The Carneys complex is a multisystemic tumorous disorder that features myxoma(heart, skin, and breast), spotty skin pigmentation(cutaneous and mucocutaneous), endocrine tumors(adrenal, testicular, and pituitary) and peripheral nerve tumors. The criteria far diagnosis of the complex is the presence of two or more of the above conditions. The condition is transmitted as an autosomal dominant trait. The most serious components of the syndrome are cardiac myxoma and psammomatous melanotic schwannoma. The cutaneous manifestations are a major clue in the disorder. We report a case of Carneys complex in a 19-year-old woman. She had spotty pigmentation on her face, left atrial myxoma, and myxoma on her right nipple. Her mother and sister had left atrial myxoma.
Diagnosis ; Female ; Humans ; Mothers ; Myxoma* ; Neurilemmoma ; Nipples ; Peripheral Nervous System Neoplasms ; Pigmentation ; Siblings ; Skin ; Young Adult

C-erbB-2 oncoprotein has been known to act as growth factor receptor responsible for the regulation of cellular growth, proliferation and differentiation and has been demonstrated in a number of cancers by immunohistochemical as well as matrix blotting techniques. Breast and ovarian cancer patients, whose tumor cells have amplification or overexpression of this oncoprotein, have been suggested to have worse prognosis. Yet, there are only a few studies on c-erbB-2 oncoprotein expression in transitional cell carcinoma(TCC) of the bladder. The aim of this study was to examine c-erbB-2 oncoprotein expression in bladder cancer to assess its potential as a useful prognostic marker in transitional cell carcinoma of the bladder. Deparaffinized tumor specimens from 42 patients with TCC of the bladder and 3 normal bladder tissue specimens were utilized. C-erbB-2 oncoprotein expression was detected by immunohistochemical analysis and then correlated with conventional prognostic variables such as histologic tumor grade, stage and DNA ploidy. In addition, we related the expression of c-erbB-2 oncoprotein to indicators of cellular proliferative activities such as proliferating cell nuclear antigen(PCNA), mean number of silver nucleolar organizer regions(AgNORs) per nucleus, flow cytometric S-phase fraction(CPF) and proliferation index(PI). The incidence of c-erbB-2 oncoprotein expression in Ash grade IV TCC of bladder was higher than that in Ash grade II and III (Chi-square test, p<0.05). The incidence of positive immunoreaction was higher in cases with muscle invasion and metastasis than in superficial tumors with statistical significance(p<0.05). In addition, statistical significant correlation was noted between c-erbB-2 oncoprotein expression and PCNA expression rate. But there were no significant differences in c-erbB-2 oncoprotein expression to DNA ploidy, PI nor SPF by flow cytometry and mean number of AgNORs per nucleus. The results of this study suggests that the c-erbB-2 oncoprotein together with other predictive parameters may serve to provide a phenotypic profile which permits more accurate forecasting of bladder cancer behavior and may prove to be useful in the future as an important guide to specific anti-tumor therapy.
Breast ; Carcinoma, Transitional Cell* ; DNA* ; Flow Cytometry ; Forecasting ; Humans ; Incidence ; Neoplasm Metastasis ; Nucleolus Organizer Region* ; Ovarian Neoplasms ; Ploidies* ; Prognosis ; Proliferating Cell Nuclear Antigen* ; Silver ; Urinary Bladder Neoplasms ; Urinary Bladder*

BACKGROUND: The catalytic subunit of telomerase, hTERT (telomerase reverse transcriptase), is one of the most important components of telomerase, and performs a pivotal role in the mechanism underlying the regulation of telomerase activity in cellular immortalization and carcinogenesis. The principal objective of this study was to investigate hTERT expression in patients with non-small cell lung carcinomas (NSCLCs), and to evaluate its clinical significance and association with the expression of p16 and p53. METHODS: Using tissue microarray, the protein expression profiles of hTERT, p16 and p53 were investigated via immunohistochemistry in 167 samples of NSCLCs. RESULTS: Expression was observed in 54.5% (91/167) of the tumors, which were predominantly squamous cell carcinomas. Patients evidencing hTERT expression in their tumors exhibited significantly poorer survival rates than did patients without hTERT expression in early-stage NSCLCs (p=0.0125). According to the results of our Cox regression analysis, hTERT expression proved to be an independent prognostic factor (p=0.006), particularly for squamous cell carcinomas (p=0.019). hTERT expression was not correlated with p16 expression, but was rather associated with the expression of p53 (p=0.002). CONCLUSIONS: Our results show that hTERT may perform a function in the progression of NSCLC, and that its detection may be useful in predicting the prognosis of NSCLC patients in the early stages of the disease, as well as in the development of a targeted therapy in these tumors.
Carcinogenesis ; Carcinoma, Non-Small-Cell Lung ; Carcinoma, Squamous Cell ; Catalytic Domain ; Humans ; Immunohistochemistry ; Lung* ; Prognosis ; Survival Rate ; Telomerase*

