Miyamoto Kodai, Yanagi Teruki, Maeda Takuya, Ujiie Hideyuki
Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo 060-8638, Japan.
Cancers (Basel). 2022 Oct 25;14(21):5232. doi: 10.3390/cancers14215232.
Eccrine porocarcinoma, also known as porocarcinoma (PC) and malignant eccrine poroma, is very rare and is known to arise from the cutaneous intraepidermal ducts of the sweat glands. Its etiology is not well understood; however, some studies suggest that PC tumors originate from benign eccrine poroma. Recently, several gene alterations have been reported in PC that can reveal mechanisms of the oncogenic process. Since the clinical and histopathological findings of PC are variable, PC is difficult to diagnose precisely, especially when the histology resembles that of cutaneous squamous cell carcinoma or poroma. Immunohistochemical staining with carcinoembryonic antigen and epithelial membrane antigen may help to distinguish PC from other tumors. The standard treatment for local PC is wide local excision. The prognosis of patients with metastatic PC is poor, with mortality rates of approximately 60-70%. The efficacy of radiation and chemotherapy for metastatic PC is limited; however, immunotherapy with pembrolizumab, a programmed cell death protein 1 inhibitor, could be a promising treatment. This review focuses on the history, pathogenesis, pathological features, diagnosis, and treatment of eccrine porocarcinoma.
小汗腺汗孔癌，也称为汗孔癌（PC）和恶性小汗腺汗孔瘤，非常罕见，已知起源于汗腺的皮肤表皮内导管。其病因尚不完全清楚；然而，一些研究表明，PC肿瘤起源于良性小汗腺汗孔瘤。最近，在PC中报道了几种基因改变，这些改变可以揭示致癌过程的机制。由于PC的临床和组织病理学表现各不相同，PC难以准确诊断，尤其是当组织学与皮肤鳞状细胞癌或汗孔瘤相似时。癌胚抗原和上皮膜抗原的免疫组化染色可能有助于将PC与其他肿瘤区分开来。局部PC的标准治疗方法是广泛局部切除。转移性PC患者的预后较差，死亡率约为60-70%。放疗和化疗对转移性PC的疗效有限；然而，使用程序性细胞死亡蛋白1抑制剂帕博利珠单抗进行免疫治疗可能是一种有前景的治疗方法。这篇综述重点关注小汗腺汗孔癌的历史、发病机制、病理特征、诊断和治疗。
