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Rule H13 states to code the histology to metaplastic carcinoma when there is metaplastic carcinoma (or a subtype/variant) and invasive carcinoma NST. This rule makes no mention of lobular carcinoma. However, in Table 3, Note 2 for metaplastic carcinoma (8575) states metaplastic carcinoma, NOS and subtypes are almost always mixed with invasive mammary carcinoma, NST and at times lobular carcinoma. These tumors should be coded to metaplastic regardless of percent invasive mammary carcinoma or lobular carcinoma present.

While Table 2 (the mixed histology code table) does include an entry for metaplastic carcinoma AND carcinoma NST OR lobular carcinoma, it is unclear why lobular carcinoma has not been added to Rule H13 as well.

If a single tumor has metaplastic plus lobular carcinoma, Rule H13 does not apply and one has to continue through the rules. Unfortunately, the next rule registrars would be tempted to use is Rule H18: Code the histology that comprises greater than 50% of tumor when two histologies are on different rows in Table 3. This Rule does not state it does NOT apply to metaplastic carcinoma (only mucinous). So, if for some reason the lobular was greater than 50%, the incorrect histology would be coded (unless the registrar happened to remember Note 2 in the metaplastic carcinoma entry in Table 3).

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

11/18/2019 Left 1st Digit/Thumb Biopsy: Atypical Melanocytic Proliferation consistent with Early Acral Lentiginous Melanoma in situ. Margins Positive. (Not a reportable diagnosis for 2019.)

12/5/2019 Left 1st Digit Shave Biopsies: Malignant Melanoma in situ. Margins Positive.

1/15/2020 Started Aldara (treatment plan: use for ~3 months then Mohs/excision, but due to COVID could not get resection until 7/2020).

7/29/2020 Left Thumb Excision: Residual Melanoma in situ. Margins Positive. Treatment Plan: re-excision.

8/6/2020 Left Thumb Re-Excision: Atypical Lentiginous Melanocytic Proliferation at the 12-2 margin may represent the advancing edge of melanoma in situ. (8/19/2020 Plan to treat the 12-2 margin as positive with in situ; plan for re-excision).

8/20/2020 Left Thumb Re-Excision & Left Nail Plate Excision: Malignant Acral Lentiginous Melanoma with extensive melanoma in situ. Breslow 1.3mm. Margins Positive. Nail plate & bed epithelium with hemorrhage and a mild increase in melanocyte density likely represent melanoma in situ.

9/4/2020 Left thumb partial amputation & Left axillary Sentinel Lymph Node Excision: Residual Malignant Melanoma in situ. 0/3 sentinel nodes positive.

In Table 3 (Specific Histologies, NOS/ NST, and Subtypes/Variants), the entry for papillary carcinoma, NOS includes a change in column 3 of the 2018 Breast Solid Tumor Rules that conflicts with the H Rules. It is not accounted for in the change log. No explanation is offered as to why this change was made. This is a major change because encapsulated papillary carcinoma is frequently associated with carcinoma NST, and we have not been collecting these as such.

Encapsulated papillary carcinoma (8504) in column 3 now includes an indented entry, with invasive carcinoma, NST/invasive duct carcinoma 8504/3. However, most encapsulated papillary carcinomas are in situ or there is no definitive statement of invasive encapsulated papillary carcinoma, so when in situ and invasive tumors are present, we are instructed to code the invasive histology (which would be the invasive carcinoma (NST), 8500/3). How are registrars to arrive at the correct histology without a new H rule or a clarification regarding this update being documented in the change log?

Does the same change/addition apply to solid papillary carcinoma? These are often also associated with carcinoma, NST. Again, without an explanation regarding the change mentioned above, it is difficult to understand why the change was made.

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Example: Diagnosis is made on liver core biopsy path showing Metastatic carcinoma, poorly-differentiated, consistent with lung primary. Diagnosis Comment notes: Carcinoma cells are positive for CK7 and TTF-1, negative for CK20.

Subsequent immunohistochemistry report for PD-L1 testing states Liver: Metastatic adenocarcinoma consistent with lung primary. Interpretation: no PD-L1 expression.

IHC/Biomarker testing is often performed to determine treatment type, but it seems like some of the biomarkers for treatment planning are also histology specific. The Solid Tumor Rules do not address the use of biomarkers reports in the histology coding instructions.

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.

Per the 2018 Solid Tumor Rules instructions, Final Diagnosis and Staging Summary (synoptic report) have equal coding priority. However, it is unclear which takes priority, or if this should be a combination of components, when the histologies are two different specific histologic types per Table 3 of the Breast Solid Tumor Rules Manual.

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.

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.

10/4/2018 Frozen Section Diagnosis: Brain tissue with atypical cells and inflammatory cells, defer to permanents for further evaluation. Note: Tissue for flow cytometry is submitted. Final Diagnosis: Preliminary Diagnosis: Brain Tumor, Biopsy: Neoplasm, suggestive of lymphoma (see comment). Comment: The tumor exhibits nuclear atypia and increased mitosis. The tumor cells are immunologically positive for LCA and with very high ki67 labeling index. GFAP and synaptophysin are not expressed by tumor cells. The above suggests a lympho-proliferative process. This case is forwarded to the hematopathology service of this department for further evaluation. The final diagnosis report will be issued by the hematopathologist as an addendum.

Supp Rpt Add Addendum Diagnosis: The brain biopsy showed brain tissue large lymphoid cell infiltrate. Additional immunohistochemical stains are performed. The large cells are positive for CD20, BCL2, BCL6 (subset), MUM1, and CD30, negative for CD3, CD5, and CD10. Staining for c-MYC is negative. Ki-67 positive large cells are approximately 18%. EBER is strongly positive by ISH. Diagnosis: Brain lesion, biopsy: EBV+ Diffuse Large B-cell Lymphoma. Addendum Comment: The concurrent flow cytometric study showed monoclonal lambda-positive B-cells without out CD5 and CD10 expression, consistent with B-cell lymphoma.