Neurofibromat osis is a familial discase with widespread manifestations such as lesions of the skin, tumors of the central and peripheral nervous system and abnormalities of bone. The presence of at least two of the following features was considered for diagnostic criteria; positive family history, positive blopsy, a minimum of six cafe-au-lait spots each with a diameter of at least 1.5 cm and multiple subcutaneous nodules. From January 1971 to December 1979, 17 patients of neurofibromatosis and 30 patients of neuro- fibroma treated at Yonsei Medical Center were clinically analized for the study and the results obtained from this study were as follows; 1. Positive family history was obtained In 2 per cent of cases with neurofibromatosis. 2. Cafe-au-lait spots or tumors were the most common findings in neurofibromatosis. The bone changes were presented in 8 patients(45 per cent) and scoliosis has been In 62.5 per cent of them. 3. The location of the neurofibroma was variable. Central nervous sustem was involved in 40.4 per cent and peripheral nervous system in 59.4 per cent. 4. Myelography and determinations of the cerebro-spinal fluid protein concentration led us to a correct diagnosis of the neurofibroma which involved the spinal cord. For the treatment of the neurofibroma, 9 out of 30 patients were performed laminectomies and excision of the intradural masses, and one of them was flxed the spinous processes with a plate and wires after laminectomy for prevent spinal instability. 5. in 3 patients with progressive scoliosis, the best results were obtained with early Harrington instrumentation and posterior fusion.
Cafe-au-Lait Spots ; Chymopapain ; Clinical Study ; Diagnosis ; Fibroma ; Humans ; Laminectomy ; Myelography ; Neurofibroma ; Neurofibromatoses ; Peripheral Nervous System ; Scoliosis ; Skin ; Spinal Cord

Transient synovitis is characterized by the development of pain arising from the hip and often felt in the thigh or knee in children between the ages of 2 and 12 years. The benign self-limiting nature of this condition has made it difficult to establish the caus'e. However, the condition is generally conceded to be the commonest cause of a painful hip in childhood. The disease is of interest, not because of its disabling condition, but because of the difficulty in differentiating it from more serious diseases of the hip, such as tuberculosis, osteomyelistis, or Legg-Perthes disease. The author had studied the clinical symptoms and signs, roentgenologic findings, and treatment of the 34 patients who had admitted to Severance Hospital Yonsei Medical Center in Seoul, Korea from January 1970 to December 1979, under the diagnosis of the transient synovitis of the hip and summerized the results as follows; 1. There was probably associated with predisposing factors such as infection and allergy. 2. Among the 34 patients, 28 cases(82.4%) were male and 6 cases(17.6%) were female. The most prevalent age were between 6 and 10 years old. 3. Pain and limitation of motion of the hip joint were the most common symptoms. In 56.5% of the patients, roentgenographic findings were positive. In 50% of the patients, the erythrocyte sedimentation rates were increased. 4. The treatment was conservative including bed rest, skin traction on the affected leg, non-weight bearing, antibiotics, and sedatives. Most of all patients had improved symtoms and signs within two weeks of treatment. 5. The course of this condition was short and benign with complete resolution. The occasional hip with chronic or recurrent symptoms could be distinguished from Legg-Perthes disease by the short history, normal radiographs, and the complete resolution.
Anti-Bacterial Agents ; Bed Rest ; Blood Sedimentation ; Causality ; Child ; Clinical Study ; Diagnosis ; Female ; Hip Joint ; Hip ; Humans ; Hypersensitivity ; Hypnotics and Sedatives ; Knee ; Korea ; Leg ; Legg-Calve-Perthes Disease ; Male ; Seoul ; Skin ; Synovitis ; Thigh ; Traction ; Tuberculosis

No Abstract Available.
Diagnosis* ; Enzyme-Linked Immunosorbent Assay* ; Flagellin* ; Leptospirosis*

The Sturge Weber syndrome is an uncommon disorder characterized by port-wine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated with this syndrome are mental retardation, choroidal angioma, buphthalmus or glaucoma, seizure and hemiplegia. We studied a case of incomplete form of Sturage Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calcification and leptomeningeal angiomatosis. There are no characteristic facial nevus, mental retardation, occular changes and hemiplegia.

