Gonadoblastoma occurs almost always in association with a Y chromosome cell line, and developes in one third of patients with Mixed gonadal dysgenesis. Removing of gonads of intersex patients with the Y chromosome is very important because of the strong association of the genesis of tumor in dysgenetic gonads with the presence of a Y chromosome. But it is always possible that an XY cell line could be missed, or that a fragment from Y chromosome could have been translocated and not discovered by chromosomal analysis. PCR with Y specific probe or Southern blotting would reveal the presence of a Y or a translocated fragment. We experienced an 18-year-old woman represent with primary amenorrhea who had 45,X/46,X,+mar. Y-specific PCR revealed that the marker chromosome was drived from Y chromosome. After both gonadectomy and clitorial recession, we found the gonadoblastoma in dysgenetic testis. So we report it with brief review of literatures.
Adolescent ; Amenorrhea ; Blotting, Southern ; Cell Line ; Female ; Gonadal Dysgenesis, Mixed* ; Gonadoblastoma* ; Gonads ; Humans ; Polymerase Chain Reaction ; Testis ; Y Chromosome

PURPOSE: To evaluate the role of mediastinoscopy and CT in the preoperative nodal evaluation in patients with T1 lung cancer. MATERIALS AND METHODS: Between November 1994 and July 1996, 125 patients underwent thoracotomy and/or mediastinoscopy for surgical treatment of lung cancer. Among them, 35 patients had T1 lung cancer(peripheral lung cancer less than 3cm in diameter) on CT. One patient finally proved to have T4 lung cancer with pleural seeding at thoracotomy. In the remaining 34 patients, pathologic evaluation of mediastinal lymph nodemetastasis was feasible and the results were correlated with CT findings. On CT, nodes larger than 10mm in short-axis diameter were regarded as abnormal. RESULTS: The patients had adenocarcinoma in 12, squamous cellcarcinoma in 11, bronchioloalveolar carcinoma (BAC) in 10, and large cell carcinoma in one. Fifteen among total 478 sampled lymph nodes contained malignant tumor. Six (three with adenocarcinoma, two with squamous cell carcinoma, and one with large cell carcinoma) of 34 patients (18%) had nodal metastasis. With 112 sampled nodes, BAC did not show any nodal metastasis. Sensitivity and specificity of CT for nodal detection were 0% and 100% for2R, 0% and 100% for 4R, 100% and 97 % for 5, 50% and 100% for 7 and 0% and 100% for 10R, respectively. CONCLUSION: T1 lung cancer shows relatively high (18%) prevalence of mediastinal lymph node metastasis. Because small nodesless than 10mm in diameter contain malignancy and CT is insensitive in detection of metastatic nodes,mediastinoscopy is still needed for preoperative nodal evaluation except BAC.
Adenocarcinoma ; Adenocarcinoma, Bronchiolo-Alveolar ; Carcinoma, Large Cell ; Carcinoma, Squamous Cell ; Diagnosis* ; Humans ; Lung Neoplasms* ; Lung* ; Lymph Nodes ; Mediastinoscopy* ; Neoplasm Metastasis ; Prevalence ; Sensitivity and Specificity ; Thoracotomy

METHOD: 34 patients with a solitary pulmonary nodule less than 6 cm of its diameter who visited Samsung Medical Center from Semptember, 1994 to Semptember, 1995 were evaluated prospectively. Simple chest roentgenography, chest computer tomography, FDG-PET scan were performed for all patients. The results of FDG-PET were evaluated comparing with the results of final diagnosis confirmed by sputum study, PCNA, fiberoptic bronchoscopy, or thoracotomy. Results: (1) There was no significant difference in nodule size between malignant (3.1 1.5cm) and benign nodule(2.81.0cm)(P>0.05). (2) Peak SUV (standardized uptake value) of malignant nodules (6.93.7) was significantly higher than peak SUV of benign nodules(2.71.7) and time-activity curves showed continuous increase in malignant nodules. (3) Three false negative cases were found among eighteen malignant nodule by the FDG-PET imaging study and all three cases were nonmucinous bronchioloalveolar carcinoma less than 2 cm diameter. (4) FDG-PET imaging resulted in 83% sensitivity, 100% specificity, 100% positive predictive value and 84% negative predictive value. Conclusion: FDG-PET imaging is a new noninvasive diagnostic method of solitary pulmonary nodule that has a high accuracy of differential diagnosis between malignant and benign nodule. FDG-PET imaging could be used for the differential diagnosis of SPN which is not properly diagnosed with conventional methods before thoracotomy. Considering the high accuracy of FDG-PET imaging, this procedure may play an important role in making the dicision to perform thoracotomy in diffcult cases.
Adenocarcinoma, Bronchiolo-Alveolar ; Bronchoscopy ; Diagnosis ; Diagnosis, Differential ; Humans ; Proliferating Cell Nuclear Antigen ; Prospective Studies ; Radiography ; Sensitivity and Specificity ; Solitary Pulmonary Nodule* ; Sputum ; Thoracotomy ; Thorax

