Acute necrotizing encephalopathy predominantly affects young children and infants living in Japan and Taiwan, and is characterised by acute encephalopathy with seizures and decreased level of consciousness. The Hallmark of the disease is diffuse and symmetrical CNS lesions of both thalami, brainstem tegmentum, cerebral periventricular white matter and cerebellar medula. The clinical, radiological and pathological features of this disease, a disease entity established recently, is proposed by Masashi Mizuguchi et al in 1995. The aetiology is unknown but infectious or parainfectious process seems likely. The diagnosis can be made without difficulty on the basis of the combination of a typical clinical figures and characteristic radiologic findings. There is no specific therapy or prevention. The prognosis was poor in the 1980s but has improved recently. We experienced a case of 6-month-old female infant with acute necrotizing encephalopathy and a thalamic hemorrhage. We report this case with a review of the related literatures.
Brain Stem ; Child ; Consciousness ; Diagnosis ; Female ; Hemorrhage* ; Humans ; Infant ; Japan ; Prognosis ; Seizures ; Taiwan

Neurocutaneous melanosis is a rare congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We studied a case of Dandy-Walker syndrome with neurocutaneous giant melanosis in a 3 years old boy.
Brain ; Child, Preschool ; Dandy-Walker Syndrome* ; Dilatation ; Fourth Ventricle ; Humans ; Male ; Melanosis* ; Neurocutaneous Syndromes ; Nevus ; Parturition ; Seizures ; Skin

Craniopharyngiomas exhibit benign histologic features. However, such tumors have a relatively high incidence of recurrence after surgical removal, In order to device reliable and efficient methods in identifying craniopharyngiomas with increased risk for recurrence after surgical removal, proliferating cell nuclear antigen(PCNA) expressions as well as histological characteristics of the tumor were analyzed. There were 43 patients who had been surgically confirmed and had paraffin-embedded tissue from June, 1984 to May, 1993 available for analysis from our department. Of the 43 patients, eighteen cases were in children(age of 15 years of less) and 25 cases were in adults. The mean follow-up period was 42.7 months. The histologic types were adamantinomatous in 30 cases, squamous papillary in 9 cases and mixed in 4 cases. There was no case of tumor recurrence in the squamous papillary group, while the recurrence rate was about 55% in the adamantinous group after surgical removal alone. The mean age of the admantinous group was younger than that of the squamous papillary group(17.5 vs. 37.9 years old. p=0.0012), and the squamous papillary type was found only in adults(age over 20). In the group of 30 patients treated by surgical removal without radiation, the PCNA labelling index, calculated by counting the basal cell layer only, was significantly higher in the group with recurrence than without recurrence(9.51 vs. 6.58. p=0.001). However, the PCNA labelling index obtained by counting all cells in the four high-power(x400) fields failed to demonstrate any correlation with tumor recurrence. With a reference value of 8, PCNA index of the basal cell layer demonstrated the predictive sensitivity of 81.8% and specificity of 84.2% for tumor recurrence. There was no significant difference in PCNA labelling indices between adamantinous and squamous papillary types. As PCNA labelling index of the basal cell layer, as well as the histologic type, are sensitive indicators for prediction of tumor recurrence after surgical removal of craniopharyngiomas, therefore they should be considered as an index for biologic behavior of the tumor.
Adult ; Craniopharyngioma* ; Follow-Up Studies ; Humans ; Incidence ; Proliferating Cell Nuclear Antigen* ; Recurrence ; Reference Values ; Sensitivity and Specificity ; Treatment Outcome*

