Department of Otolaryngology, Head and Neck Surgery, Poznań University of Medical Sciences.
Institute of Human Genetics, Polish Academy of Sciences.
Curr Opin Otolaryngol Head Neck Surg. 2023 Apr 1;31(2):134-145. doi: 10.1097/MOO.0000000000000872. Epub 2023 Jan 24.
The current review is to present the current knowledge regarding epidemiology, diagnostics, and management of malignant adnexal neoplasms (MANs).
Immunotherapy and gene-related therapies are still being developed as the methods of salvage treatment in advanced and disseminated cases: CACNA1S, ATP2A1, RYR1, and MYLK3, as well as p53 or the JAK/STAT pathways, may be therapeutic targets; the efficiency of talimogene laherparepvec and nivolumab is assessed.
MANs are rare tumors, but due to the aging of population their incidence is increasing. Their clinical presentation is unspecific, which makes the diagnosis challenging. Histopathological assessment is difficult even for experienced pathologists. Mohs micrographic surgery or wide local excision are recommended to treat primary lesions. Adjuvant radiotherapy may be beneficial in case of insufficient or positive surgical margins, in nodal metastases, in selected types of MANs like sebaceous, trichilemmal, and pilomatrix carcinomas, and as the induction treatment in large tumors located in medically fragile or cosmetically important regions. The role of chemotherapy is not well defined; however, it is recommended in distant metastases. Immunotherapy can improve the prognosis in advanced stage of the disease.
本文旨在介绍恶性附件肿瘤（MANs）的流行病学、诊断和治疗的最新进展。
免疫治疗和基因相关治疗仍在开发中，作为晚期和播散性病例的挽救治疗方法：CACNA1S、ATP2A1、RYR1 和 MYLK3 以及 p53 或 JAK/STAT 通路可能是治疗靶点；评估了 talimogene laherparepvec 和 nivolumab 的疗效。
MANs 是罕见的肿瘤，但由于人口老龄化，其发病率正在增加。它们的临床表现不特异，这使得诊断具有挑战性。即使是有经验的病理学家，组织病理学评估也很困难。Mohs 显微外科手术或广泛局部切除术推荐用于治疗原发性病变。对于手术切缘不足或阳性、淋巴结转移、某些类型的 MANs（如皮脂腺癌、毛根鞘癌和毛囊基质癌）以及位于医学脆弱或美容重要区域的大型肿瘤，辅助放疗可能是有益的，可作为诱导治疗。化疗的作用尚未明确；然而，在远处转移时推荐化疗。免疫治疗可以改善晚期疾病的预后。
