Molas G, Bougis-de-Brux M A, Potet F
Service d'Anatomie et de Cytologie Pathologique, Hôpital Beaujon, Clichy.
Gastroenterol Clin Biol. 1987 Dec;11(12):904-7.
A pediculed tumor of the rectum was discovered in a 63 years old man. Within the tumor adenomatous dysplastic proliferation was associated with a neuroendocrine small-cell anaplastic carcinoma. The neuroendocrine nature of the tumor was suspected on conventional optic microscopy and confirmed by a positive Grimelius technique. Specific typical granules were also found on electron microscopy. Immunohistochemical techniques using neurospecific enolase were also positive. Carcinomatous invasion was limited to the submucosa, but the surgical specimen showed that one lymph node was metastatic. Three months later, hepatic metastasis was suspected on physical examination and the patient died of hepatic failure ten months after the discovery of the tumor. Twenty-two similar cases were found in the literature: of these five cases were associated with benign adenomatous lesions. In all cases the patients died of early metastatic diffusion. This tumor raises the problems of diagnosis, terminology, classification and therapy: only aggressive chemotherapy, similar to that applied to the same type of carcinoma in the respiratory tract might improve prognosis.
一名63岁男性被发现患有直肠带蒂肿瘤。肿瘤内腺瘤样发育异常增殖与神经内分泌小细胞间变性癌相关。在传统光学显微镜下怀疑肿瘤具有神经内分泌性质，并通过阳性的 Grimelius 技术得以证实。电子显微镜下也发现了特异性典型颗粒。使用神经特异性烯醇化酶的免疫组织化学技术也呈阳性。癌性浸润局限于黏膜下层，但手术标本显示有一个淋巴结发生转移。三个月后，体格检查怀疑有肝转移，该患者在肿瘤发现十个月后死于肝功能衰竭。文献中发现了22例类似病例：其中5例与良性腺瘤性病变相关。所有病例患者均死于早期转移扩散。这种肿瘤引发了诊断、术语、分类和治疗等问题：只有采用类似于应用于呼吸道同类型癌的积极化疗，才可能改善预后。
