Minni F, Casadei R, Santini D, Verdirame F, Zanelli M, Vesce G, Marrano D
Department of Surgical and Anaesthesiological Science, University of Bologna, Italy.
Ital J Gastroenterol Hepatol. 1997 Dec;29(6):558-63.
Gastrointestinal autonomic nerve tumor is very rare and it is difficult to distinguish this tumor from other gastrointestinal tumors due to the absence of clinical, instrumental and macroscopic features which allow pre- or intraoperative diagnosis. Our aim was to recognize the characteristic features (preoperative, intraoperative, pathological) that would allow diagnosis of gastrointestinal autonomic nerve tumor. A case of gastrointestinal autonomic nerve tumor of the jejunum is reported. Surgical specimen was routinely processed. Immunohistochemical staining was performed according to modified immunoperoxidase Avidin-Biotin-Peroxidase Complex method. An electron microscopy study was also performed. The tumor mass showed some characteristic pathological findings: histologically, it was composed of spindle cells and epithelioid cells; immunohistochemically, a focal positivity for Neuron Specific Enolase was shown, and finally, ultrastructural examination showed neuron-like cells with long cytoplasmic processes containing microtubules and bulbouns synapse-like structures with dense core neurosecretory-type granules. Preoperatively gastrointestinal autonomic nerve tumor of the jejunum must be considered and treated as a malignant tumor. A correct diagnosis is possible only with immunohistochemical and ultrastructural studies. It is probable that this tumor is more common than previously thought.
胃肠道自主神经肿瘤非常罕见，由于缺乏能够进行术前或术中诊断的临床、影像学及大体特征，难以将该肿瘤与其他胃肠道肿瘤相鉴别。我们的目的是识别出能够诊断胃肠道自主神经肿瘤的特征（术前、术中、病理）。本文报道了1例空肠胃肠道自主神经肿瘤病例。手术标本按常规方法处理。采用改良免疫过氧化物酶抗生物素蛋白-生物素-过氧化物酶复合物法进行免疫组织化学染色。同时进行了电子显微镜研究。肿瘤块呈现出一些特征性病理表现：组织学上，由梭形细胞和上皮样细胞组成；免疫组织化学显示神经元特异性烯醇化酶呈局灶性阳性；最后，超微结构检查显示具有长细胞质突起的神经元样细胞，其突起内含有微管以及带有致密核心神经分泌型颗粒的球状突触样结构。术前必须将空肠胃肠道自主神经肿瘤视为恶性肿瘤并进行治疗。只有通过免疫组织化学和超微结构研究才能做出正确诊断。这种肿瘤可能比之前认为的更为常见。
