RESUMEN
PURPOSE: The N-myc amplification is one of well known poor prognostic markers in neurblastoma. Because the traditional detection method, Southern blot, is expensive, labor-intensive and time-consuming, the detection of N-myc amplification is not routinely performed in Korea. The purposes of this study are to develop polymerase chain reaction (PCR) for detecting N-myc amplification in neuroblastoma tumor tissue, and to elucidate the clinical significance of N-myc amplification. METHODS: The clinical data and paraffin embedded tumor specimen of 54 neuroblastoma cases were collected from 10 medical centers in Korea. We have developed semiquantitative method of estimating gene copy number that uses differential PCR. N-myc gene primers (RC N-myc, N-myc 7-1) are amplified together with primers from a single-copy internal control gene (beta-globin). After ethidium bromide-stained agarose gel electrophoresis, the ratio of the two PCR products, which stands for N-myc amplification, is determined. Kaplan-Meier survival analysis was performed to evaluate the prognostic significance of N-myc amplification. RESULTS: The differential PCR was very effective, less expensive, less labor-intensive, and simple detection method for N-myc amplification. The percentage of N-myc amplification was higher in the patients older than 1 year old (34.1%: 14/41), when they were compared to the patients younger than 1 year old (16.7%: 2/12). The percentage of N-myc amplification was higher in the patients who have primary tumor at adrenal gland (40.9%: 9/22) than who have primary tumor at retroperitoneum (17.6%: 3/17) or at mediastinum (16.7%: 2/12). In Stage I, II, and III patients, the mean survival time of N-myc amplified group was 18 months and that of N-myc umamplified group was 64 months (Log Rank 4.35, P=0.037). CONCLUSION: The differential PCR was very effective, less expensive, less labor-intensive, and simple detection method for N-myc amplification. The N-myc amplification is one of poor prognostic indicators in Neuroblastoma.
Asunto(s)
Humanos , Glándulas Suprarrenales , Southern Blotting , Electroforesis en Gel de Agar , Etidio , Dosificación de Gen , Genes myc , Corea (Geográfico) , Mediastino , Neuroblastoma , Parafina , Reacción en Cadena de la Polimerasa , Pronóstico , Tasa de SupervivenciaRESUMEN
The Duhamel procedure has been the authors' treatment of choice for children with Hirschsprung's disease over the last 15 years. Owing to advancements in instrumentation and technique, laparoscopic correction of some congenital anomalies, including congenital megacolon, have become possible. In March and May of 1997, laparoscopic Duhamel procedures were performed on a girl and boy aged 7 months and 2 years and 9 months, respectively. The primary diagnosis in both patients was Hirschsprung's disease, which was confirmed by barium-enema and mucosal suction biopsy. The boy did not need construction of a loop colostomy, while the girl had a colostomy made neonatally. Using 4 trocars, the sigmoid colon, proximal rectum, and posterior rectal wall were mobilized laparoscopically. Immdeiately after severing the proximal resection line, the proximal end was pulled down posterior to the dentate line for side-to-side anastomosis with GIA and Endo-GIA staplers perineally. The colonic remnant was then resected with staplers and harvested through the right lower quadrent port site. Operative time was 210 minutes for the boy and 200 minutes for the girl. No perioperative complications were noted, and conversion to laparotomy was never required. Regular diet was resumed 4 days (boy) and 2 days (girl) after the operations. Postoperative hospital stay was 7 days in the two cases. We successfully performed laparoscopic Duhamel procedures for the first time in Korea, and think it feasible, safe, andminimally invasive owing to size reduction of the incision and avoidence of intraperitoneal opening of the bowel.