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Scenario: Patient had a transurethral resection of the prostate on 8-29-19, positive for basal cell carcinoma with focal squamous differentiation involving approximately 50% of tissue (determined not to be mets by consult). On 11-14-19, the patient had a prostatectomy positive for residual basal cell carcinoma and a small focus of infiltrating prostatic adenocarcinoma. According to AJCC, 8th edition, page 724, basal cell carcinoma of the prostate is 8147/3 and we ignored the small focus of adenocarcinoma. The above scenario was reported as two primaries (8090/3 and 8140/3), but we are thinking it is one.

In 2018, patient has lymph node metastasis confined to left retroperitoneal area; core biopsy was done which showed metastatic adenocarcinoma, unknown primary site. There are no other sites of disease found.  Should I code Mets at Diagnosis--Distant Lymph Node(s) as 1, and the others such as bone and lung as 0?

Unless there is a biopsy that proves in situ tumor (EOD code 000, Tis) or extratesticular invasion into the scrotum, penis, or further contiguous extension (EOD code 700, T4), EOD Primary Tumor must be coded based on the PATHOLOGICAL assessment (orchiectomy). There are no other CLINICAL codes because the AJCC indicates imaging is not used for local T-categorization, and the EOD derives the AJCC TNM staging. If the case can not be coded to either EOD Primary Tumor codes 000 or 700 clinically, the only clinical code that seems to apply is 999 (Unknown).

We are seeing more cases treated with neoadjuvant chemotherapy prior to orchiectomy, especially in patients with distant metastatic disease. The EOD Manual indicates that clinical evidence takes priority over pathological evidencewhen neoadjuvant treatment is given, unless the extent of disease following neoadjuvant treatment is greater than pre-treatment clinical findings. If the clinical and pathological information are the same, code the extension based on the clinical information.

Do these general rules also apply to testis even though we cannot code CLINICAL findings for these tumors? If so, will EOD Primary Tumor be coded to 999 (Unknown) for any testis primary that is not in situ or invasive into the scrotum, etc., that is treated with neoadjuvant therapy? Or should the post-neoadjuvant PATHOLOGICAL assessment be coded for these tumors because the CLINICAL assessment would otherwise be unknown?

How is the EOD Primary Tumor coded for the following two cases?

1. Left testicular mixed germ cell tumor, biopsy-proven metastasis to a supraclavicular lymph node. The left testis contained a small mass on scrotal ultrasound. The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved no residual primary tumor (ypT0). Is EOD Primary Tumor 999 because it is clinically unknown (even though it was clinically limited) or 800 (No evidence of primary tumor) because there was no pathological evidence of tumor following neoadjuvant treatment?

2. Right testicular mixed germ cell tumor with biopsy-proven inguinal lymph node metastasis. There was a palpable mass in right testis on physical exam (not described as fixed or involving scrotum). The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved a residual 2 cm tumor limited to the testis without lymphovascular invasion (LVI). Is EOD Primary Tumor 999 because it is clinicallyunknown or 200 (PATHOLOGICAL assessment only - Limited to testis WITHOUT LVI)?

1. 12/11/2020 Peripheral blood revealing what was thought to be chronic myelogenous leukemia BCR/ABL1 positive (9875/3). Patient was started on Hydrea while waiting for further tests on 12/12/2020.

2. 12/14/2020 Lymph node biopsy showed granulocytic sarcoma (9930/3), but flow cytometry states it is similar to that seen in the patient's peripheral blood and is consistent with nodal involvement by myeloblasts.

3. 12/15/2020 Bone marrow biopsy reads acute myeloid leukemia (9861/3), likely arising from BCR/ABL1 positive chronic myeloid leukemia. There is a note on this pathology from medical oncologist that says: This will dramatically change the course of his treatment, likely with a TKI.

4. 12/17/2020 Sprycel started. Patient was weaned off Hydrea.

According to Rule M3, abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. It lists 9930/3 when simultaneously (or after) with 9861/3. Technically, it was two days before, but I feel like I should and could count that as simultaneously because of Note 1 that says: These sarcomas are solid manifestations of the associated leukemia. For example, when acute myeloid leukemia and myeloid sarcoma are diagnosed simultaneously, the myeloid sarcoma is the result of myeloid cells migrating from the bone marrow or blood into tissue. It is part of the disease process for the acute leukemia. Also, the providers never mention granulocytic sarcoma

Based on that, I think that #2 & #3 above are the same primary, which would be acute myeloid leukemia (9861/3).

Per the hematopoietic database, 9875/3 transforms to 9861/3. Therefore, Rule M8 is confusing with the "only one" biopsy. Does this rule apply because the 9875/3 was from peripheral blood only? But peripheral blood is coded in Diagnostic Confirmation as histology.

Rule M9 reads: The two diagnoses are likely the result of an ongoing diagnostic work-up. The later diagnosis is usually based on all of the test results and correlated with any clinical information. Because that is truly what I think is happening here though that rule states there is no available documentation. If you do not have any documentation, how would you know you are dealing with a chronic and an acute diagnosis?

M10 does not apply.

According to Rule M11, abstract as multiple primaries when both a chronic and an acute neoplasm are diagnosed simultaneously or within 21 days and there is documentation of two biopsies. The chronic myelogenous leukemia only had peripheral blood and not a bone marrow, lymph node or tissue, but that is counted as positive histology in diagnostic confirmation, but I don't know if that is kept as a separate field/thought. I would not code a peripheral blood smear as with a surgical code or a surgical diagnostic and staging procedure code, so maybe that is what I should be thinking about and therefore would probably say Rule M8 and one primary.

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.

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

Since the synoptic report and final diagnosis are equal in priority, and the Solid Tumor Rules tell us to code the more specific histology, would this be coded to signet ring cell adenocarcinoma, 8490/3, even though the pathologist used features in the final diagnosis? There is no histology adenocarcinoma with signet ring cell features on the CAP Protocol, so the pathologist may check off the next closest histology " signet ring cell carcinoma " which would not be truly representative of the actual histology. Final Diagnosis: Proximal colon, segmental resection: Invasive adenocarcinoma, poorly differentiated, with signet ring cell features. Synoptic Report A: Colon and Rectum - Resection Specimen Procedure: Right hemicolectomy, Tumor Site: Right (ascending) colon, Histologic Type: Signet-ring cell carcinoma, Histologic Grade: G3: Poorly differentiated.

Rule H6 states: Code 8742/3 (Lentigo maligna melanoma) when the diagnosis is lentigo maligna melanoma with no other histologic types. However, if the diagnosis was strictly lentigo maligna or lentigo maligna melanoma, the first rule that applies is Rule H1 because lentigo maligna melanoma is a single, specific histologic type and Rule H1 states, Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H6. Should the H Rules be reordered? Or should an example of when one would use Rule H6 be added to clarify when to use this rule?