We describe a case of polyposis coli, which was followed by development of desmoid in the rectus adbominis muscle and fibromas in the mesentery during an interval of two years. This case supports the hypothesis that, in Garder's syndrome, the traumatic injury by colectomy triggers an unusual fibrous proliferation in the peritoneal cavity and incision site under the possible genetic basis.

No abstract available.

Overexpression of the nuclear phosphoprotein p53 is the most common genetic anomaly found in primary human cancer and mutation of the tumor suppressor gene p53 has been identified in breast cancer cell lines. In this study, we evaluated the prognostic significance of p53 protein expression in patients with mammary infiltrating ductal carcinoma and its correlation with histopathologic grade, lymph node status, tumor size, p53 protein expression and survival. Among 53 cases, p53 protein expression was detected in 26(49.1%) cases by immunohistochemistry. There was no correlation between p53 protein overexpression and histopathologic grade(p=0.09) or lymph node status(p=0.38) and between survival and histopathologic grade (p=0.68) or lymph node status(p=0.52). However, p53 protein expression was significantly correlated with survival(p=0.01) and patients with p53 protein-positive tumors showed poorer survival times. But Cox multivariate analysis showed the lymph node status is significant(p=0.01). The authors conclude that the presence of mutant p53 protein and lymph node status may serve a prognostic role, in a subset of mammary infiltrating ductal carcinoma cases.
Humans ; Genes, Tumor Suppressor ; Breast Neoplasms

PURPOSE: To analyze the variable MRI features and clinical significance of intracranial cavernous realformation. MATERIALS AND METHODS: Forty patients(mean age 35.4) with cavernous malformation were evaluated by MRI. Eleven patients were surgically confirmed. Cavernous malformations were divided into four categories on the basis of the MR imaging characteristics, especially on T2-weighted image. Type I lesion was defined as an extralesional subacute hemorrhage outside the low signal rim, type II as an intralesional hemorrhage surrounded by low signal rim, type III lesion as an intralesional thrombosis with variegated central core surrounded by low signal rim, and type IV lesion as a focal old hemorrhagic core with small low signal intensity. Type IV was further divided into IVa and IVb, whether the lesion has small iso- or hypersignal central core (IVa) or not (IVb). Follow-up MRI was evaluated in 12 patients who were managed conservatively. Follow-up intervals ranged from 2 weeks to 29 months (mean 6months). RESULTS: Total 80 lesions were detected in 40 patients. Multiple lesions were noted in 10 patients. The topography of the cavernous malformations was supratentorial in 75% and infratentorial in 23%. There were 10 lesions in type I, 15 in type 11, 21 in type III, 14 in type IVa, and 20 in type IVb. Type I lesions mainly showed mass effect and edema. Type III lesions showed minimal contrast enhancements in 7 lesions on delayed images. Type II lesions showed the characteristics of both type I and type III lesions. On follow up images, decrease in size in 5, change of type in 7, rebleeding in 2 and no change in 12 lesions were demonstrated. Hemorrhage, edema and mass effect were combined in the cases of rebleeding. On follow-up study, the estimated risk of bleeding was 32.3%/person-year and 13.7%/lesion-year. CONCLUSION: Cavernous realformations show as variable appearance, on MR imaging suggesting variable stages of evolution. The MR morphologic classification and evaluation of secondary findings are helpful to predict natural course and possibility of rebleeding of the lesion.
Classification ; Edema ; Follow-Up Studies ; Hemorrhage ; Humans ; Magnetic Resonance Imaging* ; Thrombosis

This report describes the pathologic features of 17 cases of Castleman's disease, examined at the Department of Pathology, Seoul National University Hospital during a period from 1973 to 1989. The lesions in 12 cases were hyaline-vascular type and the remainders plasma cell type. The pathologic features favoring the plasma cell type over the hyaline vascular type included a sufficient number to large-sized follicles. However, a histologic overlapping between two types was present. In the hyaline vascular type the age of the patients ranged from 7 to 76 years and they appeared to be no particular sex predominence. The majority of the lesions occurred in the neck and within the chest. Almost all cases presented with a solitary mass except three cases. Neither conventional symptoms nor systemic manifestations were associated. The plasma cell type was characterized by presentation of constitutional symptoms, involvement of intra abdominal and inguinal lymphnodes, in association with unusual clinicopathologic features including IgA nephropathy, diabetes mellitus, systemic progressive sclerosis, peripheral neuropathy, and anemia. Immunohistochemical study was performed in three cases of the plasma cell type. Two cases revealed poly-clonal plasma cell infiltration. In a patient with IgA nephropathy, however, serum IgA was increase and a strong immunoreactivity to IgA heavy chain was found. Another case, associated with systemic progressive sclerosis and neuropathy, revealed monoclonal plasma cell infiltration (IgG and lambda light chain). The above results support a possibility that in some of the plasma cell type an altered immune mechanism is involved in its pathogenesis.

