Purpose: Epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors have greatly improved survival in EGFR-mutant (EGFRm) non–small cell lung cancer (NSCLC); however, their effects on the tumor microenvironment (TME) are unknown. We assessed the changes induced by neoadjuvant erlotinib therapy (NE) in the TME of operable EGFRm NSCLC. Materials and Methods: This was a single-arm phase II trial for neoadjuvant/adjuvant erlotinib therapy in patients with stage II/IIIA EGFRm NSCLC (EGFR exon 19 deletion or L858R mutations). Patients received up to 2 cycles of NE (150 mg/day) for 4 weeks, followed by surgery and adjuvant erlotinib or vinorelbine plus cisplatin therapy depending on observed NE response. TME changes were assessed based on gene expression analysis and mutation profiling. Results: A total of 26 patients were enrolled; the median age was 61, 69% were female, 88% were stage IIIA, and 62% had L858R mutation. Among 25 patients who received NE, the objective response rate was 72% (95% confidence interval [CI], 52.4 to 85.7). The median disease-free and overall survival (OS) were 17.9 (95% CI, 10.5 to 25.4) and 84.7 months (95% CI, 49.7 to 119.8), respectively. Gene set enrichment analysis in resected tissues revealed upregulation of interleukin, complement, cytokine, transforming growth factor β, and hedgehog pathways. Patients with upregulated pathogen defense, interleukins, and T-cell function pathways at baseline exhibited partial response to NE and longer OS. Patients with upregulated cell cycle pathways at baseline exhibited stable/progressive disease after NE and shorter OS. Conclusion NE modulated the TME in EGFRm NSCLC. Upregulation of immune-related pathways was associated with better outcomes.

Consequences of an untreated sleep related breathing disorder include failure to thrive, enuresis, attention deficit disorder, behavior problems, poor academic performance, and cardiopulmonary disease. The most common etiology of sleep related breathing disorder in children is adenotonsillar hypertrophy. Other upper airway lesion should be evaluated. We experienced a case of a child whose etiology of sleep related breathing disorder was suspected to have been caused by a tubal tonsil hypertrophy. Therefore, in this paper, we present a case of a sleep related breathing disorder in a child which was successfully treated by removing the tubal tonsil hypertrophy.
Attention Deficit Disorder with Hyperactivity ; Child* ; Enuresis ; Failure to Thrive ; Humans ; Hypertrophy* ; Palatine Tonsil ; Respiration ; Sleep Apnea, Obstructive*

Intestinal lymphangiectasia is characterized by protein- losing enteropathy, and is diagnosed by a small bowel biopsy demonstrating dilated lymphatics in the mucosa, submucosa and serosa in the absence of coexistent inflammation. We report a case of primary intestinal lymphangiectasia that occurred in a 2-year-6-month-old girl who was treated successfully with antiplasmin and octreotide. Initially, the patient was treated with a lipid restriction diet with medium chain triglyceride oil, but her symptoms were not relieved. This case shows that antiplasmin and octreotide therapy might be useful for treating refractory primary intestinal lymphangiectasia.
Biopsy ; Diet ; Female ; Humans ; Inflammation ; Mucous Membrane ; Octreotide* ; Serous Membrane ; Triglycerides

BACKGROUND/AIMS: Barrett's esophagus is a premalignant lesion of the esophagus in which normal squamous epithelium is replaced by intestinalized columnar epithelium. In Korea, adenocarcinoma associated with Barrett's esophagus is rare compared with that of Western country. The purpose of this study was to investigate the immunohistochemical expression of p53 and Ki-67 in Barrett's esophagus which had predictive value for cancer risk in Korea. METHODS: Ninety five patients (43 male and 52 female, median age 44, range 21-75) who have been suspected to have Barrett's esophagus by endoscopic assessment were enrolled in this study. Alcian blue (pH 2.5) and high ion diamine stain for the evaluation of specialized intestinal metaplasia (SIM) and immunohistochemical stain for p53 and Ki-67 were done. RESULTS: 57.9% (55/95) of biopsies from the columnar lined esophagus showed SIM, but no dyspalsia. 56.4% (31/55) of Barrett's esophagus showed sulfomucin positive colonic metaplasia. The p53 expression was observed in 10.9% (6/55) of the patients of Barrett's esophagus and all of them showed colonic metaplasia. Ki-67 labeling index showed no difference significantly. CONCLUSIONS: In Korea, 10.9% of Barrett's esophagus had p53 mutation and moreover all of them had colonic metaplasia. Consequently, we expect that these patients have high risk of developing dysplasia and adenocarcinoma and need careful follow-up.
Adenocarcinoma/etiology/genetics ; Adult ; Aged ; Barrett Esophagus/complications/*metabolism ; Esophageal Neoplasms/etiology/genetics ; Female ; Humans ; Immunohistochemistry ; Ki-67 Antigen/*metabolism ; Male ; Middle Aged ; Risk Factors ; Tumor Suppressor Protein p53/*metabolism

