Rhabdomyosarcoma in children: a SEER population based study.

OBJECTIVE: To examine incidence and outcomes for pediatric rhabdomyosarcoma (RMS). METHODS: The SEER registry was examined for patients with RMS < 20 y old. RESULTS: Overall, 1544 patients were identified for an incidence of 0.4414/100,000 per year. Males outnumbered females 3:2. Tumors were classified as embryonal (67%), alveolar (32%), and pleomorphic (1%). Alveolar and pleomorphic RMS were more common in adolescents, whereas embryonal type was more common in younger children (P = 0.0001). Pleomorphic (47%) and alveolar (39%) RMS commonly presented with distant disease, in contrast to embryonal (25%). Most patients had surgical resection (81%) and radiotherapy (63%). Overall, 5- and 10-y survival was 60% and 57%, respectively. Univariate analysis identified higher survival for age < 10 y, local stage, favorable site, embryonal type, <5 cm tumor size, and surgical resection. Multivariate analysis identified non-embryonal type (HR 1.451), non-favorable site (HR 1.570), no surgery (HR 1.726), age ≥ 10 y (HR 1.734), 1973-1978 diagnosis year (HR 1.730), and distant disease (HR 3.456) as independent predictors of mortality. CONCLUSIONS: Embryonal histology, the most common type of pediatric RMS, presents in young children and has better prognosis than alveolar or pleomorphic types. Patients with embryonal tumors, favorable tumor location, age < 10 y, localized disease, and surgical resection have improved survival.

17ß-estradiol modifies diabetic wound healing by decreasing matrix metalloproteinase activity .

UNLABELLED: Postmenopausal women are more susceptible to poor wound healing. This phenomenon can be reversed by estrogen replacement therapy in non-diabetic individuals. Postmenopausal women with type 2 diabetes are more susceptible to wound healing complications, potentially secondary to an estrogen deficiency. Few studies have examined the mechanism of action and effects of estrogens on diabetic wound healing in females. It appears that multiple factors influence delayed wound healing among individuals with diabetes including: an imbalance in cytokines, growth factors, extracellular matrix (ECM) turnover, and oxidant stress (OS). Estrogens have been shown to regulate the expression of genes important for extracellular matrix turnover, including collagen and matrix metalloproteinases (MMP). METHODS: For this reason, the effects of 17ß-estradiol (E2) on MMP-2, MMP-13, and MMP-14 and estrogen receptor alpha and beta (ER-α and -ß) expression in the wound tissue of estrogen-deficient female mice with established type 2 diabetes mellitus (C57BL/6J-m Leprdb/2+) were studied. RESULTS: Topical E2 upregulates ERα in wound tissue thereby improving and accelerating diabetic wound healing in estrogen deficient mice. CONCLUSION: The mechanism appears to decrease MMP-2, MMP-13, and MMP-14 mediated tissue matrix destruction and increasing collagen content. .

Estrogen deficiency and tobacco smoke exposure promote matrix metalloproteinase-13 activation in skin of aging B6 mice.

Estrogen deficiency may contribute to extracellular matrix turnover in skin. This has led previous authors to postulate that aged skin heals less efficiently when compared to younger skin. Also, cigarette smokers have been shown to heal less efficiently than nonsmokers. Matrix metalloproteinase (MMP)-13, an enzyme that participates in the degradation of the extracellular matrix, has been implicated in physiologic aging and wound healing. This study investigates the effects of smoke exposure and estrogen deficiency on MMP-13 in young and aged female mouse skin. Young and aged female C57Bl/6J mice were ovariectomized. They were then randomly administered either 17beta-estradiol (E2) or placebo pellets. Half the animals in each age group were further randomized to exposure to cigarette smoke for a period of 6 months. Smoking and estrogen deficiency increased MMP-13 protein and activity in aged skin. The tissue inhibitors of metalloproteinases, which inhibit MMPs, activity was unchanged across all groups. E2 replacement decreased the actual level of MMP-13 protein and activity. We also found an increased collagen content and decreased ER receptor protein level in aged, smoke-exposed female mice. Our experimental data show that tobacco smoke exposure and estrogen deficiency are additive risk factors for promoting increased activity of MMP-13 in aged skin. These findings suggest that MMP-13 functions as a mediator of smoke-induced skin injury in susceptible, aged experimental female mice.

Surgery for rectal cancer performed at teaching hospitals improves survival and preserves continence.

We sought to compare the outcomes of teaching and community hospitals on long-term outcomes for patients with rectal cancer. All rectal adenocarcinomas treated in Florida from 1994 to 2000 were examined. Overall, 5,925 operative cases were identified. Teaching hospitals treated 12.5% of patients with a larger proportion of regionally advanced, metastatic disease, as well as high-grade tumors. Five- and 10-year overall survival rates at teaching hospitals were 64.8 and 53.9%, compared to 59.1 and 50.5% at community hospitals (P = 0.002). The greatest impact on survival was observed for the highest stage tumors: patients with metastatic rectal adenocarcinoma experienced 5- and 10-year survival rates of 30.5 and 26.6% at teaching hospitals compared to 19.6 and 17.4% at community hospitals (P = 0.009). Multimodality therapy was most frequently administered in teaching hospitals as was low anterior resection. On multivariate analysis, treatment at a teaching hospital was a significant independent predictor of improved survival (hazard ratio = 0.834, P = 0.005). Rectal cancer patients treated at teaching hospitals have significantly better survival than those treated at community-based hospitals. Patients with high-grade tumors or advanced disease should be provided the opportunity to be treated at a teaching hospital.

Percutaneous retropleural drainage of a posttraumatic pancreatic mediastinal pseudocyst in a child.

