Department of Otolaryngology, Head and Neck Surgery, Philipps University Marburg, Marburg, Germany.
Melanoma Res. 2011 Dec;21(6):475-82. doi: 10.1097/CMR.0b013e32834b58cf.
Approximately 50% of mucosal melanomas affect the head and neck region representing approximately 9% of all malignant head and neck tumors. The pathogenesis of this disease is unknown. Mucosal melanomas are characterized by an aggressive biological behavior, leading to a 5-year survival rate of less than 25%. Data for this review were identified by searches of Medline, Current Contents, PubMed, and references from relevant articles using the terms 'mucosal melanoma,' 'head and neck melanoma,' 'c-kit mutation in melanoma,' and 'c-kit inhibitors'. Therapy aims for the complete surgical excision of the primary tumor, whereas sentinel node biopsy is not established and present data do not support the addition of radiotherapy. Mutilating operations of larger tumors should be avoided, as they do not inhibit the frequent development of distant metastasis. C-kit mutations and amplifications are found in approximately 15-30% of mucosal and acral-lentiginous melanomas. Therefore, the use of so-called targeted therapies addressing molecular structures in mucosal melanomas seem to represent new promising treatment tools. In this study, we review the literature regarding epidemiology, molecular pathology, and therapy of mucosal melanomas of the head and neck emphasizing c-kit protein inhibiting treatment modalities for tumors carrying c-kit mutations.
约 50%的黏膜黑色素瘤影响头颈部，占所有头颈部恶性肿瘤的约 9%。该疾病的发病机制尚不清楚。黏膜黑色素瘤的生物学行为具有侵袭性，导致 5 年生存率低于 25%。本综述的数据通过在 Medline、Current Contents、PubMed 中搜索以及使用“黏膜黑色素瘤”、“头颈部黑色素瘤”、“黑色素瘤中的 c-kit 突变”和“c-kit 抑制剂”等术语从相关文章的参考文献中确定。治疗的目的是完全切除原发性肿瘤，而前哨淋巴结活检尚未建立，目前的数据不支持增加放疗。不应避免对较大肿瘤进行破坏性手术，因为它们并不能抑制远处转移的频繁发生。约 15-30%的黏膜和肢端雀斑样黑色素瘤中存在 c-kit 突变和扩增。因此，使用针对黏膜黑色素瘤中分子结构的所谓靶向治疗似乎代表了新的有前途的治疗手段。在这项研究中，我们回顾了头颈部黏膜黑色素瘤的流行病学、分子病理学和治疗方面的文献，重点介绍了携带 c-kit 突变的肿瘤的 c-kit 蛋白抑制治疗方法。
