No abstract available.
Wilms Tumor*

Familial lichen planus is an uncommon disease, and there are 104 cases reported in the English literature. Several theories regarding the etiopathogenesis of lichen planus have been proposed. However, immunological mechanisms and genetic susceptibility have been emphasized the most. Several reports showed HLA analysis in lichen planus patients, and recently 2 cases have revealed an increased frequency of DR1 antigen in patients with lichen planus compared to a control group. We observed two brothers with typical skin lesions of lichen planus of almost simultaneous onset. We confirmed lichen planus by skin biopsies in both brothers and analyzed HLA typing. We also observed the occurrence of DR1 antigen in both of them.
Biopsy ; Genetic Predisposition to Disease ; Histocompatibility Testing ; Humans ; Lichen Planus* ; Lichens* ; Siblings ; Skin

Dystrophic epidermolysis bullosa is a rare, chronic non-inflammatory bullous disease, which easily forms bullae by minor mechanical trauma or spontaneously, is inherited either in an autosomal dominant or autosomal recessive fashion. We report herein two cases which presented with bullae, erosions and ulcers on extremities, buttock, chest, abdomen and face and loss of all nail since birth in two sisters. Bulla occured bencath the basal lamina histopathologically, anchoring fibrils were almost absent on electron miaoscopy in both cases. The two sisters represented dystrophic epidermolysis bullosa considering the absence of family history inheritcd in an autosomal dominant fashion and the clinical, histological and electronmicroscopic findings.
Abdomen ; Basement Membrane ; Buttocks ; Epidermolysis Bullosa Dystrophica* ; Extremities ; Humans ; Parturition ; Siblings* ; Thorax ; Ulcer

Anti-E and anti-c is one of the clinical significant irregular antibodies developing a delayed hemolytic transfusion reaction and hemolytic disease of the newborn. Since anti-c occurs frequently with anti-E in immunized people whosoe cells are E-and c-, it has been recommended to select blood of the patient's own R1 phenotype for transfusion, even when the presence of anti-c cannot be demonstrated in his/her serum. To determine the utility of this approach, we reviewed the blood bank laboratory records of patients identified anti-E and anti-c in his/her serum in Severance hospital over a 12 year period (1985-1996). During the 12-year period of study, 53 patients were identified with anti-E and/or anti-c; 30(56.6%) patients had anti-E alone, 6(11.3%) had anti-c, and 17(32.1%) had both. Thirty eight of forty two patients whose Rh-hr phenotypes were tested were R1R1. Of these 38 R1R1 patients, 16 patients (42.1%) presented with anti-c concomitant with anti-E. Ouru study showed that the incidence of antni-c in R1R1 patients with anti-E is similar to that of studies reported in Caucasian groups. We highly suggest the transfusion protocol of prophylactic use of c negative blood for R1R1 patients with anti-E. Furthermore, because anti-c may be present in concentrations too low to be detected, the enzyme technique is recommended in parallel with standard serologic methods for the identification of irregular antibodies.
Antibodies ; Blood Banks ; Blood Group Incompatibility ; Humans ; Incidence ; Infant, Newborn ; Phenotype

Cutaneous metastasis from renal cell carcinoma is not unusual, occurring in 3 to 7%, although it may be overlooked. We report a case of metastatic renal cell carcinoma in a 74-year-old man who was presented with a 1.5 x 1.5 x 0.5 cm-sized nodule on the right zygoma for 3 months. A skin biopsy demonstrated the typical clear cell adenocarcinoma with PAS(+) granules in the cytoplasm and honeycombed or glandular configuration, compatible with metastatic renal cell carcinoma. Kidney ultrasonogram and abdomina1 CT scanning showed a large cystic mass on the upper pole of the left kidney. Furthermore radiologic studies revealed multiple metastatic lesions in the lung, liver and brain. He died 4 months later.
Adenocarcinoma, Clear Cell ; Aged ; Biopsy ; Brain ; Carcinoma, Renal Cell* ; Cytoplasm ; Humans ; Kidney ; Liver ; Lung ; Neoplasm Metastasis ; Skin ; Tomography, X-Ray Computed ; Ultrasonography ; Zygoma*