The congenital ball-and-socket ankle joint is a rare condition and is associated with congenital shortening of the lower extrimity and various skeletal abnormalities of the foot. This disease entity was reported in the German literature by Politzer in 1931 and in the English literature by Lamb in 1958. Several series have been reported since, suggesting that the condition may not be as rare as generally thought. This case is, to our knowledge, the first reported in this country.
Ankle Joint ; Ankle ; Foot

Eccrine spiradenoma is a txnign, painful tumor of the skin, which probably originates in the coil of the eccrine sweat glands and presents a characteristic clinical and pa.hological picture. However recently, the weakness and inconsistency of the enzymes histochemican, actions and electron microscopic findings, and the presence of numerous undifferentiated and indetirn, inate cells suggest a rather low degree of differentiation. We present herein four cases of eccrine spiradenoma which show livrse histopat.hologic characteristics including typical rosette-like structure, ductal structure, vacuar structure and mixed type, respectively. This report may support the premise that eccrine spiridinoma is defived not only from the eccrine duct but also from pluripotential stem cells.
Skin ; Stem Cells ; Sweat Glands

Lichen nitidus, characterized by its typical clinical and histopathologic findings, is an uncomrnon chronic dermatosis of unknowned etiology. The distribution of this disease is most often localized, but in some cases it may becorne generalized. Some authors suggested that lichen nitidus is a variant of lichen planus in terms of its coincidence, ultrastructural and immunophenot,ypic studies. We report herein a case of lichen nitidus which presents dark brown linear, grouped, or confluent papules on the face, cheat, and upper extremities, accompanying with the histopathologic findings of pigmentary incontirence in the portion of claw clutching a ball appearance. This report suggests that lichen nitidus shows hyperpigmentation clinically and could be one of the dermatoses which exhibit pigmentavy incontinence in a histopathologic study.
Animals ; Hoof and Claw ; Hyperpigmentation ; Lichen Nitidus ; Lichen Planus ; Skin Diseases ; Upper Extremity

PURPOSE: We conducted a phase II study in previously untreated patients with unresectable stage IIIB or IV non-small cell lung cancer to evaluate the response rate and toxicity of the combination chemotherapy regimen of etoposide, ifosfamide and cisplatin. MATERIALS AND METHODS: From September 1993 to December 1996, twenty patients with advanced non-small cell lung cancer (stage IIIB 5 and IV 15) (squamous cell 8, adeno- carcinoma 12), were enrolled in this study. There were 13 (65%) males and 7 (35%) females, and median age of patients were 56 years (range: 34~66). Eighteen patients had performance status (ECOG) 0~1, two patients had performance status 2. Treatment was consisted of cisplatin (20 mg/m2 i.v., day 1~4), VP-16 (etoposide) (75 mg/m2 i.v., day 1~4), ifosfamide (1000 mg/m2 i.v., day 1~4) with mesna. This treatment was repeated every four weeks. RESULTS: The overall response rate was 25%. Complete response rate was 5% (1/20) and partial response rate was 20% (4/20). The median cycle of response was 4 (2~6) cycles. The median overall survival time was 28 weeks (9~98 weeks). The median time to progression was 10 weeks (3~50 weeks). Toxicities were evaluated by WHO criteria. Toxicity > GradeIII included: leukopenia 1.6%, thrombocytopenia 3.2%, nausea and vomiting 15%, alopecia 30%, stomatitis 10%. These toxicities were tolerable and reversible. CONCLUSION: VIP regimen was not superior to previous regimens for advanced non-small all lung cancer, and the toxicities were tolerable.
Alopecia ; Carcinoma, Non-Small-Cell Lung ; Cisplatin* ; Drug Therapy, Combination* ; Etoposide ; Female ; Humans ; Ifosfamide* ; Leukopenia ; Lung Neoplasms ; Male ; Mesna ; Nausea ; Small Cell Lung Carcinoma* ; Stomatitis ; Thrombocytopenia ; Vomiting

