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

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

Osteoblastoma is a rare tumor which forms osteoid and bone with highly vascular stroma, account for less than 1 percent of all bone tumors. The lesion had been thought to be controlled with curettage and locat excision. In recent years, cases of locally aggressive but not metastasizing osteoblastomas have been described as aggressive osteoblastoma or malignant osteoblastoma, separated from the innocuous behavior of usual osteoblastoma. It represents the histologic characteristics of the presence of epithelioid osteoblasts, trabecular rather than lace-like osteoid, low mitotic rate with no atypical form with prominent giant cells of osteoclastic type The differential diagnosis between aggressive osteoblastoma and osteogenic sarcoma is extremely difficult, but its peculiar histologic pattern and ist different clinical and radiologic features and better prognosis are helpful. We report here a case of aggressive osteoblastoma of 17 years old female patient with 2 years and 5 months follow-up period and review the literature.
Female ; Humans ; Diagnosis, Differential

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.

No abstract available.
Cardiomyopathy, Dilated* ; Doxorubicin

Kawasaki disease (KD) is an acute systemic febrile illness, predominantly affecting children less than the age of 5 years. After its first description in 1967 by Dr Tomisaku Kawasaki, KD has become the leading cause of acquired heart diseases in children in developed nations. KD has been reported in all racial and ethnic groups but the incidence of KD is more common among Asians, and the incidence of the disease varies considerably among different Asian countries. There is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. which suggests differences of race-specific susceptibility. In Korea, from 1990s, a nationwide epidemiologic study has been conducted every 3 years by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. So the average annual incidence, 105.0/100,000 children < 5 years in recent study (2003-2005) was the second highest reported rate in the world after Japan.
Asian Continental Ancestry Group ; Child ; Developed Countries ; Epidemiologic Studies ; Epidemiology ; Ethnic Groups ; Heart ; Heart Diseases ; Humans ; Incidence ; Japan ; Korea* ; Mucocutaneous Lymph Node Syndrome* ; Prevalence