Takahashi Y, Kuriyama M, Kawada Y, Komeda H, Horie M, Isogai K
Department of Urology, Gifu University School of Medicine.
Hinyokika Kiyo. 1988 Dec;34(12):2149-54.
We present adult adrenal ganglioneuroblastoma with giant regional lymph node metastasis. The patient was a 21-year-old male, who had a left adrenal tumor diagnosed by ultrasonography incidentally. He was moderately developed and physical examination was almost normal. Blood pressure was within the normal range. Only the value of the vanilmandelic acid, which was 68.7 mg/day, in the urine was abnormal. Other laboratory data were almost normal. Neither chemical nor physical hormonal disorders were present. We carried out the radical surgical treatment by trans-thoracoabdominal approach under the diagnosis of left adrenal neuroblastoma. Pathological diagnosis was ganglioneuroblastoma. After the operation, the patient received both systemic chemotherapy of vincristine and cyclophosphamide and immunotherapy of OK-432 by subcutaneous administration. Furthermore, 40 Gy of the regional irradiation of 60Co to the operative space after the surgery was performed. At eight months after the operation, he is alive with no evidence of disease. Ganglioneuroblastoma is a neuroblastic tumor including neuroblastoma, which commonly occurs in childhood. The prognosis of the neuroblastic tumors which occur either in the adrenal gland of a young child or in an older child is poor. On the other hand, the biological activity of the adult neuroblastic tumors is different from that of the pediatric neuroblastic tumors. In the adult, the adrenal ganglioneuroblastoma does not always have a poor prognosis. The multimodality treatment which we performed is effective for the neuroblastic tumor. Ultrasonography is both effective and adequate for the screening of the retroperitoneal lesion such as in our case.
我们报告一例伴有巨大区域淋巴结转移的成人肾上腺节细胞神经母细胞瘤。患者为21岁男性,因超声偶然发现左肾上腺肿瘤。他发育中等,体格检查基本正常。血压在正常范围内。仅尿香草扁桃酸值异常,为68.7mg/天。其他实验室检查数据基本正常。未发现化学或生理性激素紊乱。在诊断为左肾上腺神经母细胞瘤后,我们采用经胸腹部联合入路进行了根治性手术治疗。病理诊断为节细胞神经母细胞瘤。术后,患者接受了长春新碱和环磷酰胺的全身化疗以及皮下注射OK-432的免疫治疗。此外,术后对手术区域进行了40Gy的60Co局部照射。术后八个月,他存活且无疾病证据。节细胞神经母细胞瘤是一种包括神经母细胞瘤的神经母细胞性肿瘤,常见于儿童期。发生在幼儿肾上腺或较大儿童的神经母细胞性肿瘤预后较差。另一方面,成人神经母细胞性肿瘤的生物学活性与儿童神经母细胞性肿瘤不同。在成人中,肾上腺节细胞神经母细胞瘤并不总是预后不良。我们实施的多模式治疗对神经母细胞性肿瘤有效。超声检查对于筛查如我们病例中的腹膜后病变既有效又充分。