Tow cases of congenital ectodermal defect in brothers aged 15 and 10 years were pesinted and the literature was reviewed. They had lack of sweating, sparseness of the hard hairs, absence of lanugo hairs, maldevelopment or defect of most of the teeth, etc. They seemed to be angidrotic type associated with a sex-linked reccessive inheritance in their family backgroud.
Ectodermal Dysplasia* ; Hair ; Humans ; Siblings ; Sweat ; Sweating ; Tooth ; Wills

No abstract available.
Female ; Granulosa Cell Tumor* ; Granulosa Cells* ; Humans ; Infant*

Pharmacologic therapy is still the primary management for epilpsy; however, surgical treatment is a reasonable therapeutic option for patients suffering from medically intractable seizures, especially temporal lobe epilepsy having a documented unilateral epileptogenic area. Thirty nine patients with pharmaco-resistant complex partial seizures underwent anterior temporal lobectomy and hippocampectomy in 38 cases and frontal cortisectomy in one case. On pathological examination, hippocampal sclerosis was a predominent pathologic finding and was identified in 18 cases. Other non-neoplastic lesions consisted of 5 cases of vascular lesions(2 cavernous angiomas, 2 arteriovenous malformations and 1 angiomatosis), 3 cases of fibrous nodule, 2 cases of cicatrical changes of cerebral cortex, and 1 case of parasitic infection. Neoplasms including two cases of oligodendroglioma and one case of anaplastic astrocytoma were also noted. In seven cases, there was no detectable lesion on gross and microscopic examination. On post-operative follow-up, seizures were completely terminated in most cases(31 cases, 79%). The rest of the patients also displayed marked alleviation of symptoms. The seizures tended to recur more aften among the patients with neoplasm or no pathologically detectable lesion. In order to detect any minute pathological lesion, thorough gross and microcsopic examinations are considered to be essential.

Heterotopic brain tissue is a developmental anomaly of neurogenic origin with no malignant potential, and is usually present around the nose of children and infants. So it has been called nasal glioma. But, even more rarely, heterotopic glial tissue may be found in various sites other than nasal cavity, such as the ethmoidal sinus, palate, tonsillar area, pharynx, ear, subcutaneous tissue, lung, and female genital tract. We experienced a more unusual case of a polypoid heterotopic brain tissue in the soft palate in a 3-year-old boy. The mass was microscopically reminiscent of "gliosis" of the central nervous system and interestingly contained choroid plexus focally. The glial nature of the lesion was confirmed by glial fibrillary acidic protein immunostain.
Brain* ; Central Nervous System ; Child ; Child, Preschool ; Choroid Plexus ; Ear ; Female ; Glial Fibrillary Acidic Protein ; Glioma ; Humans ; Infant ; Lung ; Male ; Nasal Cavity ; Nose ; Palate ; Palate, Soft* ; Pharynx ; Subcutaneous Tissue

No abstract available.
Oculomotor Nerve Diseases ; Oculomotor Nerve

We report here on a case of an ependymoma arising from the pelvis in a 25-year-old woman. She had no evidence of abnormality in her brain and bilateral ovaries. The diagnosis was based on light microscopic, immunohistochemical, and ultrastructural features of a typical ependymoma, including the patterns of pseudorosette or true ependymal rosette, the strong immunopositivity for glial fibrillary acid protein and intermediate filaments, and cilia of tumor cells. The mass was over 20 cm in maximum diameter, and it was located between the uterus and rectum without any connection to bilateral ovaries. There were many metastatic nodules in the pelvis and omentum. In addition, the proliferation index in the most active area was 10% by immunohistochemistry using monoclonal antibody MIB-1. Although the prognosis of the pelvic ependymoma is known to be difficult to evaluate, this case may serve to illustrate the poor prognostic course, according to the size of the tumor, the evidence of metastasis, and the MIB-1 labelling index.
Adult ; Brain ; Cilia ; Diagnosis ; Ependymoma* ; Female ; Glial Fibrillary Acidic Protein ; Humans ; Immunohistochemistry ; Intermediate Filaments ; Neoplasm Metastasis ; Omentum ; Ovary ; Pelvis* ; Prognosis ; Rectum ; Uterus

