Hospital of the University of Pennsylvania, Philadelphia, 19104, PA, USA.
Hospital of the University of Pennsylvania, Philadelphia, 19104, PA, USA.
Hum Pathol. 2021 May;111:45-51. doi: 10.1016/j.humpath.2021.01.007. Epub 2021 Feb 10.
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.
头颈部具有透明细胞特征的肿瘤提示广泛的鉴别诊断。一种相对不常见但越来越被认识的实体是透明细胞癌（HCCC）。这种肿瘤于 1994 年首次描述，由巢状或小梁状排列的透明细胞和玻璃样基质组成。这些是低度恶性肿瘤，仅偶尔转移且很少复发。它们还经常携带独特的 EWSR-ATF1 基因重排。与其他透明细胞肿瘤相比，HCCC 的预后非常好，因此正确的诊断是关键。在此，我们展示了过去十年中我院的所有 HCCC 病例，并进行了全面的文献复习，涵盖了 2015-2020 年的病例，以进一步描述这种不常见的恶性肿瘤。
