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Patient is diagnosed July 2021 with high-grade serous carcinoma on ascites cytology. Tumor debulking total abdominal hysterectomy/bilateral salpingo-oophorectomy in August shows high-grade serous carcinoma involving the right ovary (capsule intact, right fallopian tube is negative), left ovary (capsule ruptured), and fallopian tube. Pathologist has chosen tumor site to be bilateral ovaries in the staging summary, with the left fallopian tube listed as “other tissue/organ involvement” along with uterus, peritoneum, and omentum. Additional findings in staging summary includes serous tubal intraepithelial carcinoma (STIC).

Our interpretation of SINQ 20210025 is that any case with both ovarian and tubal involvement would be coded as a fallopian tube primary if STIC is present, even when the pathologist is clearly calling the case ovarian. If this is correct, then the previous SINQ 20120093 may need to be updated with a date restriction reference since it would be in disagreement with this instruction.

If our interpretation is incorrect, then the STIC would be an additional primary per MP/H Rule M11.

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.

Details

4/15/2018:  Right abdominal wall mass excision: infiltrating sebaceous carcinoma.  Noted to have a history of Muir-Torre/Lynch syndrome.

1/21/2019:  Two left upper back mass excisions and two lower back (laterality not specified) mass excisions: infiltrating sebaceous carcinomas

8/7/2019:  Excision of multiple sebaceous carcinomas from the right posterior back, left posterior thigh, left anterior abdominal wall, left anterior thigh, right scrotum, right lower abdominal fold, all positive for sebaceous carcinoma on pathology report

9/30/2020:  Right gluteal mass, left gluteal mass, back (NOS) excisions: sebaceous carcinomas.  

10/14/2020:  Right back excision: sebaceous carcinoma. Op note:  History of Lynch syndrome with multiple sebaceous carcinomas, recurrent back mass, site of prior mass resection.

10/18/2021:  Right thigh excision: sebaceous carcinoma

Proposed primaries using MP/H Other Sites Rules

#1:       4/15/2018:  C445-1

#2:       1/21/2019:  C445-2, separate from #1 per M8, same as 1/21/19 C445-9 per M18

#3:       8/7/2019:  C445-1, separate from #1 per M10, separate from #2 per M8

#4:       8/7/2019:  C447-2, separate from #1 & #3 per M8, separate from #2 per M12

#5:       8/7/2019:  C632, separate from #1 per M10, separate from #2-#4 per M11

#6:       9/30/2020:  C445-2, separate from #1 & #3 per M8, separate from #2, #4 & #5 per M10

#7:       9/30/2020:  C445-1, separate from #2, #4 & #6 per M8, separate from #1, #3 & #5 per M10; I do not think the back, NOS (C445-9) is a new primary per M18.

#8:       10/18/2021:  C447-1, separate from #2, #4 & #6 per M8, separate from #1, #3, #5 & #7 per M10

In the September 2021 Urinary Sites Solid Tumor Rules update, the term configuration was removed from the “DO NOT CODE histology when described as” list. However, it was not added as a term that can be used to code the more specific histology for urinary tumors.

Can configuration be used to code the more specific histology 8130 (papillary urothelial carcinoma) when the diagnosis is urothelial carcinoma, tumor configuration: papillary?

Example: Patient has a left cerebral meningioma that is meningothelial meningioma (9531) and two right-sided cerebral meningiomas: one that is transitional meningioma (9537) and the other that is meningioma, transitional and angiomatous, WHO Grade I. If the histology for the mixed tumor is 9534 (angiomatous meningioma), then there are three primaries. If the histology is 9537 (transitional meningioma), then there are two primaries.

Per Table 6, angiomatous meningioma is 9534/0 and transitional meningioma is 9537/0. There is no mixed histology coding rule, or mixed histology meningioma code. There is also no default rule that would instruct registrars to code the numerically higher ICD-O code or to default to a meningioma (NOS) histology code.

A recent molecular study of PNET tumors at NCI (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5139621) seems to indicate the discovery of four new CNS tumor entities, of which HGNET-BCOR is one. The article suggests that these are not primitive neuroectodermal tumors tumors (PNET), but something different.

The patient has testicular cancer with bilateral lung metastases and possible liver metastasis. The left orchiectomy final diagnosis was The Summary describes a single tumor that is, Germ cell neoplasia in situ (GCNIS) is also present. Although there is mixed germ cell tumor present, the PNET component of the tumor is locally invasive extending into the epididymis, hilar soft tissues, spermatic cord, and tunica vaginalis. The mixed germ cell tumor is limited to the testis only.

We are instructed not to use to the term to code histology in the MP/H Rules General Instructions (Other Site Rules not updated for 2018), however the PNET comprises the majority of this tumor and represents the most extensive disease. Should the PNET histology be ignored in this case as its a ?

Rule M15

Abstract a single primary when synchronous, separate/non-contiguous tumors are on the same row in Table 2 in the Equivalent Terms and Definitions.

Note: The same row means the tumors are

• The same histology (same four-digit ICD-O code) OR

• One is the preferred term (column 1) and the other is a synonym for the preferred term (column 2) OR

• A NOS (column 1/column 2) and the other is a subtype/variant of that NOS (column 3) OR

• A NOS histology in column 3 with an indented subtype/variant

Example: TURBT shows invasive papillary urothelial carcinoma 8130/3 and CIS/in situ urothelial carcinoma 8120/2. Abstract a single primary. Papillary urothelial carcinoma and urothelial carcinoma are on the same row in Table 3.

In previous STR versions and the ICD-O-3.2, SFT/hemangiopericytoma, WHO G1 is 8815/0 and only SFT/hemangiopericytoma, WHO G2 was 8815/1. However, Table 6 (Non-Malignant CNS, Specific Histologies, NOS, and Subtypes/Variants) was changed in the 2025 updates to indicate both G1 and G2 SFT/hemangiopericytoma are 8815/1.

No date range was provided for this change in the STR and the behavior of this tumor was not updated by the standard setters in other references (i.e., ICD-O-3.2). The behavior of G1 SFT/hemangiopericytoma was not updated in the 2025 ICD-O-3.2 updates. If the ICD-O-3.2 was the source of this change, should this have been documented in the 2025 NAACCR Implementation Guidelines? However, the 2025 NAACCR Implementation Guidelines indicates, "There are no ICD-O-3 changes for 2025." Is this behavior change in 2025 Solid Tumor Rules updates an error? Should the behavior of SFT/hemangiopericytoma, WHO G1 remain /0?