Of all the primary central nervous system tumors, the medulloblastoma, glioblastoma multiforme, ependymoma and pineal germinoma tend to exfoliate in the cerebrospinal space. With all other types of the tumor, abnormal cells may seldom be definitely identified in the cerebrospinal fluid. Up to now the tumor cells have been rarely found in CSF cases of retinoblastoma. We have experienced a case of advanced retinoblastoma that showed exfoliated cells in spinal fluid.

Marjolin's ulcer seated upon an old cicatrix, especially old burn scar, which may chiefly degenerated into a squamous cell carcinoma with propensity for metastasis. We are experienced two cases of Marjolins ulcer recently. A 54-year-old male who had a Marjolins ulcer(15x40cm) on his left lower extremity. He was experienced burn at the age of eight and rice sized ulcer was developed at the site of burn scar about 21 years ago. The ulcer progressively enlarged in size to reach 15x40cm for 21 years. The pathologic diagnosia was squamous cell carcinoma grade 1, but cancer cells invaded to deep dermie and subcutaneous tissues. Severe skin lesion and irreversible osteoporotic degeneration on knee joint of affected limb were present. So he was treated by high above knee amputation. Another 58-year-old female who had a Marjolins ulcer(10x20cm) on her right lower extremity. She was experienced burn at the age of forty eight and bean sized ulcer was developed at the site of burn scar about 1 year ago. The pathological diagnosis was squamous cell carcinoma grade 1 and invasian of cancer cells was limited upper dermis. On lymph node biopsy, the histological diagnosis was within normal limit. So she was treated radical surgical excision with split thickness skin graft(Mesh).
Amputation ; Biopsy ; Burns ; Carcinoma, Squamous Cell ; Cicatrix ; Dermis ; Diagnosis ; Extremities ; Female ; Humans ; Knee ; Knee Joint ; Lower Extremity ; Lymph Nodes ; Male ; Middle Aged ; Neoplasm Metastasis ; Skin ; Subcutaneous Tissue ; Ulcer*

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell disorder showing spontaneous resolution within 3-4 months. By electron microscopy, the identification of many Birbeck granules and laminated dense bodies in the infiltrated cells is mandatory for the diagnosis of CSHRH. We describe a case of congenital self-healing reticulohistiocyt~osis in a 4-month-old male infant.
Diagnosis ; Humans ; Infant ; Male ; Microscopy, Electron

A case of rather typical Crohn's disease in a 10 year old girl is described. She had suffered from intractable abdominal pain, diarrhea and fever for 1 year. Eventual right hemicolectomy revealed diffuse involvement of terminal ileum, cecum and ascending colon by confluent ulcerations and transmural inflammation. Histologically there were numerous well developed non-caseating granulomas scattered transmurally and in regional lymph nodes. Deep penetrating ulcerations were characteristic. Acid fast staining failed to demonstrate any organism. The rarity of Crohn's disease in Korea and this occurrence in pediatric age prompted this report.
Child ; Colitis/pathology ; Crohn Disease/diagnosis/*pathology ; Diagnosis, Differential ; Female ; Granuloma/pathology ; Humans ; Ileitis/pathology ; Intestines/pathology

Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.
Angiography ; Catheterization ; Catheters ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Drainage ; Estrogens, Conjugated (USP) ; Humans ; Hypertension ; Lung ; Magnetic Resonance Imaging ; Perfusion ; Pneumonectomy ; Pulmonary Edema ; Pulmonary Veins* ; Radiography, Thoracic ; Veins

Trichoblastic fibroma is a rare, benign trichogenic tumor that has both an epithelial and mesenchymal component. This tumor may be confused clinically and/or histologically with basal cell carcinoma and other tumors with hair follicle differentiation. We describe here a patient with trichoblastic fibroma on the nose. For further characterization of the tumor, we studied the bcl-2 expression in this case and compared it with those of trichoepitheliomas and basal cell carcinomas. The bcl-2 expression in this case and trichoepitheliomas were positive at the periphery of the tumor nest only, whereas those of basal cell carcinomas were positive diffusely throughout the tumor nest.
Carcinoma, Basal Cell ; Fibroma* ; Hair Follicle ; Humans ; Nose

We report a case of a 42-year-old woman who suffered from recurrent nodular skin lesions on her left foot. Sporotrichoid fungal infection was suspected and two linear nodular skin lesions that had occurred after trauma in a public pool were treated with itraconazole for 4 months. These nodular skin lesions were completely flattened. However, four months after complete flattening, a new lesion developed in the scar of a previous nodule. N. asteroides, which is extremely rare for sporotrichoid cutaneous nocardiosis, was cultured from the relapsed lesion. We treated this case with trimethoprim-sulfamethoxazole for 6 months under an emperical basis and this resulted in complete healing, and sensitivity of N. asteroide to trimethoprim-sulfamethoxazole was confirmed later. There has been no recurrence for 3 years. To our knowledge, our case is the first report in the english literature of primary sporotrichoid cutaneous nocardiosis caused by N. asteroides in terms of anatomic location below the knee and host immunocompetency.
Adult ; Cicatrix ; Female ; Foot ; Humans ; Itraconazole ; Knee ; Nocardia asteroides* ; Nocardia Infections* ; Nocardia* ; Recurrence ; Skin ; Trimethoprim, Sulfamethoxazole Drug Combination

