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While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755.

Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755.

There is a discrepancy in the SEER and STORE manual definition of code 2 for Reason for No Surgery of Primary Site. STORE includes progression of tumor prior to planned surgery as part of the definition for code 2, but the SEER Manual does not. The progression statement is included in the SEER Manual (2018 and 2021) for Reason for No Radiation, but not for Reason for No Surgery.

Cervix, loop electrosurgical excision procedure: Cervix at transformation zone with stratified mucin-producing intraepithelial lesion (SMILE). SMILE is present at the ectocervical margin. An immunohistochemical stain* for p16 demonstrates strong, diffuse positivity in the lesional epithelium. A mucicarmine stain is also positive in the lesional epithelium, supporting the diagnosis of SMILE.

Patient was diagnosed with a retroperitoneal paraganglioma on April 2021 mass resection. Final diagnosis included the comment: Based on the modified grading of adrenal pheochromocytoma and paraganglioma (GAPP), the GAPP score is 1. Scores greater than or equal to 3 are malignant.

We are aware that paraganglioma is classified as malignant for cases diagnosed in 2021+, however it is unclear how the pathologist's interpretation of the GAPP score may affect the behavior of this case.

We are confused by SINQs 20180097, 20150001, and 20140051.  The latter two indicate that “well-differentiated neuroendocrine neoplasms” of the duodenum and appendix are reportable because they’re synonymous with neuroendocrine tumor (NET).  Yet 20180097 states “primary hepatic neuroendocrine neoplasm” is NOT reportable unless there is documentation that it’s being used as a synonym for Primary Hepatic Neuroendocrine Tumor (PHNET).  In addition, we see in the 2021 ICDO-3.2 update that only “poorly differentiated neuroendocrine neoplasm” is listed with behavior code /3 and noted to be reportable for 2021+ on the companion annotated histology list.  Does reportability of neuroendocrine neoplasms depend on primary site, differentiation terminology within the histology name, or something else?  Our casefinding staff is hoping for a general reportability guideline to follow when they come across “neuroendocrine neoplasms” NOS.  For example, we have a 2020 pathology report for a core biopsy of a soft tissue pelvic mass with final diagnosis of low grade neuroendocrine neoplasm; there is no further clarification as to whether it is felt to be primary or metastatic, and we have no other associated records for this patient in our central registry.

Since AIN I is not reportable and AIN II is not reportable until 2021, we are not sure if we can say the patient was disease free because there was no intervening reportable tumor (AIN III), or was never disease free because there was evidence of related disease (lower grade dysplasia).

Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN).

Currently, First Course Therapy instruction for expectant management (also referred to as active surveillance, watchful waiting, etc.) instructs one to code 0 or 00 (not done) for all data items when the physician opts for expectant management.

We find that the treatment decisions can be driven by the patient, physician, or combination of both patient and physician depending on the options presented.

The physician stated the prior LUL adenosquamous carcinoma was PD-L1 negative and the LUL squamous cell carcinoma is PD-L1 positive and is calling it a new primary.

5/22-7/3/19 6000x30 IMRT Photons LUL lung Chemo refused Not a Surg candidate

10/01/2019 CT Chest: IMP: In comparison to CT chest 03/06/2019 and PET/CT 03/21/2019, left lingular mass has mildly decreased in size. Left apical anterior and posterior lung lesions more anterior lesion appears slightly increased in size, the other slight decreased in size, with adjacent areas of atelectasis and scarring.

06/23/2020 CT Chest: MP: In comparison to CT chest 10/1/2019, left lingular mass has increased in size concerning for increasing tumor with adjacent thicker focal pleural thickening involving the chest wall, concerning for possible chest wall invasion. Left apical anterior and posterior lung lesions appears more solid in appearance, representing known adeno CA, given that the appearance has changed, is concerning for residual tumor.

07/06/2020 PET: Hypermetabolic lingular mass and peripheral nodularity has increased in size and FDG avidity on the prior PET/CT. Left apical nodular opacity is difficult to separate from fairly uniform mild left apical pleural hypermetabolism which may be treatment related and/or neoplastic.

Autopsy/Pathology Report (2020) excerpts

External Examination

Nervous System: There is an 8.5 cm mass located in the right thoracic paraspinal area.

Final Anatomic Diagnosis

Clinical History: Paraspinal mass suspicious for neuroblastic tumor (detected by imaging studies)

Nervous System: Right thoracic paraspinal neuroblastoma, poorly differentiated