Many pathological surveys of brain tissue in patients with intractable epilepsy have been reported. There have been, however, few studies focused on the differences between childhood and adults in pathological alterations of brain. We retrospectively analyzed histopathology of 164 lobectomy specimens for intractable epilepsy in view of the differences between children and adults. Among 164 cases, 28 cases were children (less than 15 years) and 136 cases adults. We compared frequency of histopathologic features, distribution of involved cortex (temporal or extratemporal lobe), previous injury histories, such as brain trauma, encephalitis or febrile seizure, and coexistence of other lesions (dual pathology) between two groups. Pathologic alterations were encountered in 92% of 164 patients. In children focal cortical dysplasia (n=16, 57.1%), neoplasm (n=8, 28.6%), hippocampal sclerosis (n=6, 21.4%), cortical tuber (n=1, 3.6%), leukomalacia (n=1, 3.6%), and Rasmussen's encephalitis (n=1, 3.6%) were observed, whereas focal cortical dysplasia (n=81, 59.6%), hippocampal sclerosis (n=80, 58.8%), neoplasm (n=19, 14%), and cerebral cysticercosis (n=3, 2.2%) were found in adults. Pediatric patients had a higher proportion of severe focal cortical dysplasia (17.9% in children, 0.7% in adults). Neoplasia and extratemporal lobe involvement were more commonly found in children (28.6%, 50%) than in adults (14.0%, 24.3%), whereas hippocampal sclerosis and dual pathology were more common in adults (58.8%, 44.9%) than in children (21.4%, 17.9%). Previous injury history was statistically significant in patients with hippocampal sclerosis, and lent support to the hypothesis that hippocampal sclerosis is related with acquired lesions. Incidence of focal cortical dysplasia was nearly similar in both adult (59.6%) and pediatric groups (57.1%), and supported the hypothesis that focal cortical dysplasia is developmental abnormality occurring during a prenatal period.
Adult* ; Brain ; Brain Injuries ; Child* ; Cysticercosis ; Encephalitis ; Epilepsy ; Epilepsy, Temporal Lobe ; Humans ; Incidence ; Malformations of Cortical Development ; Pathology ; Retrospective Studies ; Sclerosis ; Seizures* ; Seizures, Febrile

Primary intrasellar schwannomas or neurilemmomas occur rarely and mimic pituitary adenoma, radiologically and clinically. The authors describe the 6th case of primary intrasellar schwannoma mimicking a nonfunctioning pituitary macroadenoma, clinically as well as radiologically. Light microscopically, the present case did not show the typical histology of conventional schwannoma and the confirmative diagnosis was made with the aid of immunohistochemistry and electron microscope. Here, we review the possible hypotheses for pathogenesis of sellar schwannomas on unusual locations.
Diagnosis ; Immunohistochemistry ; Neurilemmoma* ; Pituitary Neoplasms

We report a first Korean case of presumably dominantly inherited primary tubular aggregate myopathy in a 19-yr-old man, who presented with slowly progressive proximal muscle stiffness and weakness. In hematoxylin and eosin stain, it showed subsarcolemmal, or central pale basophilic granular vacuoles, which stained red with modified Gomori's trichrome and intensive blue with nicotinamide adenonine dinucleotide-tetrazolium reductase, respectively. Ultrastructurally, aggregates of 60 nm-sized hexagonal tubules were found in both type 1 and type 2 fibers. We briefly review the pathologic findings of the previously reported cases of tubular aggregate myopathy and discuss the possible pathogenesis of this disease. We briefly discuss the possible pathogenesis of sarcoplasmic reticulum and review the ultrastructural characteristics.
Adult ; Biopsy ; Frozen Sections ; Genes, Dominant ; Genes, Recessive ; Human ; Korea ; Male ; Microscopy, Electron ; Microtubules/ultrastructure ; Mitochondria, Muscle/ultrastructure ; Muscle, Skeletal/pathology* ; Myopathies, Structural, Congenital/diagnosis ; Myopathies, Structural, Congenital/genetics ; Myopathies, Structural, Congenital/pathology* ; Pedigree

