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This patient has massive splenomegaly. The biopsy final diagnosis was "low grade B lymphoma of unclear subtype (splenic marginal zone lymphoma?)." The pathologist's comment states, "Because of the clinical context (lymphocytosis and splenomegaly) a splenic marginal zone lymphoma is a possibility." There are no other histologic diagnoses. All the flow cytometry reports are as unclear as the biopsy.

The hematologist, after seeing the pathology report, states, "The bone marrow biopsy shows a significant infiltration by mature lymphocytes; their markers strongly suggest a marginal zone lymphoma, probably of splenic origin The final diagnosis is a splenic marginal zone lymphoma."

Should the clinical diagnosis of splenic marginal zone lymphoma [9689/3] be coded when a clinical diagnosis is not listed as a definitive diagnostic method for this neoplasm? Or should the histology be coded as low grade B-cell lymphoma [9591/3]? The clinicians will expect the case to be coded as a splenic marginal zone lymphoma when there's no doubt about the diagnosis.

1.  The 2022 and 2023 SEER Program Coding Manuals state under Histologic Type ICD-O-3: Beginning with cases diagnosed 01/01/2022 forward, p16 test results can be used to code squamous cell carcinoma, human papilloma virus (HPV) positive (8085) and squamous cell carcinoma, HPV negative (8086).

NAACCR 2023 Implementation Guidelines contain similar instructions on HPV histologies for cervix, vulva and vagina that are applicable back to 2022 (2021 for cervix).

The current Other Sites Solid Tumor Rules state on the Histology tables for anus, cervix, vagina, vulva, and penis and scrotum: "p16 is a valid test to determine HPV status and can be used to code HPV associated and HPV independent histologies." Since Other Sites Solid Tumor Rules apply to cases diagnosed 2023+, can p16 results only be used from 2023 onward, to code HPV-related histologies for primaries that fall under the Other Sites module? Or per the 2022 SEER Manual statement and NAACCR 2023 Implementation Guidelines, could a p16-confirmed HPV histology code also apply to a 2022 Other Sites case and if so, is that only for cervix, vulva, and vagina? Further complicating the matter are the 2024 ICD-O-3.2 update documents indicating these codes are valid 1/1/2024+ for the “Other Sites” penis and scrotum.

2.  Is using p16 testing for HPV-related histology codes ONLY allowed for sites in the Solid Tumor tables that contain the statements about p16 (Head & Neck Table 5, and the Other Sites tables noted above for anus, cervix, etc.)? Or could it apply to primary sites outside of those tables; for example, a 2022 pathology report from the ethmoid sinus C311 indicating an HPV-related histology based on p16 testing? The ICD-O-3 Annotated Histology lists include C310-C313 among the common site codes for 8085 and 8086. The Head and Neck Solid Tumor Rules “New for 2022” section and rule H1 Note 4 also mention that p16 can be used to code HPV histologies; these sections would seem to apply to all sites in that module, since only the more common histology codes are listed in the tables and if not, we are instructed to use ICD-O.

Appendix E1 of the 2018 SEER manual references a similar diagnosis as being reportable for vulva and vagina only. However, the WHO Classification of Tumors of the Urinary System and Male Genital Organs (4th ed) does include high grade penile intraepithelial neoplasia as a synonym for 8077/2.

Left frontal mass biopsy diagnosis comment states: Given the synaptophysin and patchy CD34 staining of these cells, the possibility of ganglioglioma and pleomorphic xanthoastrocytoma is raised. Astroblastoma and ependymoma were considered given the perivascular pseudorosettes, however GFAP staining is quite limited against these tumors. Reticulin stain shows limited perivascular reticulin staining however. Nevertheless, the necrosis, mitotic activity and elevated mitotic activity would point to a malignant neoplasm. Given the neural and limited GFAP staining, a generic classification of neuroglial is provided.

This is the only available information. Further clarification or discussion with the physician or pathologist is not possible. Therefore, is this diagnosis of neuroglial tumor equivalent to that described in SINQ 20091037?

Per path comment: "The neoplasm is composed of adenosquamous carcinoma which merges with spindle cell carcinoma. The cystic component shows a mixed squamous and ductal epithelial lining which shows cytologic atypia and mitotic activity and can be seen to merge with invasive carcinoma. The features suggest the possibility that the tumor may have arisen from a sclerosing and cystic papilloma with squamous metaplasia, although a clearly benign component is not evident."

Would MP/H rule H19 apply based on the pathology report comment resulting in histology for the case being coded to 8255 [adenocarcinoma with mixed subtypes]? Or, would MP/H rule H14 apply based on the final diagnosis resulting in histology for the case being coded to 8575 [metaplastic carcinoma] because adenosquamous and spindle cell are not specific types of metaplastic carcinoma?