The cliniopathological analysis was done on the 104 cases of malignant melanoma diagnosed at the Seoul National University Hospital (SNUH) from 1984 to 1993. The basic clinical data and the pathological items were based on the New Mexico Melanoma Registry Worksheet. The results were as follows. The male to female ratio was 1 : 0.79. Primary cutaneous melanoma was more common in the male (M : F=1 : 0.56) but primary extracutaneous melanoma with slight female dominancy (M : F=1 : 1.25). The peak age was the 6th decade in both cutaneous and extracutaneous malignant melanoma. In 66% (35 cases) of primary cutaneous malignant melanoma, the primary site was located in the acral area (including cases of acral lentiginous and nodular type), of which 63% (41% of total cutaneous melanoma) was acral lentiginous type. Major components of tumor cells were epithelioid. Clark's level of tumor was III or more at the time of the first visit in the majority of the cases (85%). The incidence rate of extracutaneous melanoma was 34.6% (36 cases) among the primary melanoma, and the eyeball (17.3%) was the most prevalent organ. All these features suggest that the racial difference between the Korean and the Caucasian is evident and also that etiologic role of sun damage is not quite marked in the Korean. We also suggest that an early detection program is very important to cure this malignant tumor.
Female ; Humans ; Incidence ; Male ; Melanoma* ; New Mexico ; Seoul ; Solar System

PURPOSE: The prevalence of developmental delay is 5-10% of the total pediatric population and early diagnosis and proper intervention are essential but a challenge for clinicians. We performed this study for several purposes. First is to know the distribution & characteristics of developmental disorders in Korea. Second is to identify all possible causes of these disorders through a multidisciplinary diagnostic approach, and thus to find out the clinical variables that are helpful in finding the etiology. And finally to develop a useful protocol that eliminates the cost of unnecessary tests and raises the diagnostic rate of the cause. METHODS: 518 patients(M 349, F 169) were studied who visited Ilsan Hospital Developmental Disorder Clinic(DDC) for the evaluation of developmental delay from April 2001 to Jan 2005. RESULTS: The mean age was 51.5+/-32.9 months, ranging from 2 months to 16.0 years of age with a majority of the preschool children(<6 yr)(79.3%). Phenomenological diagnosis consisted of 133 cases of mental retardation, 122 cases of autistic disorders, 101 cases of delayed language disorders, 27 cases of cerebral palsies, and 91 cases labeled as simple developmental delay requiring follow up due to age less than 2 years of age. Etiologic diagnosis was obtained in 119 cases(22.9%) out the 518 cases of developmental delays. 37 cases of chromosomal anomalies, 23 cases of Periventricular leukomalacia and hypoxic ischemic encephalopathy, 21 cases of syndromes, 7 cases of malformation of cortical development, 4 cases of myopathies, 4 cases of neuropathies, and 4 cases of cerebral infarctions were found. Among the clinical variables, low birth weight, facial dysmorphism, hypotonia, focal neurologic signs, and abnormalities in MRI, chromosome, EEG and EMG studies contributed to the yields of etiologic diagnosis significantly. CONCLUSION: Possible etiology was determined in about 23% of the subjects. The most important part of the assessment for the identification of etiology is thorough history taking, physical and neurologic examination. Neuroimaging study is useful in case of micro or macrocephaly, focal neurologic signs. Genetic studies increasingly produce a yield, when there is family history of inherited disorder and there are dysmorphic features. Routine metabolic screening test has limited utility. Development of a useful screening protocol adequate for Korean situation is required.
Cerebral Infarction ; Diagnosis ; Early Diagnosis ; Electroencephalography ; Follow-Up Studies ; Humans ; Hypoxia-Ischemia, Brain ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Korea ; Language Disorders ; Leukomalacia, Periventricular ; Macrocephaly ; Magnetic Resonance Imaging ; Mass Screening ; Muscle Hypotonia ; Muscular Diseases ; Neuroimaging ; Neurologic Examination ; Neurologic Manifestations ; Paralysis ; Prevalence

Lymphomatous involvement of the heart, occurring at initial diagnosis and presentation, is extremely rare. We report here a case of 58 year old man who presented with generalized edema, pericardial effusion, and a large right atrial mass detected by transesophageal echocardiography. There is no other evidence of disseminated lymphoma in this patient. Tumor removal and pulmonary embolectomy was done. Pathologically, the mass was malignant lymphoma, diffuse large cell type. Unfortunately, we have no chance to perform the intensive chemotherapy. The patient discharged in moribund state.
Diagnosis ; Drug Therapy ; Echocardiography, Transesophageal ; Edema ; Embolectomy ; Heart ; Humans ; Lymphoma* ; Middle Aged ; Pericardial Effusion

No abstract available.

PURPOSE: We analyzed the oncological outcome and prognostic factor of the chondrosarcoma arising from benign bone tumor due to malignant transformation. MATERIALS AND METHODS: From April 1986 to April 2009, 18 cases were considered eligible. We analyzed retrospectively the patient's characteristics and prognostic factors that affect to the local recurrence and distant metastasis. RESULTS: As classified by primary benign bone tumor, 4 cases were solitary osteochondroma, 11 cases were multiple osteochondromatosis and 3 cases were multiple enchondromatosis. The mean follow-up period was 85 months. The 5-year disease free survival rate of 18 patients was 85.9%. Their overall MSTS score was 25.2 (84%). There were local recurrence in 3 cases and no distant metastasis. We found that tumor location and surgical margin affected to the prognosis significantly. CONCLUSION: In secondary chondrosarcoma patients, the prognosis was good relatively and tumor location and surgical margin are important prognosis factor.
Chondrosarcoma ; Disease-Free Survival ; Enchondromatosis ; Follow-Up Studies ; Humans ; Neoplasm Metastasis ; Osteochondroma ; Osteochondromatosis ; Prognosis ; Recurrence ; Retrospective Studies

No abstract available.