PURPOSE: p53 gene is a well-known suppressor gene of tumor. And the p53 protein expression in immunohistochemical staining acts a role as a prognostic indicator in prostate and bladder tumor. However, the validity of p53 protein expression was not determined as an independent factor of prognosis in renal cell carcinoma(RCC). Furthermore, there were not enough studies about whether the expression of p53 protein plays a role as a prognostic indicator in RCC or not. Therefore, we evaluate the prognostic significance of p53 protein expression in RCC. MATERIALS AND METHODS: To evaluate the prognostic significance of p53 immunohistochemical staining in RCC, paraffin embedded specimens taken from 84 patients with RCC were studied from January 1982 to April 1997, retrospectively. In addition to the prognostic significance of p53 immunohistochemical staining, correlation between p53 immunohistochemical staining and pT category, nuclear grade, and histologic type was studied. RESULTS: The expression rate of p53 protein was 19.0%(16/84). The most important prognostic indicator of RCC was pT category(relative risk=2.19, p=0.007). And the second important prognostic indicator was the expression of p53 protein(relative risk=1.82, p=0.017). Nuclear grade had prognostic significance(p=0.027) but was not an independent prognostic indicator. The expression of p53 protein did not correlate with pT category, nuclear grade or histologic pattern. CONCLUSIONS: We have shown that the expression of p53 protein in RCC is the second most important prognostic indicator. In conclusion p53 immunohistochemical staining can be used to predict the prognosis in patients with localized RCC after radical nephrectomy.
Carcinoma, Renal Cell* ; Genes, p53 ; Genes, Suppressor ; Humans ; Nephrectomy* ; Paraffin ; Prognosis ; Prostate ; Retrospective Studies ; Urinary Bladder Neoplasms

Diarrhea is a common adverse event of docetaxel with 20%-40% of incidence and severe diarrhea occurs in 5%-6%. Several treatment guidelines for chemotherapy induced diarrhea (CID) exist, however the prophylaxis for that is not well known. We describe a new prophylactic approach for the CID with loperamide. A 72-yr-old male patient with stage IV non-small-cell lung cancer developed diarrhea repeatedly after docetaxel-cisplatin chemotherapy. His diarrhea persisted despite treatment including loperamide and fasting. However, the diarrhea was successfully prevented when loperamide was given before and after the chemotherapy. To our knowledge, this is the first report of prophylactic approach for the CID with loperamide.
Aged ; Carcinoma, Non-Small-Cell Lung/*drug therapy/radiography ; Cisplatin/therapeutic use ; Diarrhea/chemically induced/*etiology ; Drug Therapy, Combination ; Humans ; Loperamide/*adverse effects/therapeutic use ; Lung Neoplasms/*drug therapy/radiography ; Male ; Neoplasm Staging ; Taxoids/*adverse effects/therapeutic use ; Tomography, X-Ray Computed

BACKGROUND: Octamer-4 (Oct4), a transcriptional factor involved in regulating embryonic stem cells, may play a role in tumorigenesis. Since little is known about the role of Oct4 as a prognostic factor for squamous cell carcinoma (SCC) of lung, we investigated its expression in SCC tissue and its clinicopathologic significance. METHODS: Formalin-fixed, paraffin embedded tissues from 79 patients, including 44 complete resections and 35 biopsies, obtained from 1995 to 2008 were immunostained for Oct4, scored, and scores correlated with clinicopathologic parameters and survival. RESULTS: Oct4 expression in tumors was significantly associated with peripheral location (vs central location) (p = 0.004) and pleural invasion (p = 0.018). In 44 complete resections, survival analysis revealed that Oct4 expression and increased stage (II and III vs I) were significantly associated with worse survival in univariate analysis (p = 0.005 and p = 0.009, respectively) and in multivariate analysis (p = 0.024 and p = 0.033, respectively). CONCLUSIONS: The expression of Oct4 and high stage in SCC of lung are significant predictors of a poor prognosis and diminished overall survival.
Biopsy ; Carcinoma, Squamous Cell ; Cell Transformation, Neoplastic ; Embryonic Stem Cells ; Humans ; Immunohistochemistry ; Lung ; Multivariate Analysis ; Paraffin ; Prognosis

Dentinogenic ghost cell tumor (DGCT) is an uncommon odontogenic tumor. It is characterized by islands of odontogenic epithelial cells that contain numerous ghost cells and dysplastic dentin. Occasionally, DGCT combines with other odontogenic tumors, such as ameloblastoma. We report here on a 21-year-old female who complained of a tender solid mass in the left maxilla for the 7 month previous to her admission. MRI revealed a relatively well demarcated mass in the left maxilla with heterogenous signal intensity, measuring 3.2 x 2.8 cm, and this mass had invaded the left palate. Microscopically, the tumor was composed of nests of odontogenic epithelium that contained ghost cells and calcification with dysplastic dentin, which is all consistent with DGCT. Localized area showed odontogenic epithelial follicles that had peripheral palisading and satellite reticulum without ghost cells and dentin, and this is consistent with ame- loblastoma. The immunohistochemistry revealed cytokeratins, EMA, S100 and Bcl-2 positivity in areas of the DGCT and ameloblastoma. In the ameloblastoma, Bcl-2 positivity was noted in the palisading basal cells. We concluded that the tumor was an ameloblastoma associated with DGCT.
Ameloblastoma* ; Dentin ; Epithelial Cells ; Epithelium ; Female ; Humans ; Immunohistochemistry ; Islands ; Keratins ; Magnetic Resonance Imaging ; Maxilla ; Odontogenic Tumors ; Palate ; Reticulum ; Young Adult