PURPOSE: Total mesorectal excision has been advocated as the effective operation for patients with rectal cancer to reduce the local recurrence rate after curative resection. Its rationale is to remove possible tumor foci at the mesorectum distal to the level of the rectal cancer. This study was undertaken to clarify the rationale for total mesorectal excision. METHODS: Total mesorectal excision was performed in 72 patients with rectal cancer who admitted in Severance Hospital between December, 1996 and December, 1997. The obtained mesorectums were classified to M0 (from the proximal margin to the distal margin of the tumor), M1 (from the distal margin to 2 cm below the distal margin), M2 (from 2 cm to 5 cm below the distal margin), and microscopic examination was done. RESULTS: The nodal metastases were detected in 7 cases and tumor deposits in 4 cases. Especially in M2 with Dukes' C2, the rate of nodal metastases was 3.6 percent and the rate of tumor deposits was 7.1 percent. The nodal metastases and tumor deposits in the distal mesorectum have no correlation with gross finding, size, location and differentiation of the tumor. CONCLUSIONS: En bloc excision of all mesorectal tissue down to at least 5 cm below the lower margin of the tumor is required for patients with advanced rectal cancer to remove possible metastatic lymph nodes and tumor deposits in the distal mesorectum.
Humans ; Lymph Nodes* ; Neoplasm Metastasis* ; Rectal Neoplasms* ; Recurrence

Reversible posterior leukoencephalopathy with hypertension is characterized by an acute and severe rise in blood pressure with headache, altered mental status, cortical visual disturbance, seizure and transient edematous changes in neuroimaging. The most common abnormality in neuroimaging is presumed edema involving the cortical and subcortical white matter predominant in the posterior region of the cerebral hemisphere and rarely the cerebellum and the brain stem, but not in the spinal cord. We experienced a case of 10-year-old girl with hypertensive encephalopathy involving the brainstem and the spinal cord.
Blood Pressure ; Brain Stem ; Cerebellum ; Cerebrum ; Child ; Edema ; Female ; Headache ; Humans ; Hypertension ; Hypertensive Encephalopathy ; Leukoencephalopathies* ; Neuroimaging ; Pheochromocytoma* ; Posterior Leukoencephalopathy Syndrome ; Seizures ; Spinal Cord*

No abstract available.
Aminoquinolines

Carcinoma ex pleomorphic adenoma is rare, aggressive, poorly understood malignancy that occurs in the salivary glands. In carcinoma ex pleomorphic adenoma, an epithelial malignancy develops in association with primary or recurrent benign pleomorphic adenoma. The patient was a 37-year-old female with slow-growing preauricular mass that has been present for 2 years. She visited the hospital because of sudden increase of mass size. The initial cytologic finding by fine needle aspiration biopsy showed what was probably a benign tumor and the radiologic finding revealed a 2.0x1.9 cm sized mass without cervical lymphadenopathy. After right superficial parotidectomy, the histologic examination revealed that tumor was composed of epithelial and mesenchymal component of pleomorphic adenoma and cribriform areas mimicking adenoid cystic carcinoma. Additionally, we did right near-total parotidectomy and postoperative radiotheraphy for 6 weeks. We present a rare case of carcinoma ex pleomorphic adenoma including adenoid cystic component in the parotid gland.
Adenoids* ; Adenoma, Pleomorphic* ; Adult ; Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Adenoid Cystic* ; Female ; Humans ; Lymphatic Diseases ; Parotid Gland* ; Salivary Glands

BACKGROUND AND OBJECTIVES: One of the hallmarks of aural cholesteatoma is chronic and recurrent infection. Initiation and perpetuation of the inflammatory response of cholesteatoma may result from an exaggerated host defense reaction of the cholesteatoma epithelium. However, the role of innate immune system in cholesteatoma has not been fully elucidated. Toll-like receptors (TLRs) are a part of the innate immune system involved in the response to microbial pathogen. TLRs appear to respond to pathogens and induce NF-kappaB activation. TLR 2 and TLR 4 seem to be related to the initiation of immune responses against gram negative and positive bacteria. We have investigated the expression of TLR 2, and 4 in the normal external auditory canal skin and cholesteatoma. SUBJECTS AND METHOD: A real time RT-PCR was performed to determine and quantify the expression of TLR 2 and 4 mRNA. Immunohistochemical staining was performed for 17 cases of cholesteatoma and 8 cases of normal auditory canal skin to demonstrate the distribution of TLR 2 and 4. RESULTS: All cholestatoma and normal external auditory canal skin expressed both TLR 2 and 4 mRNA. The mRNA of TLR 2 and 4 were expressed significantly higher in cholesteatoma than in the normal external auditory canal skin (p<0.05). Immunohistochemistry using anti-TLR2 and anti-TLR4 antibody revealed the expression of TLR 2 and 4 in the epithelial cells of the cholesteatoma. CONCLUSION: These findings suggest that distinctive patterns of the innate immune related receptors, TLR 2 and 4 system, constitute a part of the innate immune system in the cholesteatoma.
Bacteria ; Cholesteatoma* ; Cholesteatoma, Middle Ear ; Ear Canal ; Ear, Middle ; Epithelial Cells ; Epithelium ; Immune System ; Immunohistochemistry ; NF-kappa B ; RNA, Messenger ; Skin ; Toll-Like Receptor 2* ; Toll-Like Receptor 4* ; Toll-Like Receptors*