Achalasia is an uncommon esophageal motility disorder in which affected patients present with progressive dysphagia. Various causes were known in this disease. Esophageal achalasia is diagnosied by barium esophagogram, endoscopy and esophageal manometry. Treatment of esophageal achasia are pharmacotherapy, pneumatic dilatation, or botulinum toxin injection and surgical therapy. Balloon dilatation is a safe effective first line treatment. We report two cases of achalasia with normal lower esophageal sphincer pressure, typical endoscopic and typical esophagogram findings, which was treated successfully with pneumatic balloon dilatation.
Barium ; Botulinum Toxins ; Deglutition Disorders ; Dilatation ; Drug Therapy ; Endoscopy ; Esophageal Achalasia* ; Esophageal Motility Disorders ; Esophageal Sphincter, Lower* ; Humans ; Manometry

BACKGROUND/AIMS: [18F]FDG-PET is a functional imaging modality reflecting cellular glucose metabolism. In most malignant cells, accumulation and trapping of [18F]FDG allows the visualization of increased uptake compared with normal cells. The aim of this study was to assess the value of PET in differentiating benign from malignant hepatic lesions and to determine in which types of hepatic tumors PET can help evaluate stage, monitor response to therapy, and detect recurrence. METHODS: Eighty patients with liver lesions were enrolled (hepatocellular carcinoma 34, cholangiocarcinoma 8, metastatic liver cancer 25, hemangioma 6, liver abscess 7). Liver metastases were 22 adenocarcinoma, 2 lymphoma, 2 squamous cell carcinoma. The PET images of these patients were analyzed. SUV and lesion-to-normal liver background SUV ratio were obtained and compared among the disease groups. RESULTS: All liver metastases and all cholangiocarcinomas had increased uptake value, with SUV ratios greater than 2. Hepatocellular carcinoma had SUV ratios greater than 2 in 20 of 34 patients (59%). All hemangiomas had poor uptake, a SUV ratio of less than 2. All liver abscesses showed definite uptake. CONCLUSIONS: The PET technique using FDG static imaging was useful in differentiating malignant from benign lesions of the liver in limited situations. Limitations included false negative results in some patients with hepatocellular carcinoma. Liver abscesses raised problems in differential diagnosis from malignant liver tumors. The findings of this study suggest that the PET technique might be applied in tumor staging and the detection of recurrence, as well as monitoring responses to therapy for all adenocarcinomas and some hepatocelluar carcinomas.
Adult ; Aged ; Diagnosis, Differential ; English Abstract ; Female ; Fludeoxyglucose F 18/diagnostic use ; Human ; Liver Diseases/*radionuclide imaging ; Liver Neoplasms/radionuclide imaging ; Male ; Middle Aged ; Radiopharmaceuticals/diagnostic use ; *Tomography, Emission-Computed

OBJECTIVE: The purpose of this study was to review the incidence, clinicopathological feature, treatment, outcome in the series of childhood and adolescence with ovarian mass PATIENTS AND METHODS: Retrospective reviews of the medical recordings for 116 patients with ovarian mass under the age of twenty years old who were admitted, operated and confirmed with histopatholgical study at the department of obstetric and gynecology, Gachon medical school, Inchon, Korea for 8 years from Jan. 1993 till Dec. 2000. RESULTS: Of 116 the patients who underwent surgical treatment, the incidence of malignant ovarian tumors was 15 cases(12.9%). If ovarian neoplasm alone are considered, the rate of malignancy increases to 17.6%. The frequency of ovarian malignancies correlated inversely with patient age. In the 0-10 age group ,40% had malignancies, as compared with 20.3% in the 11-15 age group and 15.3% in the 16-20 age group. On histopathological classification, the tumors originated from germ cell tumors were 55.2%, epithelial cell tumors were 40%, and sex-cord stromal tumors were 4.7%. According to FIGO classification of malignant ovarian tumor, stage I(80%) was most common, followed stage IV(13.3%), stage II(6.6%) and III(0%). 13 of the 15 malignant ovarian tumors, unilateral salpingoophorectomy was done in an attempt for reproductive organ conservation; 2 cases of stage IV disease were treated with hystrectomy, bilateral salpingoophorectomy and omentectomy. On follow up, only two ovarian malignancy stage IV died within 3 months and another is well. CONCLUSION: The frequency with which ovarian meoplasms malignancy in the under 20 age group is 17.6%. Because of their malignant potential in young girl, prompt evaluation and treatment is imperative.
Adolescent* ; Classification ; Epithelial Cells ; Female ; Follow-Up Studies ; Gynecology ; Humans ; Incheon ; Incidence ; Korea ; Medical Records ; Neoplasms, Germ Cell and Embryonal ; Ovarian Neoplasms ; Retrospective Studies ; Schools, Medical

