Yamamoto T, Imai T
Department of Neurology, Kitano Hospital, Osaka, Japan.
No To Shinkei. 1989 May;41(5):519-25.
Adenoid cystic carcinoma (ACC), usually arising in the major and minor salivary glands, is well known to otolaryngologists, but is rarely encountered by neurologists or neurosurgeons. We report two ACC patients who presented initially with cavernous sinus syndrome and in whom CT did not demonstrate apparent abnormalities. Case 1 is a 48-year-old man who first developed right cavernous sinus syndrome. The patient came to our hospital four years later. At this time, the only abnormality found on CT was the clouded sphenoid sinus on the left, which was interpreted as sinusitis. Two years later, the repeat CT revealed the enhancing lesions in the area of bilateral cavernous sinuses with bony destruction. The nasolaryngological exploration of the left sphenoid sinus made the diagnosis of ACC. Case 2 is a 43-year-old man who developed unilateral cavernous sinus syndrome over three months. No radiological abnormality was apparent to the neurologist and neurosurgeons, however, the otolaryngologist detected a nasopharyngeal mass diagnosed as ACC. Histological examinations of the biopsy specimen from both cases revealed mixed cribriform, tubular, and solid patterns characteristic of ACC. Electron microscopic study in Case 1 demonstrated microvilli-containing epithelial tumor cells forming true glandular lumens. In these cystic spaces there were cellular debris and crystalloids consisting of hexagonally-arranged tubules with a diameter of 25 nm. Although thick basement membrane was found in the basal portion of the tumor cells facing the connective tissue, there was no so-called pseudocysts lined by replicated basal lamina. It is possible that the specimen examined electron microscopically is limited to a particular epithelial region.(ABSTRACT TRUNCATED AT 250 WORDS)
腺样囊性癌(ACC)通常发生于大、小涎腺,耳鼻喉科医生对此较为熟悉,但神经科医生或神经外科医生很少遇到。我们报告了两名最初表现为海绵窦综合征且CT未显示明显异常的ACC患者。病例1是一名48岁男性,最初出现右侧海绵窦综合征。4年后患者前来我院。此时,CT上发现的唯一异常是左侧蝶窦混浊,被解释为鼻窦炎。两年后,复查CT显示双侧海绵窦区域有强化病变并伴有骨质破坏。对左侧蝶窦进行鼻咽喉探查后诊断为ACC。病例2是一名43岁男性,3个月内出现单侧海绵窦综合征。神经科医生和神经外科医生未发现明显的影像学异常,但耳鼻喉科医生检测到一个鼻咽部肿物,诊断为ACC。两例活检标本的组织学检查均显示出ACC典型的筛状、管状和实性混合模式。病例1的电子显微镜研究显示,含有微绒毛的上皮肿瘤细胞形成了真正的腺腔。在这些囊腔内有细胞碎片和由直径25nm的六边形排列小管组成的晶体。虽然在肿瘤细胞面向结缔组织的基底部分发现了厚基底膜,但没有由复制基底膜衬里的所谓假囊肿。电子显微镜检查的标本可能仅限于特定的上皮区域。(摘要截短于250字)