Fibrous hamartoma of infancy is a distinct clinicopathologic entity with unique microscopic findings and benign clinical course. This tumor is composed of intervening dense fibrocollagenous trabeculae, well defined mucoid areas and varying amounts of mature fat, which are arranged in organoid growth pattern. We report three typical cases of this tumor which were experienced during the recent two years at the Seoul National University Hospital. It is the first description on this tumor in Korean literature. Case 1 was a 10 month old boy who was admitted due to a mass in the right infra clavicular area for 6 months. The mass showed irregular and poorly circumscribed outer surface. Case 2 was a one month old girl who was presented with a diffuse ill defined hairy mass in the left buttock since birth. Case 3 was a 15 month old boy who was brought to the hospital due to well circumscribed scrotal mass fot 8 months. Microscopically all three cases showed very similar histological features. And all 3 cases are well after the removal of the tumors.
Hamartoma

No abstract available.
Mosaicism* ; Trisomy*

An ovarian sex cord tumor with annular tubules(SCTAT) in an 18-year-old woman who had secondary amenorrhea but not Peutz-Jeghers syndrome was studied by light and electron microscopy. Her clinical features were consistent with those of Turner's syndrome, i.e, shortness of stature, webbing of the neck, scanty or absent axillary and pubic hairs, lower hair line with streak gonad. Chromosomal analysis revealed 45, X/46, XX karyotype. The patient has been well and disease free for a period of 2 years after surgical removal of tumor. Histologically, the tumor was composed of nests of cells arranged in simple and complex annular tubules with central acidophilic hyaline bodies. Ultrastructurally, the cells had deeply indented nuclei and the cells were joined by specialized intercellular junctions along their lateral adjacent borders. Central acidophilic hyaline body consisted of concentric lamellae of basal lamina, but Charcot-Boettcher filaments were not observed.
Female ; Humans

During the last decade, many clinical investigators at various cancer centers have reported the efficacy of various chemotherapeutic agents in the treatment of osteosarcoma. The regimens using high-dose methotrexate (HDMTX) with citrovorum factor rescue are now considered to be one of the most effective treatments of choice. From December 1989 to May 1991, sixteen patients with Enneking's stage (Enneking et al., 1980) IIB osteosarcoma of the extremities were treated with a high-dose methotrexate regimen. After two cycles of preoperative chemotherapy, an operation was performed; either limb salvage or amputation. The resected lesions were examined pathologically and classified according to Huvos' criteria. On pathological examination, 8 (50%) cases showed Grade IV; 1 (6.25%) Grade III; 4 (25%) Grade II; and 3 (18.75%) Grade I. The types of surgery performed were tumor prosthesis replacement (11); wide resection with or without reconstruction (2); resection and arthrodesis (1); and amputation (2).
Adolescent ; Adult ; Bone Neoplasms/*drug therapy/pathology/surgery ; Child ; Combined Modality Therapy ; Female ; Humans ; Male ; Methotrexate/administration & dosage/*therapeutic use ; Osteosarcoma/*drug therapy/pathology/surgery

For the study the effects of X -ray irradiation on the development of lens and interactions between optic vesicle and lens, 200 rads X -ray irradiated to the preganat rats (Sprague -Dawley strain) on gestation day 9.5. The rats were sacrificed daily from the gestation day 10.5 to 17.5, and investigated the development of lens with light microscope. The results were as follows; 1. The survival rate of X -irradiated rat fetuses was decreased significantly compared with that of the control group. 2. The mean body weight of the X -irradiated rat fetuses was decreased significantly compared with that of the control group. 3. The congenital anomalies of the eye in the rat fetuses after X -irradiation were anophthalmia, congenital aphakia, lens aplasia, lens luxation, and coloboma of the retina. 4. The critical period of lens development of the Sprague -Dawley rats was considered from GD 9. 5 to GD 10.0. 5. In the X -irradiated rat fetuses on gestation day 9.5, optic vesicle did not developed, but lens developed independently , and moved deeply toward the forebrain vesicle. According to above results, it could be concluded that the development of lens from surface ectoderm might be independent from the inductive influence of optic vesicle, and the movement of lens toward the forebrain vesicle could be mediated by chemotactic factors secreted from the brain vesicle. Further studies are needed to reveal the mechanisms of lens development and interaction between lens and brain vesicle.
Animals ; Anophthalmos ; Aphakia ; Body Weight ; Brain ; Chemotactic Factors ; Coloboma ; Critical Period (Psychology) ; Ectoderm ; Fetus ; Pregnancy ; Prosencephalon ; Rats ; Retina ; Survival Rate

