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HL-7 e-path report, Final Diagnosis

High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment.

Comments

Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation.  Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization

History provided by the oncologist

Right neck mass since 2019; 04/07/20, initial biopsy p16-negative SCC, delay of treatment due to patient preference, agreed to biopsy of tonsil and work-up August 2022; right tonsil biopsy: p16-positive, G2 SCC, nodal mass at that time >6 cm with extensive extranodal extension, Stage III (cT2, cN3, cM0, p16-positive); based on this history, was staged as a tonsil primary and p16-positive.

Patient details

1. March 2020, CT neck and chest revealed a 0.5 x 2.7 x 2.3 cm low-density necrotic nodal mass at right neck level 2 suspicious for metastatic disease.  There was a slight asymmetric increased size of the right palatine tonsil.  There are a few sub-4 mm pulmonary nodules which are nonspecific.

2.  April 7, 2020, FNA of right neck mass with pathology revealed p16-negative SCC   

3.  April 20, 2020, PET/CT revealed 3 x 2 cm right-sided level 2 node with FDG avidity  

4.  May 5, 2020, flexible laryngoscopy showed no obvious primary lesion   

5.  May 2020, after evaluation by a medical oncology, patient declined any treatment  

6.  June 17, 2022, return visit in medical oncology after PET/CT demonstrates significant progression in the neck; patient definitively declines chemo, but would like surgical opinion.  Now has more rapidly progressive disease with skin breakdown and weeping from malignant lesion right neck.  

7.  June 22, 2022, radiation oncology consultation   

8.  July 15, 2022, tonsil biopsy: Invasive squamous cell carcinoma, moderately differentiated with LVI, p16-positive  

9.  Patient now agreeing to treatment with radiation: Tooth extractions 8/30/2022, radiation planning 9/14/2022  

10.  Patient consulted with cancer specialist who explained surgery is not recommended given level of extranodal extension and risk of seventh cranial nerve paralysis and fistula formation with surgical excision and who recommended chemoradiation

11.  September 9, 2022, patient presented for radiation CT simulation/treatment planning and informs treatment team. Patient declined/refuses concurrent chemotherapy despite recommendations from two cancer institutions.

Historically, multiple invasive ductal and lobular carcinomas diagnosed within 5 years were abstracted as a single primary. However, it is not clear if Rule M10 or M14 applies to this situation per the 2023 Solid Tumor Rules updates.

Rule M10 addresses multiple tumors of carcinoma of no special type (NST)/duct and lobular, but there is no timing criteria mentioned. Does M10 apply to cases diagnosed synchronously, or metachronously, or at least within 5 years? Should Rule M10 include a Note instructing registrars to accession a single primary for the scenario in question?

If timing matters for Rule M10, then the next rule that applies is M14. Rule M14 instructs one to abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 3, and carcinoma NST/duct and lobular carcinoma are on separate rows in Table 3.

Histology - Page 85 of the SPM 2004, Histology Type Coding Instructions, #2. Use the histology stated in the final diagnosis from the pathology report. Use the pathology from the procedure that resected the majority of the primary tumor.

Based on this rule, should the following case should be coded to Ductal Carcinoma (8500/31)?

Core needle bx: WD Infiltrating Ductal Carcinoma with focal lobular features. Lumpectomy: WD Invasive Ductal Carcinoma.

Histologic Type: HPV-associated multiphenotypic carcinoma. Overall, the morphology, immunohistochemistry, and HPV testing results support the diagnosis of an HPV-related multiphenotypic carcinoma. This entity has been described in the sinonasal region, where it behaves more indolently than its other salivary gland carcinoma counterparts (e.g., adenoid cystic carcinoma), with local recurrence but rare metastases.

Head and Neck Multiple Primary Rule M8 appears to be the first rule that applies to this case and instructs the user to abstract multiple primaries when separate/non-contiguous tumors are on different rows in the appropriate site table (Tables 1-9) in the Equivalent Terms and Definitions. Table 1 (tumors of the nasal cavity) shows Non-keratinizing squamous cell carcinoma and squamous cell carcinoma on different rows making the 2019 case a new primary. Is this correct?

Guidelines for selecting grade for breast primaries prioritize nuclear grade right after B&R grade. The conversion table displays only numeric values for nuclear grade. How is grade coded for tumors in which nuclear grade is described by terminology? Does it make a difference if the tumor is invasive or in situ?

Example 1: Ductal carcinoma, intermediate nuclear grade.

Example 2: Ductal carcinoma, high nuclear grade.

Example 3: Ductal carcinoma, moderate nuclear grade.

Example 4: DCIS, intermediate nuclear grade.

Glioblastoma multiforme is listed as a synonym for the preferred term glioblastoma, NOS (9440) per Table 3 Column 2. Therefore, it seems reasonable to assume that a diagnosis of glioblastoma, NOS would be a new primary if it followed a glial or astrocytic tumor. However, in general, the Solid Tumor Rules use the preferred terminology and/or indicate when a specific rule also includes any tumor diagnosed as a subtype/variant. Rule M6 does not explicitly include a diagnosis of glioblastoma, NOS or any of its subtypes/variants (e.g., glioblastoma IDH-mutant or gliosarcoma). Does Rule M6 apply to any diagnosis of glioblastoma, NOS and any of its synonyms or subtypes/variants?

From a number of definitions reviewed, it seems unclear if it's part of the appendix or the cecum of the colon. For example: The cecum is usually located in the right iliac fossa. In the pole of the cecum, there is often the appearance of fusion of the three teniae coli around the appendix, giving rise to the tri-radiate fold (Mercedes Benz sign), but the anatomy can be variable. The most reliable landmarks of the cecum are the appendiceal orifice and ileocecal valve. The appendiceal orifice is usually an unimpressive slit, often crescentic in shape. The ileocecal valve is made up of the superior and inferior lips (usually not seen en face) and is the gateway leading into the terminal ileum. It is located on the prominent ileocecal fold encircling the cecum, between 3 and 5 cm distal to the cecal pole. (https://www.sciencedirect.com/science/article/pii/S2212097113701730)

Frequently, pathology reports describe hematopoietic diseases using ambiguous terms. Flow cytology and cytogenetics may be obtained. It appears that the clinician is the person who pulls all the information together for a diagnosis.

Example: Bone marrow biopsy is most compatible with chronic phase myeloproliferative disease. Path comment: Differential would include CML. Outside hematology report indicates an elevated peripheral WBC, primarily neutrophils. Flow cytometry showed 1.0 % of the white cells are myeloid blasts of abnormal phenotype, additionally finding 7.3 % basophils. Flow reported peripheral blasts at 1.2 % and peripheral basophilia. Cytogenetics report showed abnormality with trisomy of chromosomes 13 and 21. This finding is consistent with a clonal abnormality suggestive of acquired disease. Results were consistent with the absence of the t(9,22)(q34;q11) translocation or fusion product associated with CML. Subsequent clinical impression: Bone marrow evaluation most consistent with CML. Overall features most consistent with CML.