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

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

Paragangliomas (PGs) of the thyroid are very rare tumors. Based on the medical literature, thyroid PGs invariably affected women and they were always thought to be benign, and they have always been mistaken for other more common lesions. We report here on the first case of PG of the thyroid in a 45-year-old man in Korea. Ultrasonographically, the tumor showed hypoechoic features. Microscopically, the tumor showed the typical zellballen pattern. The small to medium-sized tumor cells contained moderate amounts of finely granular eosinophilic cytoplasm and round to oval nuclei with fine chromatin. The tumor invaded the thyroid capsule and the extrathyroidal tissue with vascular and perineural invasion. Immunohistochemically, the tumor showed positivity for chromogranin, S-100 protein, CD56a, and synaptophysin and negativity for calcitonin, thyroglobulin, galectin-3, p53, CK19, and EMA. The Ki-67 labeling Index was 10%. We concluded that our case has a high potential of metastasis, and a close follow up would be important.
Female ; Male ; Humans

Although chronic myelogenous leukemia (CML) may be involved in any part of the body, infiltration of the body fluid has rarely reported in the literature. Here we report on a 35 year-old male patient who was diagnosed chronic myelogenous leukemia ten years previously and he received allogenic hematopoietic stem cell transplantation. He then presented with left knee pain eight years after the initial diagnosis. MRI revealed a soft tissue mass at the distal femur. Cytology of the joint fluid revealed myeloblasts, promyelocytes, eosinophilic myelocytes, band neutrophils, megakaryocytes and orthochromatic erythroblasts, which was all consistent with leukemic infiltration of the knee joint fluid. The immunohistochemistry was positive for CD34, CD117 and myeloperoxidase (MPO). Despite that the patient underwent radiation therapy, MRI revealed growth of the mass, and ten months later, the lymphoid blast phase of CML was confirmed after biopsy. The patient received an above knee amputation. Five months later, multiple masses were revealed on PET-CT at the left iliopsoas muscle, abdominal wall and bones. Bilateral pleural effusion occurred shortly after this. Cytologic evaluation of the pleural fluid also revealed blast-like cells, and histologic evaluation of the abdominal mass confirmed the lymphoid blast phase of CML with positivity for CD3, UCHL-1, CD34 and CD117, but negativity for MPO.
Abdominal Muscles ; Amputation ; Biopsy ; Blast Crisis ; Body Fluids ; Eosinophils ; Erythroblasts ; Femur ; Granulocyte Precursor Cells ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunohistochemistry ; Joints ; Knee ; Knee Joint ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemic Infiltration ; Male ; Megakaryocytes ; Neutrophils ; Peroxidase ; Pleural Effusion