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

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.

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

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

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

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

BACKGROUND: The cytological examination of cerebrospinal fluid (CSF) using conventional cytology with a cytocentrifuge (cytospin) is an important method for evaluating the involvement of leukemia in the CNS. Liquid-based cytology (LBC) is now a widely used cytological method not only for gynecological and non-gynecological specimens, but its application to CSF for the identification of leukemic cell has not yet been reported. In this study, we tried to compare conventional cytology with using a cytospin with LBC and Papanicolaou (Pap) staining. We also examined the modified LBC with Wright staining to assess whether this modified method can be useful for diagnosing Leukemia. METHODS: We studied 30 cases of CSF that were obtained from 16 patients, including 17 cases of acute myeloid leukemia, 12 cases of acute lymphoblastic leukemia and 1 case of diffuse large B cell lymphoma. We applied conventional cytology with a cytocentrifuge (cytospin), LBC with Pap staining and modified LBC with Wright staining. RESULTS: The morphological features of the LBC with Pap staining showed difficulty for interpretation when compared with conventional cytology with a cytospin, and mainly because of cellular shrinkage. The modified LBC with Wright staining showed good morphological features. CONCLUSIONS: We suggest that modified LBC with Wright staining may be useful for examining CSF.
Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Lymphoma, B-Cell ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

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