Desmoid fibromatosis in children and adolescents: a conservative approach to management.

PURPOSE: Desmoid fibromatosis is associated with frequent recurrence and significant morbidity, but no metastases. To examine the impact of initial non-operative management on event-free survival (EFS) in children, we reviewed our institutional experience with this tumor. METHODS: We retrospectively reviewed our institutional database for pediatric cases of desmoid fibromatosis treated between 1970 and 2010. Survival was analyzed using the Kaplan-Meier method and log-rank test. RESULTS: Ninety-three patients were identified, with a median follow-up of 6 years. Median age at diagnosis was 16 years. Forty-seven patients presented with primary tumors, and forty-six had recurrent or progressing disease. Five-year OS was 100%, and 5-year EFS was 31.8%, with a median time to event of 1.48 years. There was no significant difference in 5-year EFS between patients who were managed expectantly and those who initially received treatment (21% versus 34%, P=.09). Sex, race, history of trauma, or familial adenomatous polyposis, multifocality, tumor size, tumor location, and resection status did not correlate with EFS. CONCLUSION: Our findings support a conservative initial approach in the management of desmoid fibromatosis. In patients at risk for morbid procedures, upfront resection should be reserved for select tumors that demonstrate aggressive growth or cause serious symptoms.

Factors associated with relapse and survival in Wilms tumor: a multivariate analysis.

PURPOSE: Lymph node metastasis and anaplasia predict relapse-free survival in Wilms tumor. We performed a multivariate analysis of our institutional database to identify factors independently associated with relapse-free and overall survival. METHODS: We retrospectively reviewed cases of confirmed Wilms tumor diagnosed between 1990 and 2010 and treated at our institution. The log-rank test was used to screen variables for consideration in the proportional hazards model. RESULTS: A total of 95 patients were treated at our institution during the study period, with a median follow-up of 3.3 years. Factors correlated with overall survival in the univariate analysis were local disease, metastasis, tumor size, anaplasia, renal vein tumor thrombus, inferior vena cava tumor thrombus, lymph node positivity, and tumor rupture. On multivariate analysis, factors associated with increased risk of death were lymph node positivity and anaplasia. Factors correlated with probability of relapse in the univariate analysis were lymph node positivity, anaplasia, and female sex. All 3 of these factors were also independently significant on multivariate analysis. CONCLUSION: Lymph node involvement and anaplasia are significantly correlated with probability of relapse and overall survival, reemphasizing the strong recommendation to sample regional lymph nodes during Wilms tumor resection.

Safety and immunogenicity of the live attenuated varicella vaccine following T replete or T cell-depleted related and unrelated allogeneic hematopoietic cell transplantation (alloHCT).

There are limited studies assessing the live attenuated varicella vaccine following allogeneic hematopoietic cell transplantation (alloHCT). Because of the morbidity of varicella acquired after childhood, we immunized and retrospectively analyzed the safety and immunogenicity of this vaccine in 46 varicella zoster virus (VZV) seronegative patients <20 years old at HCT who achieved a CD4 cell count ≥200/µL, were off immunosuppression, and responded to ≥1 post-HCT vaccines. Two vaccinated patients lacking follow-up titers were excluded from analysis. Stem cells were derived from an HLA-matched sibling (n = 18) or an alternative (HLA mismatched related or unrelated) donor (n = 26). Median time to vaccination was 4 years. Sixty-four percent of patients seroconverted following 1 immunization. There was no significant difference in response between recipients of a matched related or alternative donor graft (P = .2) or between those given a T cell-depleted or T-replete alternative donor graft (P = .27). Three of 44 patients developed a self-limited varicella-like rash within 2.5 weeks of immunization. With a median follow-up of 29.1 (range: 6.9-167.1) months, there were no subsequent cases of varicella-like rashes. No patient developed shingles. This study suggests that this vaccine is safe and immunogenic when given according to preset clinical and immunologic milestones, warranting larger prospective studies in patients ≥24 months following HCT as outlined in current post-HCT vaccine guidelines.