Purpose: Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. Materials and Methods: A search of medical records from seven centers was performed between January 2005 and December 2016. Results: Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). Conclusion Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

Purpose: Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. Materials and Methods: A search of medical records from seven centers was performed between January 2005 and December 2016. Results: Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). Conclusion Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea.
ACTH Syndrome, Ectopic ; Adrenalectomy ; Adrenocorticotropic Hormone* ; Carcinoid Tumor ; Cushing Syndrome ; Humans ; Hypertension ; Korea ; Lymph Node Excision ; Lymph Nodes ; Male ; Metastasectomy ; Middle Aged ; Obesity, Abdominal ; Small Cell Lung Carcinoma ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

Compressive femoral and lateral femoral cutaneous neuropathies from an iliacus hematoma are unusual presentation. We report a case of a 16-year-old boy who developed right femoral and lateral femoral cutaneous neuropathies as a complication of traumatic ipsilateral iliacus hematoma formation. The patient complained of numbness in the right thigh and calf as well as right leg weakness, and pain in the right inguinal area. Nerve conduction study and needle electromyography identified the neuropathies. After the electrodiagnostic studies, the pelvic bone MRI revealed a large, 9x5x4.5 cm right iliacus hematoma. As a result, diagnosis of a right iliacus hematoma compressing the femoral and lateral femoral cutaneous nerves was made, and the patient underwent an operation to remove the hematoma. Symptoms and neurological signs showed notable improvement after surgical decompression. Subsequent follow-up electrodiagnostic studies after 11 weeks demonstrated regeneration evidence.
Adolescent ; Decompression, Surgical ; Electromyography ; Femoral Neuropathy ; Follow-Up Studies ; Hematoma ; Humans ; Hypesthesia ; Leg ; Needles ; Nerve Compression Syndromes ; Neural Conduction ; Pelvic Bones ; Regeneration ; Thigh

When forensic anthropologists tried to reconstruct the biological profile, they would rely on the statistical data of documented human osteological/anatomical specimens. However, the metric data of Korean ancestry which is suitable for determining sex has not been established because documented human skeletal specimens are lack in Korean. The aim of this study is to discuss the possibility of determination of sex by metric data of atlas in Koreans. We measured 43 (male 25 and female 18) atlas from the department of anatomy, College of Medicine, Soonchunhyang University and the department of anatomy, Yonsei University College of Medicine. We measured 21 measurements of atlas using digimatic caliper and performed statistic analysis using SPSS to establish the discriminant functions. Among 21 measurements of atlas, the measurement values of the width of atlas exhibited the strongest relation with sexes. The accuracy of arbitrary discriminant functions derived from 6 measurements exhibited 93.9% and that of stepwise discriminant functions derived from 3 measurements exhibited 97.0%. In conclusion, this study could indicate that metric data of atlas is helpful to determine the sex in Koreans, but a further investigation should be followed.
Female ; Humans

Pancreatic tumors in children are very rare but have a better prognosis compared with that in adult. Pediatric pancreatic tumors are more often benign and easier to resect. To evaluate the characteristics and prognosis, the records of 13 patients who underwent pancreatic resection, from June 1997 to May 2005, at Samsung Medical Center were reviewed. The mean follow up period was 48 months. The male to female ratio was 1: 1.6. Mean age was 10.3 years. Signs and symptoms included abdominal pain (7), abdominal palpable mass (5), jaundice (1), hypoglycemic (1), and non-specific GI symptoms (4). The commonly used diagnostic tools were CT and abdominal sonography. In addition, MRI, ERCP, EEG, and hormone test were also done when indicated. Surgical procedures included distal pancreatectomy (5), pylorus preserving pancreaticoduodenectomy (4), tumor excision (3), and subtotal pancreatectomy (1). Locations of lesions in pancreas were head (4), tail (5), and body and tail (4). Postoperative complications developed in 3 cases; postoperative ileus (1), wound problem (1), and pancreatitis (1). The pathologic diagnosis included solid-pseudopapillary tumor (6), congenital simple cyst (1), pancreatic duplication cyst (1), serous oligocystic adenoma (1), mucinous cystadenocarcinoma (1), rhabdomyosarcoma (1), insulinoma (1), and pancreatoblastoma (1). Three cases received adjuvant chemotherapy and radiotherapy. Overall survival rate was 81%. One patient with a mucinous cystadenocarcinoma died. In this study, pancreatic tumors in children were resectable in all patients and had good survival. Surgery of pancreatic tumors should be regarded as the gold standard of treatment and a good prognosis can be anticipated in most cases of benign and malignant tumors.
Abdominal Pain ; Adenoma ; Adult ; Chemotherapy, Adjuvant ; Child* ; Cholangiopancreatography, Endoscopic Retrograde ; Cystadenocarcinoma, Mucinous ; Diagnosis ; Electroencephalography ; Female ; Follow-Up Studies ; Head ; Humans ; Ileus ; Insulinoma ; Jaundice ; Magnetic Resonance Imaging ; Male ; Pancreas ; Pancreatectomy ; Pancreaticoduodenectomy ; Pancreatitis ; Postoperative Complications ; Prognosis ; Pylorus ; Radiotherapy ; Rhabdomyosarcoma ; Survival Rate ; Wounds and Injuries

