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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.

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

Patient has a resection labeled as: Spine, designated intramedullary lesion. The Final Diagnosis is: Melanocytic neoplasm with features most consistent with primary intramedullary melanocytoma. The Diagnosis Comment states: The overall immunophenotypic and morphologic impression is a primary central nervous system melanocytoma.

The ICD-O-3 lists melanocytoma, NOS histology code as 8726/0, but does not provide a site-associated code. If the ICD-O-3 is used, the histology would be 8726/0 and the primary site presumably would be C720 since the tumor was specifically described as being intramedullary (i.e., within the spinal cord medulla).

Table 6 (Solid Tumor Rules, Non-Malignant CNS Equivalent Terms and Definitions) does not list either an intramedullary melanocytoma or melanocytoma (NOS). However, Table 6 does include meningeal melanocytosis 8728/0 and meningeal melanocytoma 8728/1. If Table 6 is used and the histology is coded 8728/1, then the primary site would presumably be C701 per the ICD-O-3 site-associated listing for this histology (C709).

Table 4 (Changes in reportable terminology), 2021 ICD-O-3.2 Update, does confirm that the term malignant no longer needs to be used to describe a paraganglioma, but Table 4 includes the histology for extra-adrenal paraganglioma, NOS (8693/3) as the new preferred term for paraganglioma. Paraganglioma, NOS is histology code 8680/3. Which code is correct?

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

The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019.

In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma.

Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma.

Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process.

Example: 3/22/2017-26 year old white female seen in the emergency room with abdominal pain. Patient was diagnosed about a month ago with breast cancer. Impression: menstrual pain. In this example the patient is newly diagnosed with breast cancer, but the second hospital does not treat or diagnose the patient; pain management for a separate condition is received only. Is this patient reported due to the history of active disease?

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.