PURPOSE: Current evidence of the benefits of early intensive behavioral intervention (EIBI) in children with autism spectrum disorders(ASD) is increasing. In order to get the benefits from EIBI, early identification of ASD is essential but a challenge for clinicians. Therefore, we designed this study to know what differs from other developmental disorders in terms of early developmental patterns and clinical and laboratory characteristics. METHODS: Among 518 children diagnosed developmental delay in Ilsan Hospital Developmental Disorder Clinic from April 2001 to January 2005, we analyzed 122 ASD patients as a study group, and 133 mentally retarded(MR) and 101 specific language impaired(SLI) patients as control groups. We used a questionnaire survey to assess past and family history. We performed various kinds of tools for the evaluation of the development, and cognitive and speech functions according to the patients' age. MRI, SPECT, EEG, genetic and metabolic studies were performed as optional tests. RESULTS: The mean age of ASD was 55.9+/-30.7 months and male was predominant in ASD(male:female ratio was 3.2:1). In early developmental history, motor functions are relatively well preserved in ASD(P<0.05). In speech and social milestones, single word speech was noticeably delayed in all the 3 groups. However, there was no appreciable difference in stranger anxiety among the 3 groups. In the developmental evaluation, speech and social functions were most significantly delayed in ASD(P<0.05). There was a significant motor delay in ASD and MR than SLI(P<0.05). In physical examination, a raised rate of macrocedphaly in ASD was not noted. Abnormal EEG findings were significantly noted in ASD and MR than in SLI and MRI abnormalities were least found in these groups. The etiologic diagnosis was much less identified in ASD than MR. CONCLUSION: In an early stage, absence of stranger anxiety was not statistically significant. However, this seems to be an important risk factor for predicting ASD. Motor functions were relatively well preserved in ASD in an early stage. In the developmental evaluation, social and language areas were the most significantly delayed domains in ASD and motor functions were most delayed in MR. Routine MRI studies are not indicated for identifying the etiology in ASD. It was harder to find out the etiology in ASD than MR.
Anxiety ; Autistic Disorder* ; Child ; Autism Spectrum Disorder* ; Diagnosis ; Electroencephalography ; Humans ; Magnetic Resonance Imaging ; Male ; Physical Examination ; Surveys and Questionnaires ; Risk Factors ; Tomography, Emission-Computed, Single-Photon

We describe a case of the juvenile form of Pompe's disease that presented as primary alveolar hypoventilation due to respiratory muscle involvement. This 17-year-old girl had been asymptomatic until this admission, although she had a delayed puberty. Arterial blood gas analysis, pulmonary function test as well as physical findings were compatible with chronic alveolar hypoventilation syndrome. Since she had lower extremity muscle weakness and pseudomyotonic discharge on electromyography a muscle biopsy was done, which revealed glycogen storage disease. The patient was managed successfully with nasal intermittent positive pressure ventilation.
Adolescent ; Chronic Disease ; Female ; Glycogen Storage Disease Type II/*complications/pathology ; Humans ; Hypoventilation/*etiology/therapy ; Intermittent Positive-Pressure Ventilation ; Muscles/pathology ; Pulmonary Alveoli

We report a severe case of hyponatremic encephalopathy in a 38 year old schizophrenic patient with polydipsia that was very likely precipitated by hydrochlorothiazide given for the accompanied hypertension in this patient. On admission via emergency room, this patient's electrolyte imbalances include not only hyponatremia but also hypokalemia, hypomagnesemia and metabolic alkalosis, which have been well known as the complications of thiazide diuretics. Fortunately, this patient recovered from comatose condition with the treatment of hyponatremia at the correction rate of about 0.5mEq/L/hr in addition to potassium and volume replacements. However, this case gives us the warning that the presence of hyponatremic condition such as polydipsia should be ruled out before thiazide diuretics prescribed for patients with schizophrenia, and other electrolytes imbalance in addition to hyponatremia as the complications of thiazide diuretics should be looked for the simultaneous treatment for them.
Adult ; Alkalosis ; Coma ; Electrolytes ; Emergency Service, Hospital ; Humans ; Hydrochlorothiazide ; Hypertension ; Hypokalemia ; Hyponatremia ; Polydipsia ; Potassium ; Schizophrenia ; Sodium Chloride Symporter Inhibitors

Carcinoma of the bladder is a worldwide disease with several histological patterns, 0.5 to 2.0% of which are caused by adenocarcinomas. The etiology of adenocarcinoma of the bladder is unknown Adenocarcinoma of the bladder may be classified as primary, urachal or metastatic based on the site or tumor origin. The primary site of metastatic adenocarcinoma include the rectum, stomach, endometrium, breast, prostate and ovary. Metastasis to the bladder from adenocarcinoma is a relatively rare phenomenon occurring in only 0.26% of cases. When urologic symptoms newly developed to the patients who had malignancy of digestive organs, metastatic malignancy of urinary tact should be considered. Herein, we report a case of metastatic bladder tumor from gastric cancer which occurred 15 months after subtotal gastrectomy.
Adenocarcinoma* ; Breast ; Endometrium ; Female ; Gastrectomy ; Humans ; Neoplasm Metastasis ; Ovary ; Prostate ; Rectum ; Stomach Neoplasms* ; Stomach* ; Urinary Bladder Neoplasms ; Urinary Bladder*