Matsuno A, Hashizume K, Suzuki K, Tsuzuki N, Shibayama E, Ishikawa H
Department of Neurosurgery, National Defence Forces Central Hospital.
No To Shinkei. 1992 Oct;44(10):935-9.
A 28-year-old woman was hospitalized in drowsy state with signs of increased intracranial pressure. CT scans revealed diffuse increased density with marked enhancement in the subarachnoid space, as well as ventricular dilatation. V-P shunt operation was performed to control intracranial pressure. Repeated cytological examinations of CSF couldn't determine the tumor origin. CT scan of thoracic spine showed a cystic tumor in its dorsal aspect. T2-weighted MRI revealed multiple spotty low intensity, specific to melanin granules, throughout the whole spine. Her thoracic spine was explored, and the intradural tumor was partially removed. Histopathological examination revealed the tumor cell which had dark nucleus with conspicuous nucleolus and cytoplasmic granules. These findings were compatible with malignant melanoma. Her general condition were deteriorated progressively and she died about 5 months after her admission. Postmortum examination showed diffuse leptomeningeal invasion of dark tumor throughout the entire central nervous system, and metastasis to peritoneum and omentum via V-P shunt system. Histopathological examination proved the tumor to be malignant melanoma. Electrone microscopic examination also revealed melanosome in the cytoplasm. Primary intracranial malignant melanoma is divided in two groups, nodular type and leptomeningeal type. In the latter type, early diagnosis is very difficult, just as in our case, because only a little tissue specimen can be obtained. In a case of leptomenigeal carcinomatosis, possibility of primary malignant melanoma, though rare, should always be kept in mind, and specific staining such as Fontana-Masson's staining should be tried.
一名28岁女性因颅内压升高症状处于嗜睡状态而住院。CT扫描显示蛛网膜下腔密度弥漫性增加且有明显强化，同时伴有脑室扩张。进行了脑室-腹腔分流术以控制颅内压。脑脊液的反复细胞学检查无法确定肿瘤起源。胸椎CT扫描显示其背侧有一个囊性肿瘤。T2加权磁共振成像显示整个脊柱有多个斑点状低信号，这是黑色素颗粒特有的表现。对其胸椎进行探查，部分切除了硬膜内肿瘤。组织病理学检查显示肿瘤细胞有深色细胞核，核仁明显，还有细胞质颗粒。这些发现符合恶性黑色素瘤。她的一般状况逐渐恶化，入院约5个月后死亡。尸检显示整个中枢神经系统弥漫性软脑膜被深色肿瘤侵犯，并通过脑室-腹腔分流系统转移至腹膜和大网膜。组织病理学检查证实肿瘤为恶性黑色素瘤。电镜检查也显示细胞质中有黑素小体。原发性颅内恶性黑色素瘤分为结节型和软脑膜型。在后一种类型中，早期诊断非常困难，就像我们这个病例一样，因为只能获得少量组织标本。在软脑膜癌病的病例中，虽然原发性恶性黑色素瘤可能性很小，但应始终牢记，并且应尝试进行如Fontana-Masson染色等特殊染色。
