Report | Question ID | Question | Discussion | Answer | Year |
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20240056 | Reportability/Histology--Heme & Lymphoid Neoplasms: How should this unusual 2023 pathology-only case be reported and coded for leukemia cutis? See Discussion. |
10/25/2023: Patient presents to dermatology office with a questionable drug eruption having 3 weeks of papular eruptions of Trunk (Left Chest). Punch biopsies were taken that came back as immature hemopoietic infiltrate with monocytic differentiation. Comment: Myelodysplastic syndrome and leukemia cutis are possibilities. Addendum Report: Additional stains were prepared. ERG is strongly positive. CD1a and S100 do not stain the atypical cells.The controls stain appropriately. CD123 perform with appropriate control is also negative. The pattern is that of so-called "leukemia cutis" which could be seen in the clinical setting of myelodysplasia, chronic myelomonocytic leukemia (CMML) or precursor to acute myelomonocytic leukemia (AMML). Recommend work up. The only available information at present is a diagnosis of leukemia cutis, and that there was no prior history of a hematological malignancy in this patient. |
Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available. Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis. |
2024 |
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20240024 | Reportability/Histology: Is angiomyxoma (this includes borderline or behavior code /1 cases) of the soft tissue reportable? Can you provide us with coding guidelines for angiomyxoma for when its reportable or not reportable? |
Do not report angiomyxoma. ICD-O-3.2 assigns 8841/0 to this benign tumor. This includes superficial and deep (aggressive) angiomyxoma. |
2024 | |
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20240019 | Solid Tumor Rules/Histology--Head and Neck, Other Sites: Do human papilloma virus (HPV) histologies that occur with subtype/variants of squamous cell carcinoma (SCC) in various sites apply only to sites in Solid Tumor Rules, Head and Neck, Table 5 and Other Sites, Table 23? See Discussion. |
The 2024 Solid Tumor Rules, Table 5: Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids contain notes that say beginning 1/1/2022, keratinizing or non-keratinizing SCCs, HPV positive or HPV negative, are coded 8085 or 8086, respectively, for sites listed in the Head and Neck Solid Tumor Rules, Table 5 only. Table 5 introductory section also states for cases diagnosed 1/1/2023 forward: “When the diagnosis is a subtype/variant of squamous cell carcinoma and HPV status is also noted, code the subtype/variant.” This latter instruction is also included in Other Sites Table 23 (Penis and Scrotum Histologies) as a “Penis Coding Note.” Do these instructions ONLY apply to sites on those tables (and only to Penis or to Scrotum also in Table 23)? How should we code HPV-related keratinizing/non-keratinizing or other subtype/variant SCCs, for sites NOT on those tables, given the fact that only the more common histologies are listed in the Solid Tumor tables? For example, we recently reviewed a case with HPV-positive basaloid squamous cell carcinoma of the anus (C21.0). |
Code the specific histology as stated by the pathologist according to the site-specific instructions in the Solid Tumor Rules. When the histology provides a subtype/variant in addition to the HPV histology codes, code the subtype/variant as it is important to capture this histology as in the example provided. the instruction to code the subtype/variant over 8085 or 8086 applies to the following sites: oropharynx, cervix, vagina, vulva, anus, and penis. A note will be added indicatng this in 2025. Per 2024 Cancer PathCHART expert pathologist review, morphology codes 8085/3 and/or 8086/3 are valid and applicable to head and neck, oropharynx, cervix, vagina, vulva, fallopian tube, anus, and penis (reference: Cancer PathCHART: Product Downloads and Timelines). Other coding resources will be updated to reflect these changes in 2025. |
2024 |
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20240077 | 2024 SEER Manual/Primary Site--Retroperitoneum: What is the primary site code for a final diagnosis of endometrioid adenocarcinoma from a biopsy of a right retroperitoneal mass? See Discussion. |
An 80-year-old post-menopausal female (status post hysterectomy for benign reasons) presents with a retroperitoneal mass on imaging. The pre-operative imaging shows the cervix and uterus are absent. Patient undergoes a robotic left salpingo-oophorectomy with biopsy of the retroperitoneal mass. |
