A 49-year old man was admitted to the Korea university hospital, department of surgery, for evaluation of anterior neck mass which was slowly growing for five years. His past history was unremarkable except for known hypertension for several years. Physical examination revealed high blood pressure, measuring 180 mmHg in systolic phase and 120 mmHg in diastolic phase. A soft nontender mass was palpated at anterior neck just above the sternal notch with smooth surface and its size was about 4 x 5 cm in cross. On laboratory examination, diabetic evidence such as high blood sugar (FBS 170 mg/dl, PP2hr. 234 mg/dl) and glucosuria. The CBC finding suggested polycythemia with high hemoglobin (18.0 g/dl) and hematocrit (54%) levels. The differential count and platelet count were within normal limits.
Male ; Humans

Lymphangioleiomyomatosis(LAM) is a rare disease of women of child-bearing age in which there is progressive hyperplasia of atypical smooth mucle along lymphatics in the lung, and/or axial lymphatics in the thorax and abdomen, resulting in honeycombing of lung. Interestingly there has been a speculation that it represents a forme furste or incomplete expression of tuberous sclerosis complex. This is based on the observation that patients with tuberous sclerosis can manifest pulmonary lesions indistinguishable from LAM. We report a case of LAM occuring in a 39-year-old female, who complained of recurrent pneumothorax, chest pain and shortness of breath. Three years ago, the patient had right nephrectomy under the diagnosis of ruptured angiomyolipoma. A X-ray film of the chest showed honeycombing with a diffusely reticulonodular pattern and cyst-like spaces. She had a characteristic facial appearance of adenoma sebaceum, which her father and uncle had. Microscopically, the lung showed a marked smooth muscle proliferation around the slit-like lymphatic spaces and also some respiratory bronchioles.
Female ; Humans ; Adenoma

A case of Osteogenic sarcoma arised from orbit 4 years after the radiation therapy for retinoblastoma in a 9 years old girl is presented. 4 Years ago, under the diagnosis of retinoblastoma, the left orbital exenteration was performed and was treated with x-ray radiation giving 4.500 gamma tumor dose in 4, weeks. On admission, the left orbital cavity was filled with hard mass and orbital bone revealed extensive destruction with osteoplastic changes on x-ray, and diffuse sarcomatous growth of anaplastic cells with abundant osteogenesis and osteoclastic activity microscopically. It is of authors' opinion that osteogenic sarcoma of orbital bone in this presented case might developed by irradition on retinoblastoma.
Child ; Diagnosis ; Female ; Humans ; Orbit ; Osteoclasts ; Osteogenesis ; Osteosarcoma* ; Retinoblastoma*

This study was conducted to develop an optimal strategy to achieve long-term survival after a liver resection for a hepatocellular carcinoma by analyzing the actual survival of 54 patients who underwent liver resections at least 5 years ago. The mean age was 50.5 years with a male-to-female ratio of 5 : 1. The positive rates for HBsAg and anti-HCV were 60.4% (32/53) and 10% (1/10), respectively. Seventy-eight percent of the patients had co-existing chronic liver disease. Seventy percent of the patients had either TNM stage III or IV cancer. Fifteen patients (27.8%) survived longer than 5 years after the operation. The median survival time of the 54 patients was 27 months. Prognostic factors of statistical significance were the Child class, gross type and size of the tumor, intrahepatic metastasis, portal vein invasion, serosal invasion, tumor grade, TNM stage, radicality of the operation, cancer recurrence, and disease-free interval. Although the prognosis for the patients with tumors larger than 5 cm was poor, two patients whose tumors were larger than 10 cm with satellite nodules survived longer than 5 years after undergoing complete tumor removal. For the 26 cases with no intrahepatic metastasis, there was no survival difference between the lobectomy group and the segmentectomy/subsegmentectomy group (43% vs. 42%). In those cases with satellite nodules in one segment of the liver, 80% of the lobectomy group lived longer than 5 years while none of the patients in the less-than-lobectomy group survived long-term (p=0.0060). Cancer recurred in 77.8% of the patients. It was the main cause of late death after surgery. Eighty-one percent of the recurrences occurred within 2 years after the operation. Nearly all the recurrences developed in the remnant liver. Among 15 long-term survivors, 6 patients experienced recurrence between the periods of 26 and 76 months after resection. A total of 4 patients (9.5%) out of these 42 patients were subjected to repeat resection for the recurrent tumor. One of these four patients underwent a third resection for a recurrence after the repeat resection. Although the extent of hepatic resection turned out to be a prognostic factor of little significance overall, the recurrence rate of the lobectomy group tended to be lower than that of the less-than-lobectomy group among the subset of long-term survivors (p=0.0572). For long-term survival, a systematic segmentectomy/ subsegmentectomy is adequate for those tumors without intrahepatic metastasis, while the presence of satellite nodules in that segment mandates a standard hepatic lobectomy. For long-term recurrence-free survival, a hepatic lobectomy is probably needed, even for tumors with no intrahepatic metastasis.
Carcinoma, Hepatocellular* ; Child ; Hepatitis B Surface Antigens ; Humans ; Liver Diseases ; Liver* ; Neoplasm Metastasis ; Portal Vein ; Prognosis ; Recurrence ; Survivors*