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors?in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multi- modality therapeutic approaches might be important for better outcome.
Adenoma ; Biopsy ; Carcinoma, Hepatocellular ; Child* ; Diagnosis ; Early Diagnosis ; Female ; Focal Nodular Hyperplasia ; Hair ; Hamartoma ; Hemangioendothelioma ; Hemangiosarcoma ; Hepatoblastoma ; Humans ; Jaundice ; Liver Transplantation ; Liver* ; Male ; Medical Records ; Rhabdoid Tumor ; Survival Rate ; Vomiting ; Weight Loss

PURPOSE: Bone marrow contains many kinds of primitive cells and endothelial progenitor cells that secrete several growth factors. We hypothesized that angiogenesis could be induced by autogenous whole bone marrow stem cell implantation in an animal ischemic limb. METHOD: A chronic ischemic hind limb model was created by encircling the femoral artery with an ameroid constrictor (2 mm inner diameter) in a dog model. About 20 ml of autogenous whole bone marrow stem cells were aspirated from the femur and then injected into ischemic limb muscles. Contralateral limbs injected with 20 ml of normal saline as controls. To assess angiogenic effects, an angiogram and a histologic evaluation were performed at 8 weeks after bone marrow stem cell implantation. RESULT: Imaging analysis of angiograms showed that newly developed capillaries were significantly more plentiful in treated limbs. Mean capillary density in the treated limb group was significantly greater than that in the control group (151+/-11.7 vs 81.5+/-7.2 cap/mm2, respectively, P<0.05), and the proportion of larger diameter (Fig. 6) newly developed capillaries was significantly higher in treated limbs than in control limbs. CONCLUSION: These findings indicated that autogenous whole bone marrow stem cell implantation increases the efficiency of angiogenesis.
Animals* ; Bone Marrow* ; Capillaries ; Dogs ; Extremities ; Femoral Artery ; Femur ; Intercellular Signaling Peptides and Proteins ; Models, Animal* ; Muscles ; Stem Cells*

PURPOSE: Helicobacter pylori (H. pylori) infection has been known to be vital in the pathogenesis of duodenal ulcer disease in children as well as in adults. But the relationship between H. pylori infection and the histopathologic findings of the duodenum has not been explained obviously in children yet. So the aim of this study is to determine whether duodenitis and/or gastric metaplasia in the duodenum increases the risk of duodenal ulcer disease in children infected by H. pylori. METHODS: From October 2001 to April 2004 gastric and duodenal biopsies were performed in 177 children who visited Department of Pediatrics, Gil Hospital, Gachon Medical School. Biopsy sections were stained with hematoxylin and eosin and also with Giemsa for identification of H. pylori. The grades of duodenitis and gastric metaplasia were classified from 0 to 3 and from 0 to 4, respectively. RESULTS: The incidence of H. pylori infection was 54% in total patients. Amongst 163 children with duodenitis there was a lack of correlation between H. pylori infection and the grade of duodenitis. Amongst 11 patients with duodenal ucler, only 4 children were infected by H. pylori. And amongst 5 patients with gastric metaplasia, H. pylori and duodenal ulcer were detected in 2 and 3 children, respectively. The occurrence of duodenal ulcer and gastric metaplasia were increased significantly in proportion to the grade of duodenitis (p<0.0001 and p=0.0365, respectively). CONCLUSION: As opposed to the results of previously reported articles, there were lacks of correlation between H. pylori infection and duodenitis, duodenal ulcer, and gastric metaplasia. So further study hould be done to clarify the effect of H. pylori on the duodenal histopathology in children infected by H. pylori.
Adult ; Biopsy ; Child* ; Duodenal Ulcer* ; Duodenitis* ; Duodenum ; Eosine Yellowish-(YS) ; Helicobacter pylori* ; Helicobacter* ; Hematoxylin ; Humans ; Incidence ; Metaplasia* ; Pediatrics ; Prospective Studies* ; Schools, Medical

Craniofacial cleft is a rare congenital anomaly with a wide range of clinical manifestation and severity of deformity. In 1976, Tessier announced classification system on the basis of anatomical observation derived from clinical finding or operative dissection. Nowadays, this system is in common use because it is in accordance with terminology and observational finding and clinical manifestation is accordant with operative finding. Median facial cleft(No. 0-14 facial cleft) has a wide range of congenital malformation from a midline cleft upper lip to orbital hypertelorism, among which the bifid nose is frequently associated with hypertelorism. The manifestation of a bifid nose is variable from a simple central groove at the nasal tip to a complete clefting of the osteocartilaginous framework. In consequence, the planning of correction of the bifid nose must be individualized. We contrived correction of bifid nose using rib bone graft containing small amount of costal cartilage with maneuver of 2mm incision on nasal root skin together with fixation with 9mm miniscrew through an open approach in two No. 0-14 facial cleft patients with mild hypertelorism and bifid nose. With this method we could obtain satisfactory results in the standpoint of function as well as aesthetics. We think that this method is appropriate for correction of bifid nose of mild median facial cleft.
Cartilage ; Classification ; Congenital Abnormalities ; Esthetics ; Humans ; Hypertelorism ; Lip ; Nose* ; Orbit ; Ribs* ; Skin ; Transplants*