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a diffuse inflammatory and fibrosing process that occurs within the interstitium and alveolus of the lung with invariably poor prognosis. The major problem in management of IPF results from the variable rate of disease progression and the difficulties in predicting the response to therapy. The purpose of this retrospective study was to evaluate the shortterm efficacy of steroid and immunosuppressive therapy for IPF and to identify the pre-treatment determinants of favorable response. METHOD: Twenty patients of IPF were included. Diagnosis of IPF was proven by thoracoscopic lung biopsy and they were presumed to have active progressive disease. The baseline evaluation in these patients included clinical history, pulmonary function test, bronchoalveolar lavage (BAL), and chest high resolution computed tomography (HRCT). Fourteen patients received oral prednisolone treatment with initial dose of 1mg/kg/day for 8 to 12 weeks and then tapering to low-dose prednis olone (0.5mg/kg/day). Six patients who previously had experienced significant side effects to steroid received 2mg/kg/day of oral cyclophosphamide with or without low-dose prednisolone. Follow-up evaluation was performed after 6 months of therapy. If patients met more than one of followings, they were considered to be responders: (1)improvement of more than one grade in dyspnea index, (2)improvement in FVC or TLC more than 10% or improvement in DLco more than 20% (3) decreased extent of disease in chest HRCT findings. RESULT: One patient died of extrapulmonary cause after 3 month of therapy, and another patient gave up any further medical therapy due to side effect of steroid. Eventually medical records of 18 patients were analyzed. Nine of 18 patients were classified into responders and the other nine patients into nonresponders. The histopathologic diagnosis of the responders were all nonspecific interstitial pneumonia (NSIP) and that of nonresponders were all usual interstitial pneumonia (UIP) (p<0.001). The other significant differences between the two groups were female predominance (p<0.01), smoking history (p<0.001), severe grade of dyspnea (p<0.05), lymphocytosis in BAL fluid (23.8+/-16.3% vs 7.83+/-3.6%, p < 0.05), and less honeycombing in chest HRCT findings (0% vs 9.22+/-2.3%, p < 0.001). CONCLUSION: Our results suggest that patients with histopathologic diagnosis of NSIP or lymphocytosis in BAL fluid are more likely to respond to steroid or immunosuppressive therapy. Clinical results in large numbers of IPF patients will be required to identify the independent variables.
Biopsy ; Bronchoalveolar Lavage ; Cyclophosphamide ; Diagnosis ; Disease Progression ; Dyspnea ; Female ; Follow-Up Studies ; Humans ; Idiopathic Pulmonary Fibrosis* ; Lung ; Lung Diseases, Interstitial ; Lymphocytosis ; Medical Records ; Prednisolone ; Prognosis ; Respiratory Function Tests ; Retrospective Studies ; Smoke ; Smoking ; Thorax

