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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?

SINQ 20170043 is a similar question about an ovarian mucinous borderline tumor with microinvasion, but the answer seems to be specifically referencing mucinous tumors only. It is unclear if that SINQ could be applied to this case. In addition, we were not sure how to interpret the nodal involvement. The physician assessment after surgery was low grade serous carcinoma, chemo not recommended and letrozole started.

Patient presented with hoarseness and palpable neck mass. No palpable adenopathy (per hospital abstract).

02/19/16 Thyroid Ultrasound: Right thyroid lobe with mass, 63X35X44XMM (per hospital abstract).

06/01/16 Right thyroid lobectomy, radical resection right laryngeal tumor (per hospital abstract).

06/01/16 Operative Procedure: Tumor was invading laryngeal soft tissue and cartilage anteriorly and to the right. There may be a small amount of residual tumor invading cartilage although this was not clear (per hospital abstract).

GROSS DESCRIPTION: 1. The specimen is received fresh for intraoperative consultation, labeled with the patient's name and "right thyroid mass." It consists of a 3.0 x 2.2 x 2.0 cm irregular, ragged fragment of tan-red, firm, rubbery soft tissue. The specimen is serially sectioned to reveal a tan-red, gritty cut surface with focal fleshy areas. A touch prep is performed. A representative section is submitted for frozen section analysis in 1FSA. A portion of tissue is submitted for flow cytometry with the accession number MSO-16-1786. The remaining specimen is entirely submitted in 4 additional cassettes (1B-1E). 2. The specimen is received in formalin and is labeled "right thyroid lobe." It consists of a thyroid lobe measuring 4.3 x 4.0 x 1.3 cm and weighing 10.0 g. The external surface is covered by a thin fibrous capsule with a focal area of roughening on the posterior surface. The lobe is inked black posterior, blue anterior and orange isthmus margin. Serial sectioning reveals a red-brown and beefy parenchyma. A definitive nodule is not grossly identified. The entire specimen is serially submitted from superior to inferior in 9 cassettes. 3. The specimen is received in formalin, labeled with the patient's name and "right neck/laryngeal mass." It consists of an irregular, focally nodular red-tan mass measuring 7.0 x 5.5 x 4.0 cm and weighing 54 g. The convex portion of the specimen is mostly encapsulated with focal adherent red-brown striated skeletal muscle. The concave portion of the specimen is focally ragged and disrupted. The convex portion of the specimen is inked black and the concave portion is inked blue. The specimen is serially sectioned to reveal a white-grey to red, granular, gritty cut surface with focal fleshy areas. Representative sections are submitted in 12 cassettes.

Final DX DIAGNOSIS: 1. Right thyroid mass excision Plasma cell tumor /plasmacytoma 3 cm. Tumor cells are positive for kappa and negative for lambda immunostains. Recommend correlation with flow cytometry MSO-16-1786, monoclonal plasma cell population with cytoplasmic kappa positivity. Ki-67 stains 7 percent of cells. Focal stromal hyalinization. Congo red stain for amyloid negative. No thyroidal tissue identified. 2. Right thyroid lobe excision Benign thyroid tissue with focal solid cell nest negative for malignancy. One out of two 1/2 perithyroidal lymph nodes positive for plasma cell tumor. 3. Laryngeal mass excision Plasma cell tumor /plasmacytoma 7 cm involving soft tissue and skeletal muscle. Tumor cells are positive for kappa and negative for lambda immunostains. Ki-67 stains 7 percent of cells. Focal stromal hyalinization and calcification. Congo red stain for amyloid negative

March 2017 lung biopsy showing metastatic melanoma. Subsequent workup shows imaging with additional metastatic involvement of multiple bone sites but no primary tumor is identified. Chemotherapy is started in May 2017.

July 2017 biopsy of right lower quadrant mass has a final diagnosis of ganglioneuroblastoma and pathologist's comment states I believe that this may represent transdifferentiation of metastatic melanoma. Later, partial colectomy of transverse colon Gross Description indicates this was centered in the mesentery.

The patient has a history of prostate adenocarcinoma with lymph node metastases, status post prostatectomy and treatment by Lupron in 2014. The most recent prostate serum antigen measurement (April 2018) was normal. CT scan of the abdomen and pelvis revealed new hypodense liver lesions, a slightly enlarging lung right lower lobe nodule, and enlarging lobular mass in the prostatectomy bed. The core liver biopsy contains areas of metastatic tumor with a differential diagnosis on pathology of high-grade neuroendocrine carcinoma of the prostate (small cell type), which may have been seen in association with prostate adenocarcinoma, or metastatic small cell carcinoma of a different site.

Clinically, the physician impression is that this represents metastatic castration-resistant prostate cancer. The Solid Tumor Rules note that the Multiple Primary Rules are not used for tumor(s) described as metastases. However, SINQ 20130221 indicates that, at least historically, these would have been accessioned as multiple primaries (histology 8140 & 8041 per Rule M10). Does the previous SINQ note still apply to these types of cases, and if so how would one know to move beyond the initial note indicating metastases are not new primaries?

It appears an argument could be made for both NLPHL (9659/3) and THRLBCL (9688/3). We favor coding NLPHL (9659/3) because the pathologist did specifically call this a Hodgkin lymphoma, and also specified that it only has a T-cell/histiocyte-rich large B-cell lymphoma-like pattern.