De Cesare A, Bononi M, Bianchi G, Atella F, Di Stefano D, Mingazzini P L
I Cattedra di Patologia Speciale Chirurgica, Università degli studi di Roma La Sapienza.
Ann Ital Chir. 1990 Sep-Oct;61(5):539-45.
The authors review 274 cases of Merkel's cells tumor reported in literature and present one case they personally observed. This kind of neoplasm develops itself inside the derma without involving the epidermidis and shows an aggressive biological behaviour. The authors describe its anatomopathologic characteristics and underline the importance of the immunohistochemistry particularly for a differential diagnosis with respect to lymphoma, amelanotic melanoma and the scarcely differentiated metastatic carcinoma. Undoubtedly the surgical treatment is to be preferred. It consists of the removal of the primitive lesion together with a preventive regional lymphadenectomy. Radio and chemotherapy still don't play a standard role, their results are anyway encouraging even if not well defined up to now. These therapies are used in several ways: together with surgery in case of local relapses or metastasis or as sole treatment. In consideration of the small number of available data, we can finally state that Merkel's cells tumor must be further studied in order to come to a certain definition of this neoplasm from a histogenetic and anatomopathologic point of view and, above all, to find out a treatment able to reduce the high mortality rate that still today characterizes the prognosis.
作者回顾了文献中报道的274例默克尔细胞癌病例,并展示了他们亲自观察到的1例病例。这种肿瘤在真皮内自行发展,不累及表皮,表现出侵袭性生物学行为。作者描述了其解剖病理学特征,并强调免疫组织化学对于与淋巴瘤、无黑色素黑色素瘤及低分化转移性癌进行鉴别诊断的重要性。毫无疑问,手术治疗是首选。手术包括切除原发病变并进行预防性区域淋巴结清扫。放疗和化疗尚未发挥标准作用,尽管目前其结果尚不明确,但无论如何都令人鼓舞。这些疗法有多种应用方式:在局部复发或转移时与手术联合使用,或作为单一治疗手段。考虑到现有数据较少,我们最终可以指出,必须对默克尔细胞癌进行进一步研究,以便从组织发生学和解剖病理学角度对这种肿瘤有明确的定义,最重要的是,找到一种能够降低目前仍然是其预后特征的高死亡率的治疗方法。