Fibrosarcoma is a rare malignant tumor in the larynx. It is quite different from epidermoid carcinoma seen most frequently in the larynx in the aspect of invasive behavior; metastatic route and treatment modality. This paper presents a case of pyriform sinus fibrosarcoma with a brief review of literature.
Adult ; Case Report ; Fibrosarcoma/pathology* ; Human ; Laryngeal Neoplasms/pathology* ; Male

Pontine hematomas are very rare lesions that in the past found at postmortem examination rather than diagnosed from the clinical presentations. Computed tomography has particularly stimulated further neurosurgical interest in this relatively rare condition, making surgical evacuation possible in some cases. Four patients suffering from pontine hemorrhages, revealed by computed tomography, are discussed. The classification regard to the site, clinical features and outcomes of this lesion are discussed in an attempt to establish general criteria for adequate management. With regard to the site, three types of hemorrhage are to be considered the tegmentotectal(type I), the tegmentolateral(type II) and the tegmentobasilar(type III), 2 patients(type I II, treated conservatively) had a fair outcome, and 2 patients(one of type III, treated conservatively, and one of type I, by surgical evacuation) had a death. Surgical management for the pontine hemorrhages should be reserved for the type I(tegmontotectal) and type II(tegmentolateral), whenever the neurological conditions deteriorate progressively.
Autopsy ; Classification ; Hematoma ; Hemorrhage* ; Humans ; Prognosis*

A case of pleomorphic xanthoastrocytoma that occured in a 27-year-old woman is reported. Despite of cellular pleomorphism and presence of bizarre giant cells in the microscopic picture, the relatively favourable prognosis is the most important characteristic.
Adult ; Female ; Giant Cells ; Humans ; Prognosis

Steatocystoma multiplex is a rare autosomal-dominant disorder characterized by asymptomatic multiple, skin-colored to yellowish nodules on the trunk, proximal extremities, and axillae. Although surgical excision is the most effective method, treatment is difficult when lesions occur in multiple numbers. Therefore, various treatments such as CO2 laser therapy, needle aspiration, and oral isotretinoin have been attempted, but results are variable. A 37-year-old man presented with multiple skin-colored subcutaneous nodules on the abdomen and both axillae. A histopathological examination was consistent with steatocystoma multiplex. The lesions were removed by dermal shaving with a suction-assisted cartilage shaver. It was an effective therapeutic method for multiple lesions with no significant adverse events. Herein, we report a case of steatocystoma multiplex treated with a suction-assisted cartilage shaver.
Abdomen ; Adult ; Axilla ; Cartilage ; Extremities ; Humans ; Isotretinoin ; Lasers, Gas ; Needles ; Steatocystoma Multiplex

Lichen planus pigmentosus (LPP) is chronic pigmentary disorder that shows diffuse or reticulated hyperpigmented, dark brown macules on the sun-exposed areas such as the face, neck and other flexural folds. Clinically, it is different from classical lichen planus because LPP has a longer clinical course and it manifests with dark brown macules. In case of LPP, involvement of the scalp, nail or mucosal area is rare. The histopathological findings of the lesions show an atrophic epidermis, the presence of melanophages and a vacuolar alteration of the basal cell layer with a sparse lymphohistiocytic lichenoid infiltration. Although there have been a few reports of LPP, there have only 3 cases of linear LPP along the lines of Blaschko in the Korean dermatologic literature. Our patient had lesions on the neck and chin with a linear pattern. In this report, we describe a very rare case of LPP with a linear distribution related to Blaschko's lines on the neck and chin areas.
Chin ; Epidermis ; Humans ; Lichen Planus ; Lichens ; Nails ; Neck ; Scalp

PURPOSE: A chemotherapy response assay test is performed to evaluate the degree of tumor growth inhibition by a chemotherapeutic agent. Several studies have been done on its usefulness; however, to the best of our knowledge, only a few studies concerning the relationship between chemotherapy response assay test results and breast cancer patients' prognoses have been conducted. Thus, we performed this study to analyze this relationship. METHODS: Among breast cancer patients who underwent curative surgery and neoadjuvant or adjuvant chemotherapy between August 2004 and December 2009, 102 were enrolled in this study. Chemotherapeutic regimens for patients were doxorubicin plus taxane or doxorubicin plus cyclophosphamide followed by taxane. We divided these patients into two groups (sensitive group [n=19] and resistant group [n=83]) and analyzed the relationship between chemosensitivity results and patient prognosis. RESULTS: The sensitive group was associated with poor disease-free survival (DFS) (p=0.003) and overall survival (OS) (p<0.001). No significant differences were observed in tumor histology (p=0.548), tumor size (p=0.479), number of metastatic lymph nodes (p=0.326), histologic grade (p=0.077), or nuclear grade (p=0.216) between the two groups. However, in respect to molecular subtype, the HER2-positive type and triple negative breast cancer were more frequently observed in the sensitive group (p=0.001). In a univariate and multivariate analysis for DFS, doxorubicin sensitivity was significantly associated with a poor prognosis (p<0.05). CONCLUSION: Better chemosensitivity results are associated with a poor prognosis in breast cancer patients who have undergone anthracycline- and taxane-based chemotherapy, however, examination of additional cases and the use of a longer study period are needed.
Breast ; Breast Neoplasms ; Bridged Compounds ; Chemotherapy, Adjuvant ; Cyclophosphamide ; Disease-Free Survival ; Doxorubicin ; Humans ; Lymph Nodes ; Multivariate Analysis ; Prognosis ; Taxoids