Trichoblastic fibroma is a benign trichogenic tumor that has both epithelial and mesenchymal components and exhibits partial follicular induction. We studied 4 cases of trichoblastic fibroma and reviewed their clinical and histologic features. Two tumors were present in the face. The remaining two were in the vulva and perianal area, respectively. The age of the patients ranged from 53 to 68 years, with an average age of 62. All were female. Histologically, the lesions showed a well circumscribed mass, located at dermo-subcutaneous junction in three patients and subcutaneous in one. They demonstrated mesenchymal induction evidenced by hair germ-like structure and perifollicular sheath. There was no connection between the tumor and epidermis. Differentiation toward hair structure led to the formation of the infundibulum through inner root sheath. Trichoblastic fibroma may be confused clinically and/or histologically with basal cell carcinoma. Identification of the mixed epithelial and mesenchymal components, and the absence of epidermal connection and cleft within the stroma are important in differentiating this benign neoplasm from basal cell carcinoma.
Carcinoma, Basal Cell ; Epidermis ; Female ; Fibroma* ; Hair ; Humans ; Vulva

BACKGROUND: The claudins are a family of transmembrane proteins associated with tight junctions and they are critical for maintaining cell-to-cell adhesion in sheets of epithelial cells. However, their role in the progression of cancer remains largely unexplored. The aims of this study were to evaluate the expression patterns of claudin-1 and -4 in benign lesions and invasive ductal carcinomas (IDC) of the breast, and relationships between the expression of these markers and the clinicopathological characteristics in IDC patients. METHODS: We examined the claudin-1 and -4 protein expressions by performing immunohistochemical stainings in 54 benign lesions and 120 IDCs via the tissue microarray method. We evaluated the correlation between the expression of these markers and the clinicopathological characteristics of IDC. RESULTS: The expressions of claudin-1 (p=0.099) and -4 (p=0.000) were up-regulated in IDCs as compared with benign lesions. The claudin-1 expression correlated with the loss of estrogen receptor (p=0.036) and progesterone receptor (p=0.011). The claudin-4 expression correlated with lymph node metastasis (p=0.043), the nuclear grade (p=0.030), the histologic grade (p=0.007), and the loss of estrogen receptor (p=0.001) and progesterone receptor (p= 0.029). CONCLUSIONS: These results suggest that claudin-1 and -4 may play a significant role in the carcinogenesis of IDC of the breast and these may represent novel markers for this disease.
Breast* ; Carcinogenesis ; Carcinoma, Ductal* ; Claudin-1* ; Claudin-4 ; Claudins ; Epithelial Cells ; Estrogens ; Humans ; Immunohistochemistry ; Lymph Nodes ; Neoplasm Metastasis ; Receptors, Progesterone ; Tight Junctions

We managed 118 patients with benign prostatic hyperplasia by transurethral resection during January 1994 and December 1995 in Eul Ji Medical Center and all patients were operated upon by 2 staff doctors. Among 118 cases, 7 cases with intraoperative complications such as bladder injury or severe prostatic capsule injury and 8 cases with combined neuropathic bladder or chronic urinary retention and 2 cases with urethral stricture were excluded. The average age of 101 cases were 66.4 years old (50-92), and average weight of resected adenoma was 10.0 gm (2-32). We categorized these patients randomly into 2 groups. In group I (n=48), we removed the urethral catheter within 2 days after TURP and in group II (n=53), we removed it after 3 days (mean 4.2 days) following surgery. The average hospital stay postoperatively was4.1 days in group I and 7.4 days in group II. Recatheterization rate was 8.3% (4/48) in group I and 5.6% (3/53) in group II (p>0.05). There was no statistical difference in other complication including failure to void, intraoperative bleeding, urethral stricture, incontinence and TUR syndrome. This study supports early catheter removal after TURP would become an accepted and routine postsurgical practice following uncomplicated TURP.
Adenoma ; Catheters* ; Hemorrhage ; Humans ; Intraoperative Complications ; Length of Stay ; Prospective Studies* ; Prostatic Hyperplasia ; Transurethral Resection of Prostate* ; Urethral Stricture ; Urinary Bladder ; Urinary Bladder, Neurogenic ; Urinary Catheters ; Urinary Retention