Code Primary Site to C480 (retroperitoneum). Endometrial tissue may "break away” from the uterus and implant throughout the pelvic and abdominal cavities. This can occur in patients who suffer from endometriosis. This tissue remains behind when surgical removal of the uterus is done. Common sites of implantation are colon, peritoneum, retroperitoneum, and bladder. These cells may become malignant. When the uterus is no longer present (patient had surgical removal), code the site where the carcinoma was identified. The site-morphology combination of C480 and 8380/3 was designated as an unlikely site-morphology combination by the Cancer PathCHART expert pathologist review, as this is a rare type of tumor. Assign a value of 1 in the Over-ride Site/Type [2030] data item in order to pass the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) edit. |
2024 |
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20240070 | Reportability/Histology: Does Cancer Pathology Coding Histology And Registration Terminology (Cancer PathCHART) determine if the histology is reportable or do we have to use the Excel ICD-O-3.2 spreadsheet? |
The CPC ICD-O-3 Site Morphology Validation Lists (SMVLs) designate all tumor site-morphology combinations that are either valid or impossible as determined for the sites reviewed by the Cancer PathCHART initiative. These lists provide information on the Validity Status of specific tumor site and morphology combinations, similar to the way the ICD-O-3 SEER Site/Histology Validation List used to. However, the CPC SMVLs do not include information on the reportability of specific tumor site and morphology combinations. For tumor reportability, you will continue to use the Excel ICD-O-3.2 spreadsheets posted to the NAACCR ICD-O-3 Coding Updates website: https://www.naaccr.org/icdo3/, and the most recent SEER Manual and federal, state, local, and other standard setters' reportability requirements. |
2024 | |
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20240011 | Solid Tumor Rules/Histology--Other Sites: Other Sites Table 2 (Mixed and Combination Codes) requires site designations; can sites be added? See Discussion. |
There are multiple possible entries (rows) for a tumor with a neuroendocrine component and non-neuroendocrine component, but these rows do not specify which primary sites are applicable. Row 1 (Combined small cell carcinoma, 8045) seems applicable to a prostate primary, but not to a GI primary since GI primaries are now generally referred to as MiNENs (mixed neuroendocrine non-neuroendocrine tumors), but Table 2 does not provide any instructions regarding how to determine the difference between 8045 and 8154 (or 8244). For SEER Workshop Case 03 (mixed prostate case), many users selected 8154 or 8244 as the mixed histology code per Table 2, but these histology codes are not listed as applicable in Table 3 (Prostate Histologies). Per the WHO Blue Books, these histologies are not listed as applicable to the prostate. How are registrars to determine the correct mixed code without site designations, especially if they don't have access to the WHO Blue Book or to a pathologist who may be able to clarify the codes? |
Sites may be added to certain combinations when indicated by ClinCORE review for Cancer PathCHART. Please note some sites were added in the 2024 update as a result of PathCHART review. A newly-formed Solid Tumor Editorial Board and its subgroups are currently working to evaluate the Solid Tumor Manual and make recommendations on ways to improve the structure and formatting of the manual and its content. Follow the rules and instructions in the Other Sites STRs when assigning combination histology codes. Histology Coding Rules Use the Histology Coding Rules when assigning combination codes. Coding Histology Information Use this section that includes the mixed histology (Table 2) and site-specific histology tables (Tables 3-23) for one or more histologies within a single tumor. Do not use this section in place of the Histology Coding Rules. While site-specific histology tables, based on current WHO Classification of Tumors books, have been added to Other Sites STRs, not all site groups have individual histology tables; coding may require the use of ICD-O and updates. The histology tables in Other Sites STRs include additional coding instructions and notes to assign the correct ICD-O code when appropriate. The tables are not meant to be all-inclusive; rather they are intended to address difficult coding situations to facilitate the assignment of the correct histology code. Table 2: Mixed and Combination Codes Instructions Once you have identified the histology terms and have been instructed to use Table 2 by the Histology Coding Rules, compare the terms in the diagnosis to the terms in Column 1. When the terms match, use the combination code listed in Column 2. Use adenocarcinoma mixed subtypes 8255 as a “last resort” code. |
2024 |
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20240066 | Histology--Heme & Lymphoid Neoplasms: How should histology be coded for a pathologic diagnosis of “Follicular lymphoma, diffuse pattern grade 3A of 3, equivalent to diffuse large B cell lymphoma (germinal center cell type)” when later referenced clinically as follicular lymphoma grade 3A? See Discussion. |