This paper presents an unusual solid mesenchymal hamartoma of the liver (MHL) in adult. A well defined solid mass in the left lobe of the liver was found in a 57-year-old female. Preoperative radiologic examinations demonstrated solid mass with multifocal calcifications abutting the gallbladder. By light microscopy, the lesion was composed of dense fibrous stroma with hyalinization, bile ducts and thick-walled vessels without hepatocytes. The solid and hyalinized mesenchymal component would suggest an unusual degenerative change representing a burnt-out MHL.
Case Report ; Female ; Hamartoma/surgery ; Hamartoma/radiography ; Hamartoma/pathology* ; Human ; Liver Neoplasms/surgery ; Liver Neoplasms/radiography ; Liver Neoplasms/pathology* ; Mesoderm/pathology ; Middle Age ; Tomography, X-Ray Computed

PURPOSE: The education of the patients is an effective component of comprehensive care, especially in patients with epilepsy. We developed an epilepsy educational program designed to improve the knowledge and understanding of the natural clinical course, the treatment, and prognosis of epilepsy. We evaluated the efficacy of this educational program to epilepsy patient's knowledge of their disorder and quality of life. METHODS: We studied 224 patients with epilepsy from Dongsan Epilepsy Center. All patients completed the Knowledge and Quality of Life in Epilepsy-31 (QOLIE-31) questionnaire before a one-day educational program was completed. Six months after the educational program was given, patients completed the same questionnaire. The results of the two surveys were compared. RESULTS: In the initial questionnaire, about 60% of the patients thought that epilepsy is not related with head trauma. About 40% of the patients thought that epilepsy is a genetic disorder. Over 80% of the patients thought that behavior changes are not related to epilepsy. Over 90% of the patients had correct responses about taking antiepileptic drugs. However, only 60% of the patients had their ideas regarding proper social activity correct. After completing the educational course, the results of the follow-up questionnaires were markedly improved concerning the patients' knowledge of epilepsy. In contrast, the quality of life measures changed a little. CONCLUSION: An epilepsy educational program for patients improves their knowledge of epilepsy. This study was performed with only one education workshop, which is not sufficient to improve the quality of life measures. A well-regulated, systematic educational program is needed to efficiently improve the lives of epileptic patients.
Anticonvulsants ; Craniocerebral Trauma ; Education* ; Epilepsy* ; Follow-Up Studies ; Humans ; Prognosis ; Quality of Life* ; Surveys and Questionnaires