BACKGROUND: The testes are one of the most common extramedullary sites of relapse in boys with acute lymphoblastic leukemia(ALL). The reported incidence of isolated testicular relapse varies from 3 to 40%. If these patients are treated exclusively with testicular irradialion, a systemic relapse occurs within a few months. Recently, the use of intensive chemotherapy and testicular irradiation improved the survival rate for boys with testicular leukemia. So, we performed this study to identify clinical manifestations, disease free survival and prognostic factors of testicular leukemia in children. METHODS: We reviewed 33 patients of testicular leukemia among total 410 boys with ALL diagnosed at the Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1970 to Aug. 1996. Testicular leukemia was confirmed by testicular biopsy in all 33 patients. These patients were treated with combined local testicular irradiation(2,400~2,500 cGy/8~12fractions) and systemic chemotherapy. Two patients, in whom testicular relapse was diagnosed before 1979, unilateral orchiectomy of the involved site and testicular irradiation of the opposite site were performed. Probability estimates of disease free survival (DFS) were calculated by the method of Kaplan and Meier, and the relationship of prognostic factors to DFS was compared using the chi-square test in survival analysis. RESULTS: In 410 boys with ALL, testicular leukemia occurred in 33 patients(8%). Of 33 patients, 6 patients presented with testicular involvement at initial diagnosis, 16 patients had testicular relapse while still receiving chemotherapy and 11 patients had testicular relapse 3 to 57 months(median : 15 months) after cessation of chemotherapy. The median age of 33 patients was 7.4 yrs(9 months~18 yrs) and median WBC count 7,600/ L(2,700~270,000/L). All patients presented with painless testicular enlargement and testicular leukemia was confirmed by testicular biopsy. Among 33 patients, 2 had prior CNS relapse and 11 had concomitant bone marrow and/or CNS relapse. Twenty nine patients were treated with combined local testicular irradiation and systemic chemotherapy. Eleven had second relapse(6 bone marrow, 3 CNS, 2 opposite testis). Seventeen have been followed until now: 6 patients on chemotherapy and 11 patients(37.9%) in complete remission for 48.5+/-22.3 months(19~86 months). The 3 year DFS for 29 patients was 55.3%+/-10.1%. The following prognostic factors showed no significant association with DFS in testicular relapse : age and WBC count at initial diagnosis, age at testicular relapse, and concomitant relapse. Whether testicular relapse occurred on initial therapy or off initial therapy has prognostic value in predicting DFS. The 3 year DFS for boys with testicular relapse on and off initial therapy were 40.0%+/-12.9% and 78.8%+/-13.4%, respectively(P: 0.046). CONCLUSION: With the use of chemotherapy and testicular irradiation, prolonged second re mission can be achieved in many patients with testicular leukemia. The patients with testicular relapse off initial therapy fared significantly better than patients on therapy. So, to improve the DFS for boys with testicular leukemia, a better understanding of its biology and prognostic factors is needed.
Biology ; Biopsy ; Bone Marrow ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Leukemia ; Missions and Missionaries ; Orchiectomy ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence ; Seoul ; Survival Rate ; Testis

Verrucous carcinoma is a low grade, well differentiated, unusual variant of squamous cell carcinoma. It has been described to represent an intermediate lesion between condyloma acuminata and squamous cell carcinoma. Since malignant transformation of verrucous carcinoma has been reported to occur in 30-50% of cases, the first line treatment for verrucous carcinoma is surgery, preferably Mohs surgery. Herein, we describe a case of recurrent verrucous carcinoma on the right buttock of a man with a history of radical excision and radiotherapy of a previous perianal verrucous carcinoma. The patient was successfully treated with 5% imiquimod cream, an immune response modifier with potential antiviral and antitumor effects. Imiquimod may be an effective treatment for verrucous carcinoma and presents an alternative therapy to surgical excision.
Buttocks ; Carcinoma, Squamous Cell ; Carcinoma, Verrucous* ; Humans ; Mohs Surgery ; Radiotherapy

No abstract available.
Neuroblastoma* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

No abstract available.
Heterotaxy Syndrome*

Hemorrhagic radiation proctitis is infrequently seen in patients receiving pelvic irradiation. The treatment of hemorrhagic ralation proctitis is often difficult. Many patients need reyeated hospitalizations and blood transfusions. Occasionally patiets will develop severe or recurrent rectal bleeding. In case of massive bleeding requiring excessive transfusion, colonic diversion by construction of a colostomy or excision of the diseased segment may be inevitable. However, surgical procedures in these patients are associated with a high mortality and morbidity. Moreover, medical therapy is usually ineffective. Recently formalin therapy has been introduced as a simple and effective treatment for hemorrhagic radiation proctitis. We experienced a 69 year-old woman patient who developed severe homorrhagic proctitis 1 year after radiotherapy for carcinoma of the cervix. She had not improved by conservative management and required blood tranafusions and repetitive hospitalizations. After local application of a 4% formalin solution in the diseased rectum under caudal anesthesia, the bleeding immediately stopped and the patient was discharged from the hospital, and no recurrence has been observed until now.
Aged ; Anesthesia, Caudal ; Blood Transfusion ; Cervix Uteri ; Colon ; Colostomy ; Female ; Formaldehyde* ; Hemorrhage ; Hospitalization ; Humans ; Mortality ; Proctitis* ; Radiotherapy ; Rectum ; Recurrence