Chand Momal Tara, Edens Jacob, John Reynald, Lin Tayson Taixin, Anderson Ian Jacob
Ascension St. John Hospital, Department of Pathology. Detroit, Michigan, USA.
Ascension St. John Hospital, Department of Internal Medicine. Detroit, Michigan, USA.
Autops Case Rep. 2020 May 6;10(2):e2020156. doi: 10.4322/acr.2020.156.
Hyalinizing clear cell carcinoma (HCCC), also known as clear cell carcinoma, not otherwise specified [CCC, (NOS)], is a rare minor salivary gland tumor characterized by proliferation of clear cells, organized in trabecular cords, or solid nests within loose to densely hyalinized stroma. It is considered a diagnosis of exclusion by the World Health Organization (WHO) because other salivary tumors may also have a clear cell component. Hence, there is a wide differential diagnosis. EWSR1-ATF1 gene rearrangements are fairly specific for this tumor, however, one of the recent studies have described its presence in clear cell odontogenic carcinoma (CCOC) one of its histologic mimickers. EWSR1 and CREM fusions have recently been described in these tumors but its importance is still not well described. Here we present a case of a 33-year-old woman who presented with a recurrent lesion of the soft palate. Her initial lesion was resected and diagnosed as low-grade myoepithelial tumor. Surgical margins at the time of initial resection were positive and the re-excision was recommended but the patient did not undergo surgery. Two years later, local recurrence at the same site was found and an excision was performed yielding negative margins. Histopathologic examination revealed features consistent with hyalinizing clear cell carcinoma. The patient remains disease free 1 year after the re-excision. The pathology, clinical characteristics, differential diagnosis and treatment of hyalinizing clear cell carcinoma are reviewed.
透明细胞硬化性癌（HCCC），也称为未另行特指的透明细胞癌[CCC，（NOS）]，是一种罕见的小唾液腺肿瘤，其特征为透明细胞增生，呈小梁状条索或实性巢状排列于疏松至致密的硬化性间质中。世界卫生组织（WHO）认为它是一种排除性诊断，因为其他唾液腺肿瘤也可能有透明细胞成分。因此，鉴别诊断范围很广。EWSR1-ATF1基因重排对该肿瘤具有较高的特异性，然而，最近的一项研究描述了其在组织学上与之相似的透明细胞牙源性癌（CCOC）中的存在情况。最近在这些肿瘤中发现了EWSR1和CREM融合，但它们的重要性仍未得到充分描述。在此，我们报告一例33岁女性，她出现软腭复发性病变。其最初的病变被切除并诊断为低级别肌上皮瘤。初次切除时手术切缘阳性，建议再次切除，但患者未接受手术。两年后，在同一部位发现局部复发并进行了切除，切缘阴性。组织病理学检查显示的特征符合透明细胞硬化性癌。再次切除术后1年，患者无疾病复发。本文对透明细胞硬化性癌的病理学、临床特征、鉴别诊断及治疗进行了综述。
