Hyalinizing Clear Cell Carcinoma (HCCC) of the Head and Neck: A Multicenter Retrospective Study of 87 Cases Focusing on Prognostic Pathologic Features and Grading scheme.
Bin Xu, Kartik Viswanathan, Marie Barbesier, Alexander Ladenheim, Cristina R Antonescu, Ronald Ghossein, Dan Lubin, Kelly Magliocca, Sayed Matar, Michael W Mikula, Isabella Tondi Resta, Dibisha Roy, Soo Yeon Sohn, Anuj Verma, Manju L Prasad, Lisa Rooper, Margaret Brandwein-Weber, Zubair Baloch, Nora Katabi
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引用次数: 0
Abstract
Hyalinizing clear cell carcinoma (HCCC) is a salivary gland carcinoma characterized by the presence of clear and eosinophilic cells within a hyalinized stroma and the EWSR1 rearrangement. Aiming to identify prognostic factors and establish a grading system, we herein conducted a detailed clinicopathology review of a large retrospective cohort of 87 HCCCs from seven tertiary centers. Most HCCCs (91%) originated from minor salivary glands, although major salivary glands were affected in 8%. The most common sites were base of tongue, palate, nasopharynx, and maxilla . Eosinophilic cells were more prevalent than clear cells. Histologic features included Intraosseous component (19%), perineural invasion (48%), lymphovascular invasion (LVI, 16%), nuclear pleomorphism (14%), tumor necrosis (26%), and a mitotic index (MI) ≥5/2 mm2, 9%). Factors associated with increased risk of nodal metastasis at presentation included LVI, high MI, and tumor necrosis. The 10-year disease-specific survival (DSS), disease-free survival (DFS), and distant metastasis-free survival (DMFS) were 80%, 51%, and 87%, respectively. Significant prognostic factors identified on univariate survival analysis included MI≥5/2 mm2, tumor necrosis, atypical mitosis, and nuclear pleomorphism for DSS; LVI, MI≥5/2 mm2, and percentage of clear cells for DFS; and nodal metastasis, LVI, MI≥5/2 mm2, tumor necrosis, and atypical mitosis for DMFS. The only independent prognostic factor for DFS identified on multivariate survival analysis was MI≥5/2 mm2. High grade HCCCs, defined as tumors with MI≥5/2 mm2 and/or tumor necrosis, were associated with increased risk of nodal metastasis at presentation and shortened DSS and DMFS. Among 67 HCCCs examined for EWSR1 rearrangement, 65 (97%) harbored EWSR1 translocation. In conclusion, we identified multiple prognostic factors in HCCC, including MI, necrosis, atypical mitosis, nuclear pleomorphism, lymphovascular invasion, and percentage of clear cells. We herein proposed a prognostically relevant two-tiered grading system, classifying HCCC with a MI≥5/10 2 mm2 and/or tumor necrosis as high grade.
期刊介绍:
Modern Pathology, an international journal under the ownership of The United States & Canadian Academy of Pathology (USCAP), serves as an authoritative platform for publishing top-tier clinical and translational research studies in pathology.
Original manuscripts are the primary focus of Modern Pathology, complemented by impactful editorials, reviews, and practice guidelines covering all facets of precision diagnostics in human pathology. The journal's scope includes advancements in molecular diagnostics and genomic classifications of diseases, breakthroughs in immune-oncology, computational science, applied bioinformatics, and digital pathology.