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"Resection, periampullary duodenum: Gangliocytic paraganglioma, with metastasis to one large periduodunal lymph node. Six other small lymph nodes negative. COMMENT: The primary tumor in the duodenum is made up mainly endocrine cell component. This component appears to have metastasized to a periduodenal lymph node."

The final diagnosis for a soft tissue excision is, "atypical lipomatous tumor/well-differentiated liposarcoma". The Comment section states, "Atypical lipomatous tumor/well differentiated liposarcoma has a significant risk for local recurrence, but no metastatic potential."

Per the 2010 SEER Manual, page 3, example 4: The pathologist makes the final decision about the behavior for a particular case. In this case, the pathologist uses both a reportable and a non-reportable term in the final diagnosis and in the comment section of the pathology report. Does the pathologist's comment impact the behavior and reportability of this tumor?

The managing physician has staged this pathologically as T4 N0 M0 squamous cell carcinoma of the ethmoid sinuses. The final pathology report says " Sinus, sphenoid, resection: papillary neoplasm most consistent with inverted papilloma with squamous cell carcinoma in situ, 7 cm in greatest extent, focus of probable superficial invasion (see comment).

Soft tissue, skull base, excision: involved by papillary neoplasm with squamous cell carcinoma in situ (see comment). Brain, extradural, intercranial biopsy: involved by papilloma with squamous cell carcinoma in situ. COMMENT:

This is a predominantly exophytic neoplasm with infolding of the tumor epithelium and in situ extension into submucosal glands. There are only focal areas suspicious for invasive squamous cell carcinoma, with probable invasion (<2mm) in one section....The histologic features are most consistent with an inverted papilloma with carcinoma in situ." When asked to comfirm if the diagnosis were in situ or superficially invasive, the pathologist responded "Squamous cell carcinoma in situ involving a papilloma. Locally aggressive but not technically invasive."

Rubin's Clinical Oncology states: Both the catecholamines and their metabolites are used as markers for neuroblastoma, with vanillylmandelic acid (VMA) and homovanillic acid (HVA) being the most commonly used. While their absolute values are not of prognostic significance, a higher VMA:HVA ratio suggests a better prognosis for patients with disseminated disease.

The 2018 Solid Tumor (ST) Rules General Rules state: For those sites/histologies which have recognized biomarkers, the biomarkers frequently identify the histologic type. Currently there are clinical trials being conducted to determine whether these biomarkers can be used to identify multiple primaries. Follow the Multiple Primary Rules; do not code multiple primaries based on biomarkers.

Additionally, Biomarkers is at the top of the priority order to identify histology in several sections (it appears to be excluded from only Colon, Melanoma and Other sections). In the sections that include this rule, there is not much additional information on using biomarkers. Can you please provide further explanation for prioritizing biomarkers in the histology coding rules? For example, will the ST manual be updated when we need to look for specific biomarkers in a diagnosis?

The case was reviewed by AFIP and called "extratesticular." Per our pathology consultant, the site should be coded to unknown because it is very doubtful that the tumor was primary in the soft tissue of the thigh. According to him, such tumors don't originate exclusively in the testes, but tend to occur along the central axis such as the mediastinum or retroperitoneum. If an extratesticular tumor arises in either of these areas, the primary site should be code to the mediastinum or the peritoneum rather than to unknown. Lesions primary in the testicle may also undergo maturation with fibrosis and involution. This process often leaves little evidence of the original tumor in the testis.

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?