Congenital lipoid adrenal hyperplasia, the most severe form of congenital adrenal hyperplasia, is caused by mutations in the steroidogenic acute regulatory protien (StAR). It is characterized by failure of synthesis of all three classes of adrenal steroids and massive accumulation of lipids and cholesterol in the adrenal cortex. The computed tomography (CT) unequivocally delineated massively enlarged adrenal glands of fat-tissue attenuation, enabling early diagnosis and replacement therapy. We report a case of congenital lipoid adrenal hyperplasia, in which CT established that lipoid deposition at the adrenal cortex disappeared after the adrenal hormone replacement therapy.
Adrenal Cortex ; Adrenal Glands ; Adrenal Hyperplasia, Congenital ; Cholesterol ; Early Diagnosis* ; Hormone Replacement Therapy ; Hyperplasia* ; Steroids

Multiple fixation techniques for arthrodesis of the ankle and hindfoot have been introduced. The cannulated screw fixation provides excellent internal compression for arthrodesis. We used 5.0 and 6.5mm cannulated screws for seven ankle, seven subtalar, four tibiotalocalcaneal, three pantalar and two triple arthrodesis between January 1994 and September 1995. No iliac bone graft was utilized. The follow-up period was from 12 to 25 months. Ages of the patients ranged from 25 to 58 years. All fused except one who was preoperatively diagnosed as pyogenic arthritis of the ankle and hindfoot. One patient with diagnosis of Streeter's disease was complicated with partial necrosis of the forefoot. We think this procedure has several advantages; 1. The leg or foot length is largely preserved because minimal subchondral bone is removed. So, the leg and foot is aesthetically pleasing and the musculature around the ankle and foot can be maintained in the normal balanced position. 2. The rate of fusion is high, because the soft tissues are minimally stripped and cannulated screw compresses the subchondral bone surface. 3. The cannulated screws rarely requires removal. So, we believe that the cannulated screw fixation for the ankle and hindfoot arthrodesis is an excellent treatment method.
Ankle* ; Arthritis ; Arthrodesis* ; Diagnosis ; Follow-Up Studies ; Foot ; Humans ; Leg ; Necrosis ; Transplants

Fine needle aspiration of the breast is an important diagnostic tool in malignant lesions, but is also useful in differentiation of inflammatory breast diseases mimicking carcinoma clinically and radiologically. Recently, the authors have experienced eight biopsy-proven cases of chronic inflammatory diseases of the breast, which consisted of 4 cases of duct ectasia, 2 cases of fat necrosis, and a case of tuberculous mastitis and granulomatous mastitis respectively, Their cytoiogic features mainly based on the components and the relative frequency of inflammatory cells were evaluated for differential diagnosis of chronic inflammatory breast diseases. The results are as follows ; 1. In cases of duct ectasia, varying amount of neutrophils, mononuclear leukocytes, histiocytes and multinucleated giant cells were intermixed with benign epithelial cell clusters. 2, Abundant fat tissue fragments were diagnostic for fat necrosis. Histiocytes and mononuclear cells were main components but not rich, and neutrophils and giant cells were infrequently observed. 3. Characteristic granulomas composed of epithelioid cells, mononuclear leukocytes and Langhans' type giant cells and lymphocytic infiltrates were conspicuous in tuberculous mastitis, and occasionally neutrophils, necrotic materials and epithelial cell clusters were found. 4. In granulomatous mastitis, epithelioid cell granulomas were also noted but numerous neutrophils and histiocytes were intermingled within or outside the granulomas.
Biopsy, Fine-Needle ; Breast ; Breast Diseases ; Child* ; Diagnosis, Differential ; Dilatation, Pathologic ; Epithelial Cells ; Epithelioid Cells ; Fat Necrosis ; Female ; Giant Cells ; Granuloma ; Granulomatous Mastitis ; Histiocytes ; Humans ; Leukocytes, Mononuclear ; Mastitis ; Mothers* ; Neutrophils ; Salivary Glands

In a six-year period(1988. 5-1994. 4), fine needle aspiration cytology(FNAC) of 322 pulmonary lesions from 296 patients were performed at Soonchunhyang University Hospital. Of these 322, malignancy was diagnosed cytologically in 139(43.2%), suspicious malignancy in 7(2.2%), negative in 164(50.8%), and insufficient material in 12(3.8%). Malignant lesions consisted of 54 cases of adenocarcinoma, 50 cases of squamous cell carcinoma, 18 cases of small cell carcinoma. They were verified by histologic examination in 70 cases. There were 2(0.6%) false positive cases due to florid bronchoalveolar hyperplasia and atypical bronchial epithelial cells associated with granulomatous lesion. The overall accuracy rate was 90%, the sensitivity 84.3% and the specificity 94.7%.
Adenocarcinoma ; Biopsy, Fine-Needle ; Carcinoma, Small Cell ; Carcinoma, Squamous Cell ; Child* ; Epithelial Cells ; Humans ; Hyperplasia ; Multicystic Dysplastic Kidney* ; Sensitivity and Specificity ; Tuberculosis, Pulmonary