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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?

The 2022 ICD-O-3.2 Implementation Guidelines indicate “Tubulovillous adenoma, high grade” is 8263/2 and is not SEER reportable. However, the 2024 SEER Manual and clarification from recent SINQs (20240021 and 20240025) confirm high grade dysplasia in the esophagus, stomach, and small intestine is reportable (8148/2).

Which reportability reference applies to a diagnosis of a tubulovillous adenoma with high grade dysplasia in non-colorectal sites?

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?

We have had multiple users unable to find the applicable histology in the ICD-O-3.2 (i.e., the site-specific table did not include the histology) because they were using the annotated histology list and could not find the complete list of related terms or synonyms for the histology code.

For example, the ICD-O-3.2 lists Medulloblastoma, SHH-activated, NOS as a related term for 9471/3, but many users were unable to find this valid histology because they were using the annotated histology list, not the ICD-O-3.2.

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?

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?

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).

The patient had a 2023 endometrial polypectomy and curettage with final diagnosis of “at least serous intraepithelial neoplasia arising in association with an endometrial polyp.” Diagnosis comment states, “There are multiple tissue fragments with highly atypical glandular lining consistent with a high-grade serous neoplasm. There are focal areas which are suspicious, but not conclusive, for stromal invasion.” Subsequent hysterectomy and BSO showed no residual carcinoma.

According to previous SINQ 20210043, serous tubal intraepithelial neoplasm (STIN) is reportable when stated to be high grade. Does the same logic apply to a similar neoplasm in the endometrium and/or endometrial polyp?