Richtig E, Regauer S, Jakse R, Anderhuber W, Smolle J
Universitätsklinik für Dermatologie und Venerologie, Abteilung für Allgemeine Dermatologie, Auenbruggerplatz 8, 8036 Graz, Osterreich.
Hautarzt. 2002 Feb;53(2):106-13. doi: 10.1007/s00105-001-0307-7.
Primary malignant melanoma of the nasal cavity and paranasal sinuses is a rare entity felt to have a poor prognosis. We examined the frequency and characteristics of this type of melanoma in a defined geographic region over a period of 16 years.
PATIENTS/METHODS: Nine patients (eight men, one woman, mean age 62 years) with primary nasal and sinusoidal malignant melanoma were treated between 1985 and 2000 in the Department of Dermatology in cooperation with the Department of Otorhinolaryngology. All patients underwent surgery, eight with clinical complete tumor removal and one with only partial removal. All patients had radiotherapy after surgery. History, potential risk factors, clinical presentation, histological and immunohistochemical data and clinical course were documented.
All patients died because of their tumor within the observation period (median survival: 30 months, range 7 to 75 months). There were three different courses of the disease: (1) uncontrollable primary tumor (one patient, survival 11 months); (2) local recurrence with metastasis (five patients, median survival 40.2 months); (3) metastasis after a disease-free interval (three patients, median survival 19.3 months).
Malignant melanoma of the nasal cavity and paranasal sinuses is a rare entity which is usually rapidly fatal. Effective treatment approaches are not available.
鼻腔和鼻窦原发性恶性黑色素瘤是一种罕见的疾病，预后较差。我们对某一特定地理区域内16年间此类黑色素瘤的发病频率及特征进行了研究。
患者/方法：1985年至2000年间，9例（8例男性，1例女性，平均年龄62岁）鼻腔和鼻窦原发性恶性黑色素瘤患者在皮肤科与耳鼻咽喉科合作下接受了治疗。所有患者均接受了手术，8例实现了临床肿瘤完全切除，1例仅部分切除。所有患者术后均接受了放疗。记录了患者的病史、潜在危险因素、临床表现、组织学和免疫组化数据以及临床病程。
所有患者均在观察期内因肿瘤死亡（中位生存期：30个月，范围7至75个月）。疾病发展过程有三种不同情况：（1）原发性肿瘤无法控制（1例患者，生存期11个月）；（2）局部复发伴转移（5例患者，中位生存期40.2个月）；（3）无病间期后发生转移（3例患者，中位生存期19.3个月）。
鼻腔和鼻窦恶性黑色素瘤是一种罕见疾病，通常会迅速导致死亡。目前尚无有效的治疗方法。