A primary cardiac arrest is rare in children. The usual course begins with respiratory arrest and culminates in profound bradycardia or asystole and cardiovascular collapse, so it is vitally important to quickly restore ventilation in pediatric patients(phone-fast). Children over 8 years of age follow the same sequence as adults; that is calling emergency medical services before providing rescue breathing(phone-first).
Adult ; Advanced Cardiac Life Support ; Bradycardia ; Cardiopulmonary Resuscitation* ; Child ; Emergency Medical Services ; Heart Arrest ; Humans ; Ventilation

Fabry's disease is a rare hereditary metabolic disease caused by alpha-galactosidase deficiency, resulting in abnormal accumulation of galactosyl galactosyl galactosyl ceramide (ceramide trihexoside) in various organs. Articles in English reported approximately one hundred cases but only two cases in Korea. Recently the authors experienced a case of Fabry's disease of a male patient and studied the electronmicroscopy on skin biopsied tissue and enzyme assay of alpha -galactosidase activity in his peripheral blood leukocytes. The male patient was a 21-year-old soldier who suffered from anhidrosis with heat intolerance and generalized telangietatic papules (Angiokeratoma corporis diffusum) since childhood. Other clinical findings were ocular change, paroxysmal pain of lower extremities and proteinuria with oval fat bodies on urinalysis. The ultrastructural study of skin demonstrated abnormal lysosomal deposits of finger-prints or "zebra" body configuration in the endothelial cells, pericytes, perineural cells and intercalated ductal epithelium of sweat glands. Enzyme activity of alpha-galactosidase was markedly decreased in the peripheral blood leukocytes comparing to the normal control, which was conclusive to make a diagnosis of Fabry's disease.
Child ; Male ; Female ; Humans

Fabry's disease is a rare hereditary metabolic disease caused by alpha-galactosidase deficiency, resulting in abnormal accumulation of galactosyl galactosyl galactosyl ceramide (ceramide trihexoside) in various organs. Articles in English reported approximately one hundred cases but only two cases in Korea. Recently the authors experienced a case of Fabry's disease of a male patient and studied the electronmicroscopy on skin biopsied tissue and enzyme assay of alpha -galactosidase activity in his peripheral blood leukocytes. The male patient was a 21-year-old soldier who suffered from anhidrosis with heat intolerance and generalized telangietatic papules (Angiokeratoma corporis diffusum) since childhood. Other clinical findings were ocular change, paroxysmal pain of lower extremities and proteinuria with oval fat bodies on urinalysis. The ultrastructural study of skin demonstrated abnormal lysosomal deposits of finger-prints or "zebra" body configuration in the endothelial cells, pericytes, perineural cells and intercalated ductal epithelium of sweat glands. Enzyme activity of alpha-galactosidase was markedly decreased in the peripheral blood leukocytes comparing to the normal control, which was conclusive to make a diagnosis of Fabry's disease.
Child ; Male ; Female ; Humans

