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Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

Example: Brain MRI shows a mass along underside of right tentorium extending to posterior incisura consistent with meningioma. Spinal MRI shows mass at C4-5 level consistent with meningioma. Resection of spinal meningioma shows final diagnosis of meningioma and College of American Pathologists (CAP) protocol summary indicates Histologic Type (WHO classification of tumors of the central nervous system): Meningioma, meningothelial. There is no resection of the cerebral meningioma planned. Is the CAP protocol used if it provides a further subtype for meningiomas? Per Solid Tumor Rules, the final diagnosis has priority over the CAP summary. The answer to this question does affect the number of primaries accessioned in this case.

Pathology Diagnosis: Left temporal lesion - Low grade glial/glioneuronal neoplasm BRAF mutant. Pathologist Comment: The histopathological appearance of this lesion does not allow for a definitive diagnosis. However, the low-grade appearance, fibrillary nature, immunohistochemical profile, and the presence of a BRAF V600E mutation allow this to be categorized as a low-grade glial or possibly glioneuronal tumor. Despite the lack of exact classification this neoplasm can be expected to behave in a very indolent manner consistent with a WHO grade I classification.

IMPRESSION/PLAN: Patient is a previously healthy middle aged woman with a diagnosis of adenocarcinoma of the rectum, clinical stage II (T3N0M0). We will proceed with a neoadjuvant course of radiation and concurrent chemotherapy (5-FU) to maximize local regional control and survival, and hopefully facilitate a sphincter-sparing resection in the future. The primary tumor and the pelvic nodes at risk will receive 4500 cGy delivered over 25 treatments. The primary tumor will subsequently receive an additional 1080 cGy delivered over 5 treatments, for a cumulative dose of 580 cGy.

PATHOLOGY: Adenocarcinoma of the rectum, clinical stage II (T3N0M0). The patient is referred by (dr) for a neoadjuvant course of chemoradiotherapy.

HPI: Patient presented recently with rectal bleeding and a change in bowel habits. Colonoscopy revealed an ulcerated mass located 4.0 cm above the anal verge. A biopsy was positive for invasive well-differentiated adenocarcinoma that arose from a tubular adenoma. A staging work-up demonstrated no evidence of metastatic disease.

The patient was diagnosed in 2018 with met melanoma to the brain found via imaging. The staging procedure was a single axillary lymph node excision which was positive for metastatic melanoma. The exact site of the primary was never determined and the site code is C449. Is the axillary lymph node regional or distant? This affects how I code regional lymph nodes positive, regional lymph nodes examined, and scope of regional lymph node surgery or surgical procedure other site. Similar question was asked in the past (question # 20091101) but I have not found this question restated since the 2018 changes and just want to verify this is still what we are to do.

There is a discrepancy between Notes 3 and 6 in the schemas Fallopian Tube, Ovary, and Primary Peritoneal Carcinoma for EOD Primary Tumor. Note 3 describes extension/discontinuous metastasis to the pelvic sites (code 450) and includes the sigmoid colon, rectosigmoid and rectum since these are all pelvic sites. However, Note 6 also includes rectosigmoid and sigmoid colon.

Note 6 is describing extension/discontinuous metastasis to the abdominal sites (600-750), so it should include rectosigmoid or sigmoid colon (since those are pelvic sites). Note 6 indicates, Intestine, large (except rectum). In the previous Collaborative Stage, the corresponding note used to also include: except sigmoid colon, rectosigmoid and rectum.

Did sigmoid colon and rectosigmoid get removed from the list here? That is, should Note 6 read, Intestine, large (except sigmoid colon, rectosigmoid, rectum)? Involvement of the sigmoid, rectosigmoid, or rectum via peritoneal seeding/metastasis is consistent with T2b disease and would correlate with code 450 (pelvic sites), not codes 600-750 (abdominal sites). Those codes only correlate with T3 and greater disease (i.e., peritoneal seeding/metastasis of the abdomen).

Example: Left thyroid lobectomy with final diagnosis

When the only mention of encapsulation is included in the tumor characteristics of the College of American Pathologists (CAP) summary, not the pathologist's choice of histologic type, what is the preferred histology?

Rule M11 states: Abstract a single primary when there are urothelial carcinomas in multiple urinary organs, but neither the Rule nor the Notes describe the timing of these multiple urinary organ carcinomas. Timing requirements for other rules are clearly stated.

Does Rule M11 have a timing requirement or is it intended to apply to all urothelial carcinoma tumors regardless of timing (and not already qualifying for application of a previous M rule)?