Pancreatic mediastinal pseudocyst is a rare complication of pancreatitis in children. These children usually present with abdominal pain, anorexia, vomiting, and dysphagia. Computed tomography not only is essential in defining the extent of the pseudocyst but also can guide percutaneous external drainage. We present the case of a 4-year-old child with a mediastinal pseudocyst secondary to pancreatic trauma, which was successfully treated with computed tomography-guided percutaneous drainage via a posterior, extrapleural approach.

Primary gastrointestinal tract lymphoma in the pediatric patient: review of 265 patients from the SEER registry.

OBJECTIVE: The objective of this study is to determine outcomes of pediatric patients with primary gastrointestinal tract lymphoma (PGTL) and the impact of surgery or radiation on survival. METHODS: The Surveillance, Epidemiology, and End Result database was queried from 1973 to 2006 for patients younger than 20 years with PGTL. RESULTS: 265 patients with PGTL were identified. Overall 5- and 10-year survivals were 84% and 83%, respectively. Tumors of the stomach (9%) and rectum/anus (2%) had the worst and best 10-year survivals, respectively (59% vs 100%, P = .023). There was no significant difference in 10-year survival for patients younger than 10 years of age who had surgical extirpation (83% vs 85% no surgery, P = .958) or radiotherapy (76% vs 85% no radiotherapy, P = .532). However, there was a significantly decreased 10-year survival in patients 10 years or older who had surgical extirpation (79% vs 100% no surgery, P = .013) or radiotherapy (49% vs 87% no radiotherapy, P = .001). Under multivariate analysis, tumor location was an independent predictor of improved survival (small bowel, HR 0.21, P = .002; large bowel, HR 0.23, P = .004). CONCLUSION: We found no significant survival advantage for surgical extirpation or radiotherapy in patients younger than 10 years with PGTL, whereas either treatment modality was associated with lower survival in patients 10 years or older.

Formation of functional neovagina with vertical rectus abdominis musculocutaneous (VRAM) flap after total pelvic exenteration.

BACKGROUND: Pelvic exenteration may be the only curative option for women with recurrent pelvic malignancies. After total pelvic exenteration, the resultant perineal defect heals slowly if left to do so by secondary intention. Reconstruction with the vertical rectus abdominis musculocutaneous (VRAM) flap brings a generous bulk of healthy tissue into the defect, speeding recovery by facilitating primary healing. METHODS: Six women underwent reconstruction of a neovagina using a vertical rectus abdominis musculocutaneous flap. All 6 had total pelvic exenteration for advanced gynecologic malignancy. Primary diagnosis was cervical carcinoma (n = 3), vulvar carcinoma (n = 1), nonsmall cell vaginal cancer (n = 1), and vaginal melanoma (n = 1). Four patients had received adjuvant radiotherapy preoperatively. RESULTS: All flaps remained 100% viable postoperatively. There were no cases of fistula, infection, or bowel obstruction. Two patients died of cardiovascular arrest postoperatively. The 4 other patients report satisfaction with reconstruction. Three had vaginal intercourse with orgasm. CONCLUSION: The inferiorly based vertical rectus abdominis musculocutaneous flap is a dependable source of tissue for pelvic reconstruction and is the flap of choice in the Division of Plastic Surgery. In addition to facilitating healing, the VRAM flap (neovagina) improves a woman's psychosocial well-being.

Perioperative considerations in the autologous breast reconstruction patient with systemic lupus erythematosus.

Following mastectomy, women with breast cancer are faced with choices for reconstruction. In women with systemic lupus erythematosus (SLE), with or without antiphospholipid syndrome (APS), concurrent morbidities are important considerations when deciding the method of reconstruction. The 2 cases we present represent the variable spectrum of SLE and/or APS and their potential complications. When deciding the best course of action, it is most important to consider each case on an individual basis, with important emphasis on preoperative disease status.

Continent ileocolonic urinary reservoir (Miami pouch): the University of Miami experience over 15 years.

OBJECTIVE: A patient with a recurrent central pelvic malignancy after radiation will require urinary diversion as part of the reconstructive phase of the pelvic exenteration. The aim of our study was to assess the result of our 15-year experience with a continent ileocolonic urinary reservoir, which is known as the Miami pouch. STUDY DESIGN: Since 1988, all patients who received a continent ileocolonic urinary reservoir in the Division of Gynecologic Oncology, University of Miami School of Medicine, were included in the study. Parameters that were evaluated during the study period include functional outcomes, early and late perioperative complications, and their treatment. RESULTS: A total of 90 patients were identified from February 1988 to December 2002. Seventy-eight patients (87%) had a recurrent central pelvic malignancy, and 82 patients (91%) received radiation before the Miami pouch procedure. The non-reservoir-related morbidities were fever (76%), wound complication (30%), pelvic collection (12%), ileus/small bowel obstruction (12%), and postoperative death (11%). The most common reservoir-related complications were urinary infection (40%), ureteral stricture (20%), and difficulty with self-catheterization (18%). In our study, the overall complication rate that was related directly to the Miami pouch was 53%. Conservative treatment resolved>80% of these cases. The rate of urinary continence that was achieved in our patients was 93% during our 15-year experience with the Miami pouch. CONCLUSION: The Miami pouch is a good alternative for continent urinary diversion during exenteration or radiation-induced damage. The rate of major complications that require aggressive surgical intervention is acceptable. Most postoperative complications (80%) can be corrected with the use of conservative techniques that are associated with fewer deaths than reoperation and thus should be used first. The technique is simple and effective in women who are at high risk, who have undergone previous radiation therapy, and who have a high rate of functional success and is a profound advantage for a woman's psychosocial well-being.