Lin Chin-Yew, Yang Shih-Wei, Lai Chien-Hong
Department of Pathology, Chang Gung Memorial Hospital, Keelung, Taiwan, ROC.
Chang Gung Med J. 2003 Nov;26(11):857-62.
Malignant melanoma is a highly lethal melanocytic neoplasm, usually affecting the skin. Primary malignant melanoma of the nasal cavity is rarely seen. Clinically, most patients display initial nonspecific symptoms of unilateral nasal obstruction or epistaxis. The prognosis is generally poor, with a mean survival time of 3.5 years. Extensive local invasion and distant metastasis to other organs may occur. The usual treatment of choice is radical excision. Radiotherapy and chemotherapy appear to have little effect. We report a fatal case of intranasal cavity malignant melanoma in which the patient initially presented with blood-tinged sputum, productive cough, and intermittent fever. Preoperative hepatic metastasis was found. Palliative surgery was performed to excise the nasal cavity tumor. Then, 6 courses of chemotherapy were further administered. Unfortunately, regional cervical nodal involvement and pancreatic head metastases occurred 1.5 years after the diagnosis. The patient's condition rapidly deteriorated, followed by death. We have chosen to discuss this aggressive condition because of its rarity and also to emphasize the importance of its early detection through vigilant attention to nonspecific nasal symptoms. A review of the literature concerning intranasal malignant melanoma is presented. We further discuss its possible etiology, site of origin, incidence, clinical presentations, principles of management, and outcome.
恶性黑色素瘤是一种高度致命的黑素细胞肿瘤,通常累及皮肤。鼻腔原发性恶性黑色素瘤较为少见。临床上,大多数患者最初表现为单侧鼻塞或鼻出血等非特异性症状。预后一般较差,平均生存时间为3.5年。可发生广泛的局部侵犯及远处器官转移。通常的治疗选择是根治性切除。放疗和化疗似乎效果不佳。我们报告一例鼻腔恶性黑色素瘤致死病例,该患者最初表现为痰中带血、咳痰及间歇性发热。术前发现肝转移。行姑息性手术切除鼻腔肿瘤。随后,进一步进行了6个疗程的化疗。不幸的是,诊断1.5年后出现区域颈部淋巴结受累及胰头转移。患者病情迅速恶化,随后死亡。我们之所以选择讨论这种侵袭性疾病,是因其罕见,同时也是为了强调通过警惕非特异性鼻部症状实现早期发现的重要性。本文对鼻腔恶性黑色素瘤的相关文献进行了综述。我们进一步讨论了其可能的病因、起源部位、发病率、临床表现、治疗原则及预后。