The WHO Classification of Hematopoietic Tumors (Blue Book), 5th edition states: “Rare cases of classic follicular lymphoma with cytological features of follicular lymphoma (FL) grade 3A can present with a prominent diffuse pattern. In the previous edition, such cases were defined as diffuse large B-cell lymphoma (DLBCL). Currently, it is uncertain whether such cases should be classified as FL or diffuse large B-cell lymphoma; and in such cases, individual treatment choices should be made in multidisciplinary conference settings taking into consideration clinical, laboratory, and imaging parameters. The presence of diffuse areas composed entirely or predominantly of large cells, however, warrants a diagnosis of diffuse large B-cell lymphoma.” Our concern is that the Hematopoietic (Heme) Manual and Database do not provide instruction for coding this scenario. We hesitate to interpret the terms “equivalent to” as ambiguous because one could argue it is unambiguous. Barring this argument, the M and H rules would indicate this is a diagnosis of diffuse large B-cell lymphoma. However, the physician does not seem to agree with the pathologist. |
Assign histology as DLBCL (9680/3) as supported by the WHO Classification of Hematolymphoid Tumors, 5th edition. It is consistent with how it would have been coded in the 4th edition. The Heme Manual and Database currently are based on the 4th edition. Physicians are using the 5th edition blue book, whereas the cancer registry field is not yet at this time. Regarding the Heme Manual and Database, this type of scenario is not covered because it is part of the 5th edition WHO Blue Book. The database cannot be updated until the 5th edition is approved for implementation (2026). |
2024 |
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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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20240025 | Update to the current manual/Reportability--Esophagus: Is high grade dysplasia of the esophagus reportable? The 2024 Seer Program Manual, page 21, has an example that states it is not reportable. See Discussion. |
Example 4: Esophageal biopsy with diagnosis of “focal areas suspicious for adenocarcinoma in situ.” Diagnosis on partial esophagectomy specimen “with foci of high grade dysplasia; no invasive carcinoma identified.” Do not accession the case. The esophagectomy proved that the suspicious biopsy result was false. Appendix E2 #32 of the SEER Manual states high grade dysplasia in site other than stomach, small intestines, and esophageal primary sites are not reportable. Does this mean high grade dysplasia is reportable for esophagus primaries? |
High grade dysplasia of the esophagus is reportable. The example will be corrected in the next edition of the SEER manual. |
2024 |
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20240004 | Reportability/Histology--Skin: Is a malignant spindle cell neoplasm consistent with atypical fibroxanthoma reportable for cases diagnosed 1/1/2023 and later, after thorough immunohistochemical work-up? See Discussion. |
Appendix E1 in both the 2023 and 2024 SEER Program Coding and Staging Manual (SPCSM) lists these malignant spindle cell neoplasms, consistent with atypical fibroxanthoma, as reportable when other tumors have been ruled out with immunohistochemistry. This contradicts both SINQ 20190102 and the Solid Tumor Rules (STRs) general instructions indicating ambiguous terminology (e.g., “consistent with”) cannot be used to code the more specific histology when there is a NOS (malignant spindle cell neoplasm, 8004/3) and a more specific (malignant atypical fibroxanthoma, 8830/3) histology. These tumors are typically diagnosed and treated in dermatology offices, so further chart review or confirmation by a physician is not possible for central registries. As non-melanoma skin primaries are included in the Other Sites schema, and this schema was updated for cases diagnosed 2023 and later, which instruction applies to 2023+ diagnoses? Should these continue to be collected per Appendix E1 despite the conflict with the STR Manual and SINQ? If these are reportable, should the SINQ and STR Manual be updated to reflect this? Or should these be non-reportable per the STR Manual and SINQ? |
Report malignant spindle cell neoplasms consistent with atypical fibroxanthoma as directed by Appendix E.1 of the 2023 and 2024 versions of the SEER Manual using 8830/3 (fibroxanthoma, malignant). We will update the answer in SINQ 20190102. While the Other Sites Solid Tumor Rules address coding an NOS and specific histology sub-type/variant, this situation is not specifically addressed. We will also review the rules. |
2024 |