A contemporary update on hyalinizing clear cell carcinoma: compilation of all in-house cases at our institution and a literature review spanning 2015–2020

Summary Tumors of the head and neck with clear cell features prompt a broad differential diagnosis. A relatively uncommon, but increasingly recognized, entity is hyalinizing clear cell carcinoma (HCCC). This neoplasm, first described in 1994, consists of clear cells arranged in nests or trabecule with a hyalinized stroma. These are low-grade neoplasms that only infrequently metastasize and rarely recur. They also often harbor a unique EWSR-ATF1 gene rearrangement. As the prognosis is excellent compared with other clear cell neoplasms, the correct diagnosis is key. Here we present all of the cases of HCCC in the past decade from our institution alongside a comprehensive literature review spanning 2015–2020 to further characterize this unusual malignancy.