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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?

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.

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

Instruction number 1 under the Coding Multiple Histologies instructions states to code histology when the histology is described as subtype, type or variant. The general rules do indicate we can code the histology identified as type, but when applying the H Rules, it seems an argument could be made for either H1 or H3. H1 applies if you ignore the diagnosis of carcinoma and only code the histologic type: urothelial carcinoma. However, the rules do seem to imply that you take all histologies into account (e.g., code the subtype/variant when there is a not otherwise specified (NOS) and single subtype/variant). Following this logic, Rule H3 seems to be the only rule that fits, and one would code the subtype/variant urothelial carcinoma when the diagnosis is carcinoma NOS, histologic type: urothelial carcinoma.

The problem is that urothelial carcinoma is not a subtype/variant of carcinoma (NOS) per Table 2. The entry for Carcinoma NOS in Table 2 states, Subtypes of carcinoma NOS include adenocarcinoma and all subtypes/variants of adenocarcinoma. To some, urothelial carcinoma is a more specific type of carcinoma; however, urothelial carcinoma is not also listed as a subtype of carcinoma or of adenocarcinoma; only adenocarcinoma is categorized as a subtype of carcinoma. Consistently applying the rules becomes an issue when rules are interpreted in different ways. Should this Table be amended to include urothelial carcinoma as a subtype/variant of carcinoma NOS with the same caveat given to adenocarcinoma in Table 2?

Pathology reads: 1. FNA left groin lymph node tissue (smears and cell block): B-cell lymphoma, low grade. The concurrent flow cytometry (3-FC-16-288) identifies a monoclonal B cell population with immunophenotype of CD10++, CD5-, CD23-, CD20++ and unusual CD19-. Overall findings favor follicular lymphoma. FNA Specimen Adequacy: Evaluation for specimen adequacy: Immediate cytology smear review for specimen adequacy was performed at the time of the FNA procedure by pathologist. Smears reviewed from 2 passes in one reading. The specimen was adequate cytological evaluation. Surg Path Final Report Special Studies Immunohistochemistry (CD45, MCK, CD20, CD3, CD10, Bcl6, MUM1 \T\ Ki67) was performed on block 1A to confirm the diagnosis. All controls show appropriate reaction. Lymphoma cells are positive for CD45, CD20, CD10 and weakly positive for bcl6(+) and MUM1(+/-), and negative for MCK. CD3 highlights few T lymphocytes. Ki67 labeling index is low, less than 10%. The immunoprofile supports above diagnosis. Chromosomal study for t(14;18) translocation will be performed, and an addendum report will follow. Flow Final Report Comment: The lymphoma appears to be derived from germinal centre B cells. Together with the findings from the lymph node biopsy (3-FN16-416), follicular lymphoma is favored. However, negative CD19 and CD22 are unusual.

For certain facilities in our area, the breast pathology reports using a CAP protocol format are formatted as follows; the Final Diagnosis will state Infiltrating carcinoma with the following features. The features list the specific tumor characteristics required in the CAP protocol formatting. The histology is always displayed in the list form and specified as Histologic type: (for example, Histologic type: Ductal carcinoma). Is this specific histology really to be ignored because it is preceded by the word type even if this is just a consequence of the pathology report formatting?

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?