PURPOSE: To evaluate the role of enhanced MR imaging in monitoring tumor response to preoperativechemotherapy for osteosarcomas. MATERIALS AND METHODS: Fo r t y - s even patients (30 males and 17 females, witha mean age 17 years ; range 8 -44 years) with osteosarcomas were included in this study. We obtained spin echoT1-, T2-, and enhanced T1-weighted images before and after pre-operative chemotherapy and in all patientscorrelated changes in MR parameters with histopathologic response. We also obtained 19 specimen MR images,correlating these with histopathologic results in order to estimate tissue specific signals. Patients with morethan 10% viable tumor in the resected specimen were considered poor respon-ders(n=26), while those with 10% orless viable tumor were considered good respon-ders(n=21). RESULTS: Four distinct patterns of signal intensitycorresponded, respectively to dead bone and dense fibrosis (low on T1- and T2-weighted images), viable tumor cells(in-termediate on T1- and high on T2-weighted images), necrosis (low on T1- and high on T 2 - weighted images),and hemorrhage (high on T1- and T2-weighted images), but a wide range of overlap was noted. In all four groups,viable tumor cells remained. Increased tumor vo l u m e, stable or increased edema and enhancement were goodpre-dictors of poor response (predictive values of 83%, 77%, and 89%, respectively). Decreased enhancement was theonly reliable predictor of good response (predictive value, 73%). Changes in tumor margin, homogeneity, signalintensity, and joint effu-sion did not correlate with histopathologic response. CONCLUSION: Signal intensities donot reflect histologic nature. Enhanced MR imaging is a useful predictor of tumor response to preoperativechemotherapy.
Drug Therapy* ; Edema ; Female ; Fibrosis ; Hemorrhage ; Humans ; Joints ; Magnetic Resonance Imaging* ; Male ; Necrosis ; Osteosarcoma*

This study is performed to reveal the changes of the arterial wall, especially, tunica intima and tunica media, after endothelial denudation and the effects of dexamethasone sodium on intimal hyperplasia morphologically in the rat. After arterial denudation by modified air drying technique, dexamethasone 1, 200 mg/kg/day was administered intramuscularly daily from the day of operation for 14 days. At 5 DAT (days after treatment) and 14 DAT, tunica intima was greatly thickened in control groups compared with normal group, but not in the dexamethasone-treated groups. Light microscopically, greatly increased cells and intercellular matrix in the tunica intima are observed in control group, but not in the dexamethasone-treated group. In the TEM observation, the cells considered as myofibroblasts and extracellular matrix were greatly increased in both tunica intima and tunica media just below the internal elastic lamina in the control group. Myofibroblasts and extracellular matrix migrated through the apertures of internal elastic lamina into the endothelial layer. Characteristic false internal elastic lamina also found. In dexamethasone-treated group, myofibroblasts and extracellular matrix decreased significantly, and apoptotic electron-dense cells, fragmented nucleus and autophagic vacuoles are observed. Through the apertures of internal elastic lamina, comma-shaped fragmented nuclei migrated into the tunica intima. These results suggest that dexamethasone inhibits the myofibroblast-transformation and proliferation of smooth muscle cells, migration of myofibroblasts and matrix synthetic activity, and induces the apoptosis of smooth muscle cells under the internal elastic lamina.
Animals ; Apoptosis ; Dexamethasone* ; Extracellular Matrix ; Hyperplasia* ; Myocytes, Smooth Muscle ; Myofibroblasts ; Rats ; Sodium ; Tunica Intima ; Tunica Media ; Vacuoles

Inflammatory pseudotumor is an uncommon nonneoplastic lesion of unknown etiology that occurs most commonly in the lung. However, it also occurs in diverse extrapulmonary locations: abdomen, retroperitoneum, pelvis, heart, head and neck, upper respiratory tract, trunk, bladder, and extremities. The extrapulmonary inflammatory pseudotumor is often larger, less-well circumscribed and multinodular. Proximity of the tumor to vital structures or involvement of vital organs compromises the opportunity for complete resection; thus higher recurrence rates are often reported even after surgical treatment. The authors report a case of inflammatory pseudotumor originating from common carotid artery in a 42-year-old female patient with a rapidly growing neck mass, treated by en bloc resection of inflammatory pseudotumor and a long segment of common carotid artery followed by polytetrafluoroethylene(PTFE) graft interposition.
Abdomen ; Adult ; Carotid Arteries* ; Carotid Artery, Common ; Extremities ; Female ; Granuloma, Plasma Cell* ; Head ; Heart ; Humans ; Lung ; Neck ; Pelvis ; Recurrence ; Respiratory System ; Transplants ; Urinary Bladder