Angiomyolipoma is the most common mesenchymal tumor of the kidney. It occurs sporadically and is associated with tuberous sclerosis. It can appear in any organs, but most commonly in the kidney, and it appears slightly more in females. Angiomyolipoma is pathologically composed of three heterogeneous components of blood vessels, smooth muscle cells and fat cells of varying proportion, which occasionally make several unusual histologic variants. We describe a variant of renal angiomyolipoma simulating vascular leiomyoma on routine hematoxylin-eosin stain; prominent thick-walled blood vessels interspersed with proliferation of smooth muscle cells and total absence of fat cells. Perivascular spindle-shaped smooth muscle cells were reactive for smooth muscle actin, desmin and HMB-45 immunostains. This case illustrates vascular leiomyoma-like angiomyolipoma, which was finally diagnosed on the basis of HMB-45 immunostain, and also raises a question about the real existence of renal vascular leiomyoma.
Actins ; Adipocytes ; Angiomyolipoma* ; Angiomyoma ; Blood Vessels ; Desmin ; Female ; Humans ; Kidney ; Kidney Neoplasms ; Muscle, Smooth ; Myocytes, Smooth Muscle ; Tuberous Sclerosis

We herein report a case of intractable epilepsy that occurred in a 7-year-old girl, which is consistent with radiological and clinicopathological hallmarks of Rasmussen's encephalitis. The patient showed characteristic primary unilateral involvement with secondary bilateral propagation. Microscopically, the cortical atrophy due to neuronal loss, intense GFAP-immunoreactive astrogliosis, neuronophagia, perivascular lymphocytic infiltration and microglial nodules was seen throughout the cortex and white matter. No viral inclusions were noted; no cytomegalovirus, herpes simplex virus or Epstein-Barr virus was found by in situ hybridization. Granular immunofluorescence for C4, C1q and IgG within the blood vessel walls was noted, and ultrastructurally, only nonspecific vascular injury was found. Rasmussen's encephalitis is a diagnosis of exclusion; it can be diagnosed by the combination of clinical manifestation, neuroimaging and characteristic pathologic features.
Atrophy ; Blood Vessels ; Child ; Cytomegalovirus ; Diagnosis ; Encephalitis* ; Epilepsy ; Female ; Fluorescent Antibody Technique ; Herpesvirus 4, Human ; Humans ; Immunoglobulin G ; Immunohistochemistry ; In Situ Hybridization ; Microscopy, Electron ; Neuroimaging ; Neurons ; Simplexvirus ; Vascular System Injuries

BACKGROUND: Hepatocyte growth factor (HGF) is a potent mitogenic cytokine. C-met protein, which is known to be the HGF receptor has transmembrane tyrosine kinase activity and is encoded by the c-met oncogene. The HGF/c-met signaling pathway may play various roles in the carcinogenesis of various organs. METHODS: We examined HGF and c-met mRNA expression by utilizing reverse transcription polymerase chain reaction on 40 surgically resected intracranial meningiomas (25 benign, 10 atypical, and 5 anaplastic cases). RESULTS: An HGF overexpression was detected in 28%, 50%, and 80% of the benign, atypical and anaplastic meningiomas, respectively; a high expression of HGF or the coexpression of HGF/c-met was detected in the high grade meningiomas (the atypical and anaplastic cases, p=0.046, p=0.014). An HGF expression was statistically significant in the recurrent meningiomas (p=0.003), and HGF expression was significantly lower than c-met mRNA expression in benign meningiomas (p=0.034). CONCLUSIONS: There was no correlation between histologic subtypes and HGF/c-met expression. Determination of HGF expression can be used as a molecular predictor for recurrence of meningioimas. These results suggest that HGF and c-met expression in meningiomas may be associated with anaplastic progression.
Hepatocyte Growth Factor ; Hepatocytes ; Meningioma ; Neoplasm Recurrence, Local ; Oncogenes ; Polymerase Chain Reaction ; Protein-Tyrosine Kinases ; Proto-Oncogene Proteins c-met ; Recurrence ; Reverse Transcription ; RNA, Messenger

