Kedar A, Glassman M, Voorhess M L, Fisher J, Allen J, Jenis E, Freeman A I
Cancer. 1981 Apr 15;47(8):2077-80. doi: 10.1002/1097-0142(19810415)47:8<2077::aid-cncr2820470828>3.0.co;2-o.
A 26-month-old boy with Stage III abdominal ganglioneuroblastoma had tachycardia and hypertension. The hypertension increased following the institution of chemotherapy and necessitated the use of both alpha and beta adrenergic blocking agents to control the effects of the marked catecholamine production. After excision of the residual tumor, the blood pressure and urinary catecholamine excretion returned to normal. Histologic examination of this tissue under light microscopy revealed some sections of ganglioneuroblastoma as well as large areas of ganglioneuroma. Examination by electron microscopy demonstrated a moderate number of dense core neurosecretory-type granules in the cell bodies of the ganglion-like cells and an abundance of the same type of granules in the bundles of the interwoven cytoplasmic neural processes. To data, 32 months after diagnosis and 20 months off chemotherapy, the patient remains free of tumor and is in excellent general health. We postulate that the large number of secretory granules in this tumor permitted storage and release of markedly increased quantities of norepinephrine and resulted in a clinical profile similar to that associated with pheochromocytoma.
一名患有Ⅲ期腹部神经节神经母细胞瘤的26个月大男孩出现心动过速和高血压。化疗开始后高血压加重，需要同时使用α和β肾上腺素能阻滞剂来控制大量儿茶酚胺产生的影响。切除残留肿瘤后，血压和尿儿茶酚胺排泄恢复正常。该组织的光镜组织学检查显示部分为神经节神经母细胞瘤切片，还有大片神经节瘤区域。电镜检查显示，在类神经节细胞的胞体中有中等数量的致密核心神经分泌型颗粒，在交织的细胞质神经突起束中有大量相同类型的颗粒。截至目前，诊断后32个月且停止化疗20个月，该患者仍无肿瘤，总体健康状况良好。我们推测，该肿瘤中大量的分泌颗粒允许储存和释放显著增加的去甲肾上腺素，导致了与嗜铬细胞瘤相关的临床特征。