Job's syndrome is an inflammatory skin disease characterized by (1) severe eczema, (2) recurrent staphylococcal infections of the skin and sinopulmonary tract, (3) cold subcutaneous abscesses, and (4) high serum IgE levels. We describe a 55-year-old woman with long-standing atopic dermatitis-like eczema, recurrent abscesses, and a high level of serum IgE. We suspected this case as Job's syndrome.
Abscess ; Eczema ; Female ; Humans ; Immunoglobulin E ; Job Syndrome* ; Middle Aged ; Skin ; Skin Diseases ; Staphylococcal Infections

PURPOSE: Iliac vein compression syndrome (IVCS), first characterized by Cockett and Thomas in 1965, is the development of iliofemoral deep venous thrombosis due to compression of the left common iliac vein against the spine and pelvic brim by the right common iliac artery. Thrombectomy treatment of the underlying compression is essential if significant long-term sequelae are to be avoided. Surgical treatment options include venous reconstruction or venous bypass, but treatment using endovascular techniques have recently been described. This study was conducted to evaluate the usability of endovascular treatment (esp. thrombolysis with stent insertion) in a venous outflow obstruction resulting from IVCS. METHOD: During a 2-year period, 20 patients (17 women, 3 men; mean age, 60 years) presented with clinical and imaging findings consistent with IVCS. All patients presented with leg edema or pain. The mean duration of symptom onset was 6.6+/-4.4 days, ranging from 1 to 15 days. All patients were evaluated using a Duplex scan, computerized tomography and venography. After the ascending venography had been performed, an infusion catheter system was placed, and urokinase infused locally into the thrombus burden. After near complete clot dissolution, the residual left common iliac vein stenosis was treated by means of angioplasty and the placement of a Wallstent. All patients continued to receive oral warfarin. Patients were followed-up by means of clinical visits, and the stent patency was assessed by means of a Duplex scan or computerized tomography. RESULT: The total dose of urokinase used and the duration of infusion were 2.28+/-0.93 million unit, ranging from 1.00 to 5.20 and 46.8+/-14.8 hours, ranging from 14 to 72 hours, respectively. Grade III (complete lysis) thrombolysis was achieved in 17 patients. All 17 patients successfully received a Wallstent. The initial clinical success was 100%, with complete resolution of symptoms in all patients. One patient, with combined ovarian cancer, had a recurrent symptomatic deep vein thrombosis and complete occlusion of the stent due to thrombosis 2 months after treatment. However, no other patients showed evidence of deep vein thrombosis after treatment. The overall patency rate of the stenting at 18 months was 94.1%. There were no deaths, pulmonary embolism, cerebral hemorrhage or major bleeding complications. CONCLUSION: These results suggested that the treatment of iliac vein compression syndrome, using catheter directed thrombolytic therapy and venous stent insertion, was a safe and effective method at the mid term evaluation. These patients will continue to be followed up with screening tests to further define the long-term patency.
Angioplasty ; Catheters ; Cerebral Hemorrhage ; Constriction, Pathologic ; Edema ; Endovascular Procedures ; Female ; Hemorrhage ; Humans ; Iliac Artery ; Iliac Vein* ; Leg ; Male ; Mass Screening ; May-Thurner Syndrome* ; Ovarian Neoplasms ; Phlebography ; Pulmonary Embolism ; Spine ; Stents ; Thrombectomy ; Thrombolytic Therapy ; Thrombosis ; Urokinase-Type Plasminogen Activator ; Venous Thrombosis ; Warfarin

Abdominal lymphangiomas are uncommon angiomatous tumor occurring mainly in childhood. This is a retrospective clinicopathologic study of 17 cases of abdominal lymphangioma. The patients included are five children and 12 adults, with a mean age at initial presentation of 30.7 years (age ranges 3-63). The locations of the tumors were mesentery (5), retroperitoneum (4), colon (3), omentum (3), mesocolon (1) and gallbladder (1). Infiltrative growth was more common pattern than entirely circumscribed pattern. Masses were mostly multilocular cysts and contained chyle or serous fluid. On immunohistochemical staining, 16 cases were reactive for either CD31 or factor VIII-related antigen. These fact would suggest that intra-abdominal lymphangiomas simulate the immunohistochemical features of collecting lymphatics. Follow up was possible in 12 cases for 3-50 months (mean 19 months) and only one patient showed local recurrence. Although abdominal lymphangiomas are rare in adulthood and correct preoperative diagnosis is difficult, awareness of such a possibility in adulthood will contribute to make a correct preoperative diagnosis.
Abdominal Neoplasms/physiopathology ; Abdominal Neoplasms/pathology* ; Abdominal Neoplasms/metabolism ; Adult ; Antigens, CD31/biosynthesis ; Child ; Child, Preschool ; Factor VIII/biosynthesis ; Female ; Human ; Lymphangioma/physiopathology ; Lymphangioma/pathology* ; Lymphangioma/metabolism ; Male ; Middle Age ; Retrospective Studies