Here we report the first Korean case of a girl who developed noninvasive squamous cell carcinoma of the vulva at the age of 16 years. She was taking tacrolimus, an immunosuppressive agent, after living-related liver transplantation. The vulvar masses were microscopically proved as vulvar intraepithelial neoplasm II and III, even squamous cell carcinoma in situ. Human papillomavirus subtypes (69 and 73) and human papillomavirus types (66, 70, 73, and 43) were detected in the vulvar mass and the cervicovaginal smear, respectively. The outcome of liver transplantation for children has been markedly improved during the last several decades. However, the present case highlights the need to perform periodic genital examinations for the adolescents after liver transplantation. In addition to the high risk and probable high subtypes, uncommonly found human papillomavirus subtypes were extracted from her vulvar cancer. The present case is the first to show the possible relationship between previously unknown and uncommon human papillomavirus subtypes and pediatric post-transplant vulvar squamous cell carcinoma. More attention should be paid to the vulvar and cervical surveillance of pediatric transplant recipients by both medical specialists and general physicians.
Adolescent ; Carcinoma in Situ ; Carcinoma, Squamous Cell ; Child ; Humans ; Liver ; Liver Transplantation ; Specialization ; Tacrolimus ; Vulva ; Vulvar Neoplasms

Chronic inflammation due to a Helicobacter pylori (H. pylori) infection is a representative cause of gastric cancer that can promote gastric carcinogenesis by abnormally activating immune cells and increasing the inflammatory cytokines levels. H. pylori infections directly cause DNA double-strand breaks in gastric epithelial cells and genetic damage by increasing the enzymatic activity of cytidine deaminase. Eventually, gastric cancer is induced through dysplasia. Hypermethylation of tumor suppressor genes is an important cause of gastric cancer because of a H. pylori infection. In addition, the changes in gastric microbiota and the mucosal inflammatory changes associated with a co-infection with the Epstein-Barr virus are associated with gastric cancer development. DNA damage induced by H. pylori and the subsequent responses of gastric stem cells have implications for gastric carcinogenesis. Although the pathogenesis of H. pylori has been established, many uncertainties remain, requiring more study.

Sertoli-Leydig tumors tend to relapse early and due to their rarity, limited data are available regarding a role of chemotherapy in the management of Sertoli-Leydig cell tumors. We present a case of recurrent ovarian Sertoli-Leydig cell tumor whose salvage treatment was successful with paclitaxel and carboplatin chemotherapy.
Carboplatin ; Female ; Ovary ; Paclitaxel ; Recurrence ; Salvage Therapy ; Sertoli-Leydig Cell Tumor

BACKGROUND: Robotic surgery is an alternative to minimally invasive surgery. The aim of this study was to report on current trends in robotic thoracic and cardiovascular surgical techniques in Korea. METHODS: Data from the National Evidence-based Healthcare Collaborating Agency (NECA) between January 2006 and June 2012 were used in this study, including a total of 932 cases of robotic surgeries reported to NECA. The annual trends in the case volume, indications for robotic surgery, and distribution by hospitals and surgeons were analyzed in this study. RESULTS: Of the 932 cases, 591 (63%) were thoracic operations and 340 (37%) were cardiac operations. The case number increased explosively in 2007 and 2008. However, the rate of increase regained a steady state after 2011. The main indications for robotic thoracic surgery were pulmonary disease (n=271, 46%), esophageal disease (n=199, 34%), and mediastinal disease (n=117, 20%). The main indications for robotic cardiac surgery were valvular heart disease (n=228, 67%), atrial septal defect (n=79, 23%), and cardiac myxoma (n=27, 8%). Robotic thoracic and cardiovascular surgeries were performed in 19 hospitals. Three large volume hospitals performed 94% of the case volume of robotic cardiac surgery and 74% of robotic thoracic surgery. Centralization of robotic operation was significantly (p<0.0001) more common in cardiac surgery than in thoracic surgery. A total of 39 surgeons performed robotic surgeries. However, only 27% of cardiac surgeons and 23% of thoracic surgeons performed more than 10 cases of robotic surgery. CONCLUSION: Trend analysis of robotic and cardiovascular operations demonstrated a gradual increase in the surgical volume in Korea. Meanwhile, centralization of surgical cases toward specific surgeons in specific hospitals was observed.
Adenosine-5'-(N-ethylcarboxamide) ; Delivery of Health Care ; Esophageal Diseases ; Heart Septal Defects, Atrial ; Heart Valve Diseases ; Korea* ; Lung Diseases ; Mediastinal Diseases ; Myxoma ; Robotics ; Surgical Procedures, Minimally Invasive ; Thoracic Surgery