A 63-year-old woman presented to the hospital with gross hematuria and acute renal failure. Kidney function deteriorated rapidly and progressively. A renal biopsy revealed segmental or circumferential crescents associated with linear deposits of immunoglobulin G, typical of anti-glomerular basement membrane disease. Both c-ANCA and anti-GBM antibody were detected in serum. She was treated with hemodialysis, plasmapheresis, high dose steroid and cyclophosphamide. However, she died 7 weeks after treatment because of pneumonia, without recovery of renal function. Serologic positivity of both ANCA and anti-GBM antibody are becoming more frequently recognized in rapidly progressive glomerulonephritis. The influence of c-ANCA on the clinical course of anti-GBM glomerulonephritis remains to be determined.
Acute Kidney Injury ; Anti-Glomerular Basement Membrane Disease ; Antibodies, Antineutrophil Cytoplasmic ; Basement Membrane* ; Biopsy ; Cyclophosphamide ; Cytoplasm* ; Female ; Glomerulonephritis* ; Hematuria ; Humans ; Immunoglobulin G ; Kidney ; Middle Aged ; Plasmapheresis ; Pneumonia ; Renal Dialysis

BACKGROUND: Paradoxical puborectalis syndrome (PPS) is a complex and poorly understood entity characterized by contraction rather than relaxation of the pelvic floor muscles during attempted evacuation. Anal electromyography (EMG) and cinedefecography (CD) are the most commonly used tests for the diagnosis of PPS. Therefore, the aim of this study was to prospectively assess the correlation of EMG and CD in the diagnosis of PPS. METHODS: All patients with symptoms of obstructed evacuation who underwent EMG and CD between September 1998 and February 1999, were evaluated. The clinical criteria for PPS included incomplete or difficult evacuation, straining, tenesmus, and the need for an enema or digitation. The EMG criteria included failure to achieve a significant decrease in the electrical activity of the puborectalis during attempted evacuation. The CD criteria included either paradoxical contraction or failure of the puborectalis to relax along with incomplete evacuation. RESULTS: Twenty-nine (29) patients had clinical evidence of PPS, 8 males and 21 females with a mean age of 42.6 years (range, 19-75 years), and underwent CD and EMG. The mean duration of symptoms was 7.2 years (range, 3 months-30 years). Nineteen patients (65.5%) had evidence as having PPS on CD and/or EMG. Of these patients, six patients (20.7%) were diagnosed as having PPS on both tests, five patients (17.2%) were only diagnosed on CD, and eight patients (27.6%) were only diagnosed on EMG. The remaining ten patients had normal puborectalis muscle relaxation on CD and EMG. Therefore, the correlation rate between the two tests was 55.2%. If EMG was considered as the ideal test for the diagnosis of PPS, CD had a sensitivity of 42.9% and a specificity of 66.7%. Conversely, if CD was considered as the ideal test, EMG had a sensitivity of 54.5% and a specificity of 55.6%. CONCLUSIONS: The sensitivity and the specificity values of EMG and CD for the diagnosis of PPS are suboptimal, and a low correlation existed between the two tests. This result suggests that the diagnosis of PPS should not be based upon only one test.
Constipation ; Diagnosis* ; Electromyography* ; Enema ; Female ; Humans ; Male ; Muscle Relaxation ; Muscles ; Pelvic Floor ; Prospective Studies ; Relaxation ; Sensitivity and Specificity

Acquired tumors of the nonpigmented ciliary epithelium are rare diseases. They are difficult to diagnose and present as pseudoadenomatous hyperplasia, adenoma or adenocarcinoma. They exhibit slowly progressive enlargement leading to a subluxated lens, cataract, and secondary glaucoma. And some of the more malignant tumors may extend extrasclerally. We report a case of adenoma of nonpigmented ciliary epithelium which produced a cataract.
Adenocarcinoma ; Adenoma* ; Cataract ; Epithelium* ; Glaucoma ; Hyperplasia ; Rare Diseases