Triple High Dose Chemotherapy Followed by Autologous Stem Cell Transplantation for Pediatric Neuroblastoma / 임상소아혈액종양

BACKGROUND: High-risk group neuroblastoma (HRNB) is one of the most incurable diseases in pediatric oncology field. This report explores the effectiveness, safety and feasibility of triple high dose chemotherapy followed by autologous stem cell transplantation (HDCT/ASCT) in contrast with single HDCT/ASCT for HRNB. METHODS: From Jan. 2001 to Dec. 2009, 25 patients newly diagnosed as HRNB have been analyzed. This study is a retrospective analysis with the medical records of these 25 HRNB patients. RESULTS: Eleven (44%) and the other fourteen (56%) patients were treated with single HDCT/ASCT and triple HDCT/ASCT, respectively. The 5-year event-free survival (EFS) of the whole group in HRNB is 37.2+/-10.0%. The 5-year EFS of single HDCT/ASCT and triple HDCT/ASCT were 27.3+/-13.4% and 46.8+/-13.8%, respectively (P=0.38). Between two groups, there were no statistically significant differences including clinical features, outcome, transplantation-related toxicities, and short-term/long-term complications. CONCLUSION: Triple HDCT/ASCT group showed similar transplantation-related toxicities and long-term complications when compared to single HDCT/ASCT group. We need more exploration to conclude the triple HDCT/ASCT as an optimal treatment for HRNB patients.

Inflammatory Myofibroblastic Tumor in a 3 Months Old Infant / 임상소아혈액종양

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.

Triple High Dose Chemotherapy Followed by Autologous Stem Cell Transplantation for Pediatric Neuroblastoma / 임상소아혈액종양

BACKGROUND: High-risk group neuroblastoma (HRNB) is one of the most incurable diseases in pediatric oncology field. This report explores the effectiveness, safety and feasibility of triple high dose chemotherapy followed by autologous stem cell transplantation (HDCT/ASCT) in contrast with single HDCT/ASCT for HRNB.METHODS: From Jan. 2001 to Dec. 2009, 25 patients newly diagnosed as HRNB have been analyzed. This study is a retrospective analysis with the medical records of these 25 HRNB patients.RESULTS: Eleven (44%) and the other fourteen (56%) patients were treated with single HDCT/ASCT and triple HDCT/ASCT, respectively. The 5-year event-free survival (EFS) of the whole group in HRNB is 37.2+/-10.0%. The 5-year EFS of single HDCT/ASCT and triple HDCT/ASCT were 27.3+/-13.4% and 46.8+/-13.8%, respectively (P=0.38). Between two groups, there were no statistically significant differences including clinical features, outcome, transplantation-related toxicities, and short-term/long-term complications.CONCLUSION: Triple HDCT/ASCT group showed similar transplantation-related toxicities and long-term complications when compared to single HDCT/ASCT group. We need more exploration to conclude the triple HDCT/ASCT as an optimal treatment for HRNB patients.

Inflammatory Myofibroblastic Tumor in a 3 Months Old Infant / 임상소아혈액종양

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.

Microarray Analysis of Gene Expression Profiles in Response to Treatment with Melatonin in Lipopolysaccharide Activated RAW 264.7 Cells

Melatonin, which is the main product of the pineal gland, has well documented antioxidant and immune-modulatory effects. Macrophages produce molecules that are known to play roles in inflammatory responses. We conducted microarray analysis to evaluate the global gene expression profiles in response to treatment with melatonin in lipopolysaccharide (LPS) activated RAW 264.7 macrophage cells. In addition, eight genes were subjected to real-time reverse transcription polymerase chain reaction (RT-PCR) to confirm the results of the microarray. The cells were treated with LPS or melatonin plus LPS for 24 hr. LPS induced the up-regulation of 1073 genes and the down-regulation of 1144 genes when compared to the control group. Melatonin pretreatment of LPS-stimulated RAW 264.7 cells resulted in the down regulation of 241 genes and up regulation of 164 genes. Interestingly, among genes related to macrophage-mediated immunity, LPS increased the expression of seven genes (Adora2b, Fcgr2b, Cish, Cxcl10, Clec4n, Il1a, and Il1b) and decreased the expression of one gene (Clec4a3). These changes in expression were attenuated by melatonin. Furthermore, the results of real-time PCR were similar to those of the microarray. Taken together, these results suggest that melatonin may have a suppressive effect on LPS-induced expression of genes involved in the regulation of immunity and defense in RAW 264.7 macrophage cells. Moreover, these results may explain beneficial effects of melatonin in the treatment of various inflammatory conditions.