Nowday, ionizing radiation is one of the methods eradicating the uterine cervical malignancy. However radiation alone or in combination with surgery have an effect on normal tissue as well as the malignant cells, and their changes have been well described in other countries. Unfortunately, the history of radiation modality for cancer treatment is relatively short and the reports about radiation induced changes are limited in our country. We evaluated the radiation-induced changes in cervico-vaginal smears of 107 uterine cervical cancer patients obtained from March, 1985 to October, 1987. Most patients had been received 5,400 Rads of external radiation and intracavitary radiation. Patient's age ranged from 30 to 67 years old. Of 107 cases, 24 cases were normal, 72 cases showed benign radiation changes, 7 cases revealed radiation dysplasia, and residual and recurrent carcinomas found in one and 3 cases, respectively. Cytoplasmic and nuclear enlargement were the most common and noted in 57 and 38 cases, respectively. Vacuolization and polychromasia of the cytoplasm were identified in 43 and 30 cases, respectively. The most common histiocytic change was multinucleation, which was found in about one third. The radiation changes of the cytoplasm and nuclear enlargement persisted for a long time after completion of radiation, however, nuclear degeneration and multinucleation gradually disappeared after 6 months. The inflammation in background prolonged for a long time but degeneration disappeared after 6 months. The biologic significance of post-radiation dysplasia could not evaluated because of short follow up period.

Nowday, ionizing radiation is one of the methods eradicating the uterine cervical malignancy. However radiation alone or in combination with surgery have an effect on normal tissue as well as the malignant cells, and their changes have been well described in other countries. Unfortunately, the history of radiation modality for cancer treatment is relatively short and the reports about radiation induced changes are limited in our country. We evaluated the radiation-induced changes in cervico-vaginal smears of 107 uterine cervical cancer patients obtained from March, 1985 to October, 1987. Most patients had been received 5,400 Rads of external radiation and intracavitary radiation. Patient's age ranged from 30 to 67 years old. Of 107 cases, 24 cases were normal, 72 cases showed benign radiation changes, 7 cases revealed radiation dysplasia, and residual and recurrent carcinomas found in one and 3 cases, respectively. Cytoplasmic and nuclear enlargement were the most common and noted in 57 and 38 cases, respectively. Vacuolization and polychromasia of the cytoplasm were identified in 43 and 30 cases, respectively. The most common histiocytic change was multinucleation, which was found in about one third. The radiation changes of the cytoplasm and nuclear enlargement persisted for a long time after completion of radiation, however, nuclear degeneration and multinucleation gradually disappeared after 6 months. The inflammation in background prolonged for a long time but degeneration disappeared after 6 months. The biologic significance of post-radiation dysplasia could not evaluated because of short follow up period.

Neurofibromatosis, first clearly described by von Recklinghausen in 1882, is a dominantly inherited mesodermal and ectodermal dysplasia with a broad spectrum of clinical findings. Most common is the classical neurofibromatosis, which has three major features: (1) multiple neural tumors dispersed anywhere on or in the body; (2) numerous pigmented skin lesions, some of which are "cafe au lait" spots; and (3) pigmented iris hamartomas also called Lisch nodules. Other lesions sometimes seen in patients with von Recklinghausen's disease include congenital malformations of various types, vascular lesions, neurilemoma meningioma and other intracranial neoplasms, pheochromocytoma, medullary carcinoma of thyroid gland, neuroblastoma, ganglioneuroma and Wilms' tumor. Approximately 10% of the patients with neurofibromatosis have the gastrointestinal tract tumors as neurofibroma, ganglioneuroma and leiomyoma. Some of them show evidence of malignancy. Increased activity of the protein "nerve growth factor" in the sera of the patients with disseminated neurofibromatosis has been reported. We would like to report here two cases of von Recklinghausen's neurofibromatosis with involvement of the gastrointestinal tract.
Hamartoma ; Meningioma