The incidence of lung cancer and its mortality rate are increasing in Korea. At the time of diagnosis, 40% patients of lung cancer patients had metastatic lesions. The common metastatic sites are the contralateral lung, bone, liver, adrenal gland and the brain. Metastasis to oral mucosa is rarely encountered in lung cancer and metastasis to the gingiva is more uncommon. Approximately 1% of malignant carcinomas in the oral cavity are the result of metastases, and 10-25% of metastatic cancers originate from lung cancer. Clinically metastatic gingival lesions are benign including hemangioma, pyogenic granuloma, giant-cell granuloma or a peripheral fibroma. Often metastases to the gingiva are diagnosed too late and by the time they are detected, they have metastases to other organs. Here we report a case of small cell lung carcinoma that had metastased to the gingiva with review of relevant literature.
Adrenal Glands ; Brain ; Diagnosis ; Fibroma ; Gingiva* ; Granuloma ; Granuloma, Pyogenic ; Hemangioma ; Humans ; Incidence ; Korea ; Liver ; Lung ; Lung Neoplasms ; Mortality ; Mouth ; Mouth Mucosa ; Neoplasm Metastasis* ; Small Cell Lung Carcinoma*

BACKGROUND: To compare the efficacies and side effects of etoposide, cisplatin/cyclophosphamide, adriamycin, vincristine(VPP/CAV) with that those of carboplatin etoposide(CE) in extensive stage small cell lung cancer patients. METHOD: Patients with extensive stage small cell lung cancer who has measurable disease were eligible. VPP/CAV group(n=22) was treated with cisplatin(60mg/m2 iv. D1) etoposide(100mg/m2 iv. D1-3),after and 3 weeks later cyclophosphamide(1000mg/m2 iv. D1), adriamycin(40mg/m2 iv. D1), and vincristine(1.4mg/m2 iv. D1), were administered alternatively. CE group (n=22) was treated with carboplatin (325mg/m2 iv. D1) and etoposide (100mg/m2 iv. D1-3)(;) repeated treatment was performed every 3 weeks. RESULT: Forty four patients were eligible for the study. Overall The overall response rate was 61.4% (complete remission rate 0%, partial response rate 61.4%, stable disease rate 25%, progressive disease rate 13.6%), and median survival was 10.8 months. In VPP/CAV group, response rate was 54.5%(complete remission rate 0%, partial response rate 54.4%, stable disease rate 27.3%, progressive disease rate 18.2%), and, in carboplatin/etoposide group, the response rate was 68.2%(complete remission rate 0%, partial response rate 68.2%, stable disease rate 22.7%, progressive disease rate 9.1%). The median survival time was 9.5 months in the VPP/CAV group and 11 months in CE group. The toxicity of both group was moderate, and anemia was more frequent in the CE group. CONCLUSION: VPP/CAV regimen and CE regimen produced similar response rate and survival time rates and survival times in extensive stage small cell lung cancer patients. We may suggest that CE regimen are effective may be effective as part of the initial therapy of for extensive stage small cell lung cancer.
Anemia ; Carboplatin ; Doxorubicin ; Drug Therapy* ; Etoposide ; Humans ; Small Cell Lung Carcinoma*