SEER Inquiry System - Search

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 right breast ultrasound shows a 1.4 cm mass at 8 o'clock and a separate mass .6 cm at 7 o'clock (site code for both C50.5). Pathology report: Right 8 o'clock core needle biopsy fragments of intracystic noninvasive papillary carcinoma (8504/2), right 7 o'clock core needle biopsy fragments of intracystic noninvasive papillary carcinoma (8504/2). Then, another facility performs a right breast lumpectomy (operative note not available). Outside Facility: Right breast lumpectomy pathology shows invasive ductal carcinoma .6cm (8500/3) multifocal DCIS .5cm greatest dimension tumor site right breast NOS.

Should we use Rule M12-Abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant. Note: Each row in the table is a distinctly different histology. So would this be two primaries C50.5 (8504/2) and C50.9 (8500/3)?

Example: Initial CT scan impression is large appendiceal mucocele with a moderate amount of right-sided abdominal ascites. Faint mural enhancement suggesting an underlying appendiceal neoplasm (mucinous adenoma or adenocarcinoma).

Appendectomy follows two days later with final diagnosis of high-grade appendiceal mucinous neoplasm, see comment. Histologic grade: Grade G2 of 4 (based on the CAP protocol) . . . Ascites fluid (ThinPrep(r) and cell block preparations): Mucin, fragments of debris, and macrophages. No diagnostic neoplastic cells are identified . . . Pathologic stage: pT4a, pNX, pM1a (AJCC 8th ed).

Diagnosis Comment states, We feel that there are areas of this tumor where the cytologic atypia is beyond what one would expect in low-grade appendiceal mucinous neoplasm. While mitotic figures are not strikingly increased, there are focal nuclear changes that would support classification of this tumor as high-grade appendiceal mucinous neoplasm.

Approximately two weeks later the patient has an Oncology assessment stating new diagnosis of T4a, NX, M1a, Stage IVA high-grade mucinous adenocarcinoma of the appendix with mucinous ascites. Patient has had an appendectomy but no further surgery so far. However, anecdotally, the best reported case series has been with surgical debulking followed by HIPEC chemotherapy In that instance I have recommended surgery with intraperitoneal chemotherapy.

Is this a reportable malignancy? If so, what is the best histology for the diagnosis?

Cytology is not listed in the Priority Order for Using Documentation to Identify Histology (Histology Coding Rules) in the Head and Neck schema. Other schemas do include cytology in the hierarchy below tissue from a biopsy or resection. Cytology is often less specific than histology, so one would expect cytology to be listed below tissue in this hierarchy. Was this an oversight? Or would cytology be equivalent to histology if it provided the most specific histology for the case?

Since high grade squamous intraepithelial lesion (HGSIL) is reportable for the vulva in 2018 (per SINQ 20130185) but VIN II-III is not reportable, we need to clarify this reporting format seen in our area.

Example 1: 8/23/2017 debulking path diagnosis of low-grade appendiceal mucinous neoplasm (LAMN) with involvement of intrapelvic mucin, left ovarian mass, uterine serosa and pelvic tumor, consistent with disseminated peritoneal adenomucinosis, that may also be called low-grade mucinous carcinoma peritonei. 8/8/2018 resection of sigmoid and rectum, path diagnosis of peri-colorectal fibroadipose issue with low-grade mucinous carcinoma compatible with the prior diagnosis of pseumomyxoma peritonei with low-grade mucinous carcinoma histology.

Example 2: Path diagnosis of low-grade appendiceal mucinous neoplasm in association with low grade mucinous carcinoma peritonei involving the serosa of the small intestine and mesentery. Also, there is involvement of serosal lined soft tissue of peritoneum, omentum, stomach, falciform ligament, gallbladder, diaphragm and spleen.

Some pathologists in our area are referring to DPAM as mucinous carcinoma peritonei, which is causing confusion because the term carcinoma is being used. One would assume that because the pseudomyxoma peritonei/underlying tumor itself is low-grade (LAMN), then the case is not reportable, but we would like clarification.

The hospital registrar reports that the case was identified at the hospital because of the referral for chemotherapy for the metastatic GIST. The records from the out-of-state hospital mentioned a history of a small bowel resection in 2003 for a borderline tumor. The registrar went back through the hospital's old records and found the surgery was done for GIST of low malignant potential at her facility. The question is whether to report the case or not, and if reported, is 2003 the diagnosis date.

The rules say to change the behavior and backdate the diagnosiswhen a tumor is presumed benign and islater diagnosed as malignant. Another problem for this case is that the out-of-state hospital did not review the slides from the 2003 surgery.