PURPOSE: This is to evaluate the acute complication, resection rate, and tumor down-staging after pre-operative concurrent chemoradiotherapy for stage IIIA (N2) non-small cell lung cancer. MATERIALS AND METHODS: Fifteen patients with non-small cell lung cancer were enrolled in this study from May 1997 to June 1998 in Samsung Medical Center. The median age of the patients was 61 (range, 45~67) years and male to female ratio was 12:3. Pathologic types were squamous cell carcinoma (11) and adenocarcinoma (4). Pre-operative clinical tumor stages were cT1 in 2 patients, cT2 in 12, and cT3 in 1 and all were N2. Ten patients were proved to be N2 with mediastinoscopic biopsy and five had clinically evident mediastinal lymph node metastases on the chest CT scans. Pre-operative radiation therapy field included the primary tumor, the ipsilateral hilum, and the mediastinum. Total radiation dose was 45 Gy over 5 weeks with daily dose of 1.8 Gy. Pre-operative concurrent chemotherapy consisted of two cycles of intravenous cis-Platin (100 mg/m2) on day 1 and oral Etoposide (50 mg/m2/day) on days 1 through 14 with 4 weeks' interval. Surgery was followed after the pre-operative re-evaluation including chest CT scan in 3 weeks of the completion of the concurrent chemoradiotherapy if there was no evidence of disease progression. RESULTS: Full dose radiation therapy was administered to all the 15 patients. Planned two cycles of chemotherapy was completed in 11 patients and one cycle was given to four. One treatment related death of acute respiratory distress syndrome occurred in 15 days of surgery. Hospital admission was required in three patients including one with radiation pneumonitis and two with neutropenic fever. Hematologic complications and other acute complications including esophagitis were tolerable. Resection rate was 92.3% (12/13) in 13 patients excluding two patients who refused surgery. Pleural seeding was found in one patient after thoracotomy and tumor resection was not feasible. Post-operative tumor stagings were pT0 in 3 patients, pT1 in 6, and pT2 in 3. Lymph node status findings were pN0 in 8 patients, pN1 in 1, and pN2 in 3. Pathologic tumor down-staging was 61.5% (8/13) including complete response in three patients (23.7%). Tumor stage was unchanged in four patients (30.8%) and progression was in one (7.7%). CONCLUSION: Pre-operative concurrent chemoradiotherapy for Stage IIIA (N2) non-small cell lung cancer demonstrated satisfactory results with no increased severe acute complications. This treatment scheme deserves more patient accrual with long-term follow-up.
Adenocarcinoma ; Biopsy ; Carcinoma, Non-Small-Cell Lung* ; Carcinoma, Squamous Cell ; Chemoradiotherapy* ; Disease Progression ; Drug Therapy ; Esophagitis ; Etoposide ; Female ; Fever ; Follow-Up Studies ; Humans ; Lymph Nodes ; Male ; Mediastinum ; Neoplasm Metastasis ; Radiation Pneumonitis ; Respiratory Distress Syndrome, Adult ; Thoracotomy ; Tomography, X-Ray Computed

PURPOSE: To determine the accuracy of CT in the evaluation of mediastinal nodal metastases of non-small celllung cancer. MATERIALS AND METHODS: Between November 1994 and June 1997, 178 patients with non-small cell lung cancer underwent thoracotomy and full nodal sampling. The results of preoperative CT scanning and of pathologicexamination of regional lymph node metastases were compared. Each scan was prospectively interpreted by one chestradiologist. Mediastinal lymph nodes were localized according to the lymph node mapping scheme of the AmericanThoracic Society and were considered abnormal if they exceeded 10mm in short-axis diameter. All accessible nodeswere either removed or sampled during thoracotomy. RESULTS: Of the 178 non-small cell lung cancers, 90 weresquamous cell carcinoma, 60 were adenocarcinoma, 13 were brochioloalveolar carcinoma, ten were large cellcarcinoma, and five were others (basaloid, 1; sarcomatoid, 1; spindle cell, 1; adenosquamous cell, 2). A total of615 mediastinal nodal stations were obtained. The sensitivity of CT for the diagnosis of mediastinal nodemetastasis on a station-by-station basis was 21%, with a specificity of 93% (squamous cell carcinoma: 21% and 91%;adenocarcinoma: 20% and 95%, respectively). Sensitivities were higher for groups 7 and 5. In 13 bronchioloalveolarcarcinomas, no lymph node metastasis was found on either CT or pathologic examination. The sensitivity of CT forthe diagnosis of mediastinal node metastasis on a per-patient basis was 43%, with a specificity of 83%. CONCLUSION: Because of the relative insensitivity of CT for the detection of mediastinal lymph node metastasis, nodalsampling with mediastinoscopy or thoracotomy is essential in the staging work-up of non-small cell lung cancerother than bronchioloalveolar carcinoma.
Adenocarcinoma ; Adenocarcinoma, Bronchiolo-Alveolar ; Carcinoma, Non-Small-Cell Lung ; Diagnosis ; Humans ; Lung Neoplasms* ; Lung* ; Lymph Nodes* ; Mediastinoscopy ; Neoplasm Metastasis* ; Prospective Studies ; Sensitivity and Specificity ; Thoracotomy ; Tomography, X-Ray Computed