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A patient had posterior cervical lymphadenopathy status post biopsy and subsequent lymph node dissection showed metastatic melanoma in 2018. Workup showed no skin lesions or primary site. Final diagnosis is melanoma of unknown primary (unknown if cutaneous or non-cutaneous). Should C760 be used as the primary site for this case since the histology codes of 8700-8790 are included in the Cervical Lymph Nodes and Unknown Primary Tumors of the Head and Neck schema in SEER*RSA?

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.

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.

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.

The Terms and Definitions Introduction discusses how these are older terms, but pathologists may still use them. In our region, pathologists do, in fact, still use these terms. Can these terms be added to Table 1? For registrars who do not reference the Introduction every time they code histology but go directly to Table 1, coding consistency would likely improve if such terms were added in the Table.

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

The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that,

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.

The bone marrow biopsy proved bone marrow with blasts comprising 15-19%. Neither the pathologist nor the physician specifically diagnosed this as AML, calling this only high risk disease or early evolving AML prior to starting the patient on Vidaza.

No further information can be obtained from the pathologist or the physician for this case. Should this early evolving AML be accessioned as an additional primary per Rule M10, or is this the same MDS that is now high risk as the blast count is up to 19%, but has not yet reached the threshold of 20% blasts usually required for AML per the Hematopoietic and Lymphoid Neoplasm Database?

Lip has not been added to any of the site-specific histology tables, nor has any other instruction been provided for coding tumors in this site.

Coding histology for lip primaries is difficult because registrars do not know where to look first. The Solid Tumor Rules indicate one should use the tables first, but then do not inform registrars what table to use for a lip primary (i.e., a specific table, any table, no table).

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