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

No abstract available.
Liver* ; Neoplasm Metastasis*

No abstract available.
Cardiomyopathy, Dilated* ; Doxorubicin

Three case of plexiform schwannoma displayed multinodular masses and microscopically a multicentric pattern of growth featuring Antoni A cellular component, Verocay bodies and presence of Antoni B areas. Clinically von Recklinghausen's disease was not observed in all cases. The first patient was a 17 year old male who had a protruding nodule of walnut size which was located at the dermis of the left flank for 13 years. The second case, a 25 year old male, had an irregular whitish brown multinodular mass in the choana for 5 years. The last case, a 56 year old woman, had an ovoid yellowish brown mass with multiple nodules in the retroperitoneum.
Female ; Male ; Humans

A 53-year-old woman developed a psinful erythematous papular skin eruption around the biting site by a tick on the left lower abdomen. A excision biopsy specimen from the skin lesion showed perivsscular infiltration of inflammatory cells composed mainly of neutrophiles, lymphocytes, and a few eosinophiles and histiocytes throughout the dermis. Some of the vessels were dilated with protruding and proliferated endothelial cells. The tick was identified as an adult female of Ixodes nipponensis by the scanning electron microscopic examination.
Abdomen ; Adult ; Biopsy ; Dermis ; Endothelial Cells ; Eosinophils ; Female ; Histiocytes ; Humans ; Ixodes* ; Lymphocytes ; Middle Aged ; Neutrophils ; Skin ; Tick Bites* ; Ticks*

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.