-Langerhans cell histiocytosis. However, this does appear to be a non-malignant histiocytic neoplasm. If this were a Langerhans cell histiocytic neoplasm in the CNS it would be reportable. Should this non-Langerhans cell histiocytic neoplasm also be accessioned as a reportable CNS neoplasm? If so, how is the histology coded? |
Report this case as a pituitary tumor (8000/1) based on the information provided. This is the best choice as no specific histology code exists for this generic term “non-Langerhans cell histiocytosis” in ICD-O-3.2, WHO Classification of CNS Tumors, 5th ed., and WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th ed. Be sure to double-check the behavior code of the tumor. Histiocytosis can be benign, borderline, or malignant. There was no mention of the behavior so we defaulted to uncertain behavior for this case. |
2024 |
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20240040 | First course treatment--Kidney: How should the different treatment fields be coded if surgery is planned but cancelled due to patient noncompliance, then the tumor is treated with ablation, and eventually surgery is given due to residual disease? See Discussion. |
Patient was diagnosed in July 2022 with biopsy confirmed left kidney renal cell carcinoma. Initially, partial nephrectomy was planned for February 2023 but canceled at the last moment due to the patient’s “history of narcotic use.” The details of that cancellation were otherwise unclear. It appears the treatment plan was changed due to patient non-compliance. Patient then had cryoablation of the tumor in May of 2023. Subsequent imaging in October found residual tumor, but no disease progression was noted. Again, additional ablation was offered but patient decided on surgical treatment which did not occur until December 2023. Is the cryoablation second course due to a change of plan if there is no disease progression, recurrence, or treatment failure? If the cryoablation is first course treatment, then would the partial resection also be first course treatment because it was documented as the treatment plan? |
The treatment with cryoablation is second course. Once the initial treatment plan is changed, everything after the change is no longer first course of treatment. If the cryoablation was not mentioned as part of the original treatment plan, it is second course. |
2024 |