A Case of Multiple Endocrine Neoplasia Type 2B early Diagnosis by RET Proto-oncogene Analysis and Prophylactic Total Thyroidectomy / 대한소아내분비학회지

Multiple endocrine neoplasia type 2B (MEN 2B) is a rare autosomal-dominant hereditary syndrome which includes medullary thyroid carcinoma (MTC), pheochoromocytoma, multiple ganglioneuromas, gastrointestinal disorders and marfanoid face. MTC is the main cause of death in patients that have not received early and prophylactic treatment. Clinically useful direct DNA tests have recently been available to identify gene carriers before any clinical or biochemical abnormalities are present. Prophylactic total thyroidectomy is recommended to affected children at an early stage of the disease to prevent the development of metastases of MTC. We report a case of MEN 2B patient who was diagnosed by RET proto-oncogene analysis and performed prophylactic total thyroidectomy.

Initial Experience of Laparoscopic Pyeloplasty: Retrograde Stenting Using Flexible Cystoscopes / 대한비뇨기과학회지

PURPOSE: To describe our initial experience of laparoscopic pyeloplasty, with retrograde stenting, using flexible cystoscopes, in 9 cases of ureteropelvic junction obstruction (UPJO). MATERIALS AND METHODS: Between September, 2004 and January, 2006, 9 cases of UPJO were treated by laparoscopic pyeloplasty. All of the medical records were reviewed retrospectively. The cases comprised of 5 males and 4 females, with a mean age of 37 years (13-58). All patients showed a preoperative grade 4 hydronephrosis. RESULTS: Two laparoscopic pyeloplasty approaches were performed; a transperitoneal approach in 5 cases and a retroperitoneal approach in the other 4. All cases were treated with dismembered Anderson-Hynes pyeloplasty. The mean operating time was 249 minutes (170-324), including a mean of 7.1 minutes (6.5-8) for ureteral stenting. The patients were found to have aberrant vessels and renal stones in 1 and 3 cases, respectively. There were no intra-operative complications or open conversion. Of the 9 cases, 8 (89%) showed a decrease in their hydronephrosis grade on the excretory urographs taken 12 weeks postoperatively. The remaining case showed a marked improvement in the hydronephrosis, despite having no change in the hydronephrosis grade. No case experienced postoperative flank pain or recurrence of acute pyelonephritis. Therefore, symptomatic improvements were observed in all our cases (100%). CONCLUSIONS: Laparoscopic pyeloplasty, with retrograde stenting, using flexible cystoscopes, can be considered an effective and convenient method for the treatment of UPJO.

A Torsion of the Cyst of Tunica Vaginalis Presented with Acute Scrotum / 대한비뇨기과학회지

We report a case of torsion of a benign cyst arising from the parietal layer of the tunica vaginalis, which presented on an acute scrotum. Physical examination revealed a tender swelling of the left hemiscrotum. Surgical, we happened to find torsion of a cyst originating from the parietal layer of the tunica vaginalis. The pedicle of the cyst was twisted about 360 degrees.

A Case of Primary Syphilis in the Rectum

A 30-yr-old man was referred for suspicious rectal cancer because of ulcerated lesions in the rectum and a palpable mass in left inguinal area. Sigmoidoscopy showed two indurated masses and histologic evaluation of biopsy revealed obliterative endarteritis with heavy plasma cell infiltration. Both venereal disease research laboratories (VDRL) and fluorescent treponemal antibody absorption (FTA-ABS) tests were positive. After injection of penicillin G benzathine for 3 weeks, the rectal chancre and the palpable mass disappeared.