Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT. METHODS: A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS). RESULTS: Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months). CONCLUSIONS: HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.

Munchausen syndrome by proxy: a case report.

Munchausen syndrome by proxy is difficult to diagnose unless healthcare providers are astute to its clinical features and management. A case is presented to educate nurses and advanced practice nurses, of the nursing, medical, legal, and social complexities associated with Munchausen syndrome by proxy. This article also provides a brief review of the definition of Munchausen syndrome by proxy, its epidemiology, common features of the perpetrator, implications for healthcare personnel, and the legal and international ramifications of Munchausen syndrome by proxy.

The child with a tracheostomy and gastrostomy: parental stress and coping in the home--a pilot study.

Caring for the technology dependent child in the home creates parental stress. Parenting children with device-based respiratory support is particularly stressful as changes in this area can be immediately life threatening. A retrospective pilot study was conducted to describe perceived stressors and coping strategies of the primary caregiver of a child with a tracheostomy and gastrostomy. A convenience sample of 50 families who had a child that received a tracheostomy and gastrostomy in the past two years at our hospital was invited to participate. Three questionnaires were used: the Impact on Family Scale (Stein & Riessman, 1980), the Family Crisis Oriented Personal Evaluation Scale (McCubbin, Olsen, & Larsen, 1981), and a demographic tool. Eighteen (36%) primary caregivers completed the questionnaires. Findings suggest caregivers were coping and perceived themselves as financially stable. However, these data also revealed that caregivers believed there was disruption of social interactions within and outside the family because of the child's condition. Data indicate a need for developing home care plans that include respite services.