BACKGROUND: It is now thought that the earliest manifestation of idiopathic pulmonary fibrosis is alveolitis, that is, an accumulation of inflammatory and immune effector cells within alveolar walls and spaces. Inflammatory cells including alveolar macrophages and resident normal pulmonary tissue cells participate through the release of many variable mediators such as inflammatory growth factors and cytokines, which contribute to tissue damage and finally cause chronic pulmonary inflammation and fibrosis. This study was performed to investigate the source and distribution pattern of transforming growth factor-beta1(TGF-beta1), platelet derived growth factor(PDGF), basic fibroblast growth factor(bFGF), interleukin 1(IL-1), interleukin 6(IL-6), tumor necrosis factor-alpha(TNF-alpha) and the role of these mediators on bleomycin(BLM)-induced pulmonary injury and fibrosis in rats. METHOD: Wistar rats were divided into three groups(control group, BML treated group, BML and vitamine E treated group). Animals were sacrifices periodically at 1, 2, 3, 4, 5, 7, 14, 21, 28 days after saline or BLM administration. The effects were compared to the results of bronchoalveolar lavage fluid analysis, light microscopic findings, immunohistochemical stains for six defferent mediators(TGF-beta1, PDGF, bFGF, IL-1, IL-6 and TNF-alpha) and mRNA in situ hybridization for TGF-beta1. RESULTS: IL-1 and IL-6 are maximally expressed at postbleomycin 1~7th day which are mainly produced by neutrophils and bronchiolar epithelium. It is thought that they induce recruitment of inflammatory cells at the injury site. The expression of IL-1 and IL-6 at the bronchiolar epithelium within 7th day is an indirect evidence of contribution of bronchiolar epithelial cells to promote and maintain the inflammatory and immune responses adjacent to the airways. TNF-alpha is mainly produced by neutrophils and bronchiolar epithelial cells during 1~5th day, alveolar macrophages during 7~28th day. At the earlier period, TNF-alpha causes recruitment of inflammatory cells at the injury site and later stimulates pulmonary fibrosis. The main secreting cells of TGF-beta1 are alveolar macrophages and bronchiolar epithelium and the target is pulmonary fibroblasts and extracellular matrix. TGF-beta1 and PDGF stimulate proliferation of pulmonary fibroblasts and TGF-beta1 and bFGF incite the fibroblasts to produce extracellular matrix. The vitamine E and BLM treated group shows few positive cells(p<0.05). CONCLUSION: After endothelial and epithelial injury, the neutrophils and bronchiolar epithelium secrete IL-1, IL-6, TNF-alpha which induce infiltration of many neutrophils. It is thought that variable enzymes and O2 radicals released by these neutrophils cause destruction of normal lung architecture and progression of pulmonary fibrosis. At the 7~28th day, TGF-beta1, PDGF, bFGF, TNF-alpha secreted by alveolar macrophages sting pulmonary fibroblasts into proliferating with increased production of extracellular matrix and finally, they make progression of pulmonary fibrosis. TNF-alpha compares quite important with TGF-beta1 to cause pulmonary fibrosis. Vitamine E seems to decrease the extent of BLM induced pulmonary fibrosis.
Animals ; Bites and Stings ; Bleomycin* ; Blood Platelets ; Bronchoalveolar Lavage Fluid ; Coloring Agents ; Cytokines* ; Epithelial Cells ; Epithelium ; Extracellular Matrix ; Fibroblasts ; Fibrosis ; Idiopathic Pulmonary Fibrosis ; In Situ Hybridization ; Intercellular Signaling Peptides and Proteins* ; Interleukin-1 ; Interleukin-6 ; Interleukins ; Lung ; Lung Injury ; Macrophages, Alveolar ; Necrosis ; Neutrophils ; Pneumonia ; Pulmonary Fibrosis* ; Rats ; Rats, Wistar ; RNA, Messenger ; Transforming Growth Factor beta ; Transforming Growth Factor beta1 ; Tumor Necrosis Factor-alpha ; Vitamins