Sanjo N, Komiya T, Tamaki M, Arai M, Fukaya S
Department of Neurology, Kanto Teishin Hospital.
Rinsho Shinkeigaku. 1994 Aug;34(8):838-41.
We reported a 69-year-old man exhibiting Garcin's syndrome caused by adenoid cystic carcinoma of the right submandibular gland. The patient first experienced abnormal sensations in his right cheek, and the cranial nerves on his right side gradually became affected. He was admitted for hoarseness and dysphagia. Physical examination revealed a right submandibular mass, and neurological examination revealed that the first cranial nerve and the fifth to twelfth cranial nerves on the right side were affected. Laboratory examination showed a rise of both serum and cerebrospinal fluid (CSF) ferritin, suggesting an intracranial invasion of the submandibular tumor. But other tumor markers, CSF protein and cell counts, CSF pathologic study and radiological studies for the central nervous system (CNS) were all negative. A submandibular tumor biopsy revealed adenoid cystic carcinoma. The radiation therapy, including the skull base, provided relief of the patient's symptoms, the tumor was reduced and serum and CSF ferritin level decreased. It is possible that CSF ferritin is a sensitive marker for CNS involvement of malignant tumor because of its permeability of the blood brain barrier and the absence of correlation between serum and CSF.
我们报告了一名69岁男性,其患有由右下颌下腺腺样囊性癌引起的加辛综合征。患者最初右侧脸颊出现异常感觉,右侧颅神经逐渐受到影响。他因声音嘶哑和吞咽困难入院。体格检查发现右下颌下肿块,神经学检查显示右侧第一颅神经以及第五至第十二颅神经均受影响。实验室检查显示血清和脑脊液(CSF)铁蛋白均升高,提示下颌下肿瘤颅内侵犯。但其他肿瘤标志物、CSF蛋白和细胞计数、CSF病理研究以及中枢神经系统(CNS)的放射学检查均为阴性。下颌下肿瘤活检显示为腺样囊性癌。包括颅底在内的放射治疗使患者症状缓解,肿瘤缩小,血清和CSF铁蛋白水平下降。由于其血脑屏障通透性以及血清与CSF之间缺乏相关性,CSF铁蛋白有可能是恶性肿瘤中枢神经系统受累的敏感标志物。