原发性肝脏恶性黑色素瘤的诊治进展

Advances in the diagnosis and treatment of primary malignant melanoma of the liver

恶性黑色素瘤是由皮肤等器官黑色素细胞产生的肿瘤,该病多见于中老年人,其常见发生部位为皮肤、眼部、肛周等,少数可发生于鼻腔、鼻窦、口腔、喉部、脑脊膜、胆囊、消化道、骨、肺、肾上腺等,原发于肝脏的恶性黑色素瘤临床极为罕见。原发性黑色素瘤主要临床表现为肝大、黄疸、消瘦、肝区叩击痛等,表现特异性不强,其诊断需借助于病理学及免疫组织化学染色支持,并结合临床排除其他部位恶性黑色素瘤生长可能。在治疗方面,目前国内外肝脏原发性恶性黑色素瘤无特效的治疗方法。对于单发或肿瘤体积相对较小的个体可考虑积极手术治疗,多发及体积较大者可行放射及化学治疗、免疫调节治疗。但一般情况下,原发性黑色素瘤预后相对较差。

Malignant melanoma is a kind of tumor generated from melanocytes of skin and other organs that often offends old people. The common site for it is the skin,eyes and crissum,and it seldom occurs in the nasal cavity,paranasal sinus,oral cavity,laryngeal,meninges,gallbladder,digestive tube,bone,lung,adrenal. Primary liver malignant melanoma is extremely rare. The main clinical manifestations are hepatomegaly,jaundice,weight loss,liver percussion pain,and the specificity of performance is not strong. The diagnosis should be made with the aid of pathology and immunohistochemistry after other primary origins have been excluded combined with clinic. At present,primary malignant melanoma of the liver is still lack of effective treatment methods. Patients with single or small lesion can be considered aggressive surgery,and patients with multiple and large lesions should be treated by radiotherapy,chemotherapy and immune modulation. But in general,the prognosis is relatively poor.