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Example: Patient diagnosed following a resection of a cystic mass at L2-4 that proved an intradural tumor excision with final diagnosis of schwannoma, WHO grade 1.

Per new Solid Tumor Rules, the primary site in this case should be coded C476 (peripheral nerves of trunk, NOS) and histology is 9560/0 (schwannoma, NOS). However, there are currently no coding options in the Soft Tissue of Trunk and Extremities EOD schema relating to a benign tumor. Likewise there are no coding options in the Soft Tissue and Sarcoma Summary Stage 2018 schema relating to a benign tumor.

How should EOD 2018 and Summary Stage 2018 be coded for reportable benign schwannomas of the spinal nerve roots?

Example: Patient had a 2023 nasopharyngeal mass biopsy showing “Nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type.” 

The Head and Neck Solid Tumor Rules (STRs) do not include an H Rule that instructs us how to code histology when there are two subtypes/variants present for a head and neck primary, nor does the STR define undifferentiated carcinoma as a subtype/variant for 8072.

The WHO Classification of Head and Neck Tumors states non-keratinizing nasopharyngeal carcinoma (non-keratinizing squamous cell carcinoma (SCC) is the most common subtype for nasopharyngeal ca, but that non-keratinizing can be subdivided into undifferentiated and differentiated subtypes.

Should histology be 8020 (undifferentiated carcinoma) or 8072 (non-keratinizing SCC)?

Example: Patient had a diagnosis on February 2003, plasmacytoma of the sinus; June 2003, plasmacytoma of the alveolar ridge; July 2003, plasmacytoma of the skin; and June 2004, multiple myeloma.

If this represents a transformation of plasmacytomas to multiple myeloma, will the information on multiple myeloma be available for statistical and research purposes?

Example: Patient had an original diagnosis of small lymphocytic lymphoma (9670/3) of lung in 1986 and later presents with small B-cell non-Hodgkin lymphoma (9670/3) of small bowel in 2008 at Hospital A. Slides sent for review at Hospital B where patient was also seen. Slides there read as low grade B-cell lymphoma most consistent with extranodal marginal B-cell lymphoma of mucosal associated tissue (MALT Lymphoma). Hospital A's pathology report stated that immunostains would exclude mantle cell lymphoma and MALT lymphoma and the original pathology report has not been amended to match the outside path diagnosis. Is this a second primary of MALT lymphoma (9699)?

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?

Example: Patient has a 2023 brain MRI described as having a “new dural based nodule, statistically meningioma, along the left distal tentorial incisura.” All subsequent chart information is related to patient’s unrelated diagnosis of multiple sclerosis only.

Is the terminology “statistically” reportable ambiguous terminology in this context?

Example: Patient has a left cerebral meningioma that is meningothelial meningioma (9531) and two right-sided cerebral meningiomas: one that is transitional meningioma (9537) and the other that is meningioma, transitional and angiomatous, WHO Grade I. If the histology for the mixed tumor is 9534 (angiomatous meningioma), then there are three primaries. If the histology is 9537 (transitional meningioma), then there are two primaries.

Per Table 6, angiomatous meningioma is 9534/0 and transitional meningioma is 9537/0. There is no mixed histology coding rule, or mixed histology meningioma code. There is also no default rule that would instruct registrars to code the numerically higher ICD-O code or to default to a meningioma (NOS) histology code.

Example: Patient has a secretory carcinoma of minor salivary gland tissue (mammary analogue secretory carcinoma [MASC]) of the mucosal lower lip; it is unclear which table to use and how to arrive at the correct histology using the H Rules.

Rule H1 (code the histology when only one histology is present) states, Note 1: Use Tables 1-9 to code histology. There is no table that includes the lip. The correct histology should be 8502 which is listed in Table 6 (Tumors of Salivary Glands) however this does not correspond to minor salivary glands of the mucosal lip (site C003 per ICD-O-3 coding instruction).

The 2018 ICD-O-3 Update table does not include this histology, however Table 6 indicates code 8502 (secretory carcinoma) is a new code that was approved by IARC/WHO.

The ICD-O-3 only includes this histology as secretory carcinoma of breast. Therefore, in order to arrive at the correct histology, one must be aware of previous SINQ entries 20160036 and 20130003 that indicate secretory carcinoma (or MASC) is histology 8502. However, these are related to MP/H Rules, so registrars may be hesitant to apply this guideline to cases coded using Solid Tumor Rules.

Example: Patient has left thigh skin excision with final diagnosis of atypical smooth muscle cell proliferation, inked peripheral margin is involved and inked deep margin is free of disease in the sections examined. See Comment.

Diagnosis comment states: The terminology regarding this lesion is controversial. Lesions with identical features are designated as leiomyosarcoma in the dermatopathology literature, whereas, the preferred classification in the soft tissue pathology is atypical intradermal smooth muscle neoplasm. Although the lesion appears predominantly dermal based, since the margin is involved, the lesion cannot be entirely evaluated, and therefore the final designation is deferred to the findings in the excisional specimen. (This slide was read by bone and soft tissue pathologist.)

There has been no excision of this tumor and, as a central registry, we have no access to the pathologist for clarification.

Is this skin case reportable based on the dermatopathology interpretation when further documentation is not available?

Example: Patient has multiple biopsies with final diagnosis of in situ papillary urothelial carcinoma in the prostatic urethra and invasive papillary urothelial carcinoma in the bladder.

How should primary site be coded in this type of mixed in situ and invasive situation?