Outcomes in pediatric atypical spitz tumors treated without sentinel lymph node biopsy.

The diagnosis of atypical Spitz tumor (AST) in a pediatric patient conveys an uncertain potential for malignancy. Although pediatric melanoma is rare, AST may be treated aggressively with sentinel lymph node biopsy (SLNB) and subsequent completion lymphadenectomy. These procedures have unclear therapeutic benefit and potential morbidity. We aimed to collect outcomes on children with AST treated with excision alone to assist in the management of these lesions. We queried our institution's pathology database for AST specimens submitted between 1994 and 2009. A dermatopathologist reviewed pathology slides to confirm AST diagnosis. Clinical information was obtained from medical records, and outcomes surveys were administered to children with AST. Twenty-nine patients met AST diagnostic criteria and were included in this study. Mean age at first excision was 9.0 ± 4.2 (range 2.3-17.5), and 19 patients underwent more than one excision procedure to achieve clear margins. No patient had SLNB. Fourteen patients (48%) with mean follow-up time of 8.4 years (range 3.5-15.8) completed clinical outcomes surveys. Outcomes with mean follow-up time of 2.8 years (range 0.02-8.1 years) were obtained for 10 additional patients from medical records. There were no reports of recurrence, additional lesions, or metastases in these 24 patients. We report one of the largest series of children with AST treated using excision alone and who remain disease free after a significant follow-up period. Our data suggest that SLNB is not warranted in the routine management of pediatric AST. We recommend complete excision with clear margins and careful clinical follow-up.

Incidence of Fanconi anemia in children with congenital thumb anomalies referred for diepoxybutane testing.

PURPOSE: Fanconi anemia (FA) is a rare genetic disorder of DNA repair that with near uniformity leads to bone marrow failure and resulting morbidity and mortality. Approximately 50% of FA patients are born with anomalies of the thumb or thumb and radius, and it has been recommended that all patients born with thumb anomalies undergo testing. However, the risk of FA in this population is unknown. We determined the incidence of FA in children with congenital thumb anomalies referred for FA testing and characterized those who tested positive. METHODS: We queried our database for patients who presented with congenital thumb anomalies and who underwent diepoxybutane (DEB) testing for FA between 1999 and 2008 at Children's Hospital Boston and the Dana-Farber Cancer Institute. RESULTS: During this time period, 543 congenital thumb anomaly patients (235 with thumb hypoplasia) presented to our institution. A total of 81 patients with thumb abnormalities underwent DEB testing. Six patients (7% of those tested; 1% of the total; 3% of thumb hypoplasia patients) had a positive DEB test consistent with the diagnosis of FA; all had other non-upper-extremity anomalies associated with FA. Of 6 FA patients, 5 had bilateral involvement; all had some degree of thumb hypoplasia (3 also had radial dysplasia). Mean age at testing was 2.6 years (SD 4.3). Most of the patients tested had multiple physical anomalies (n = 66). The anomaly distribution was: thumb hypoplasia and radial dysplasia (n = 29), thumb hypoplasia (n = 26), radial polydactyly (n = 12), radial polydactyly and radial dysplasia (n = 1), and proximally placed thumb and radial dysplasia (n = 1). Twelve patients had other thumb anomalies. CONCLUSIONS: Although the incidence of FA in patients with thumb anomalies may be low, patients with thumb hypoplasia and other physical findings associated with FA, specifically café au lait spots and short stature, appear to have an increased risk of FA. Because hand surgeons see these patients early in life, they have the opportunity to refer these patients for FA testing to initiate early education, family genetic counseling, and treatment if warranted. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

The impact of macromastia on adolescents: a cross-sectional study.

OBJECTIVE: To determine the physical and psychosocial impact of macromastia on adolescents considering reduction mammaplasty in comparison with healthy adolescents. METHODS: The following surveys were administered to adolescents with macromastia and control subjects, aged 12 to 21 years: Short-Form 36v2, Rosenberg Self-Esteem Scale, Breast-Related Symptoms Questionnaire, and Eating-Attitudes Test-26 (EAT-26). Demographic variables and self-reported breast symptoms were compared between the 2 groups. Linear regression models, unadjusted and adjusted for BMI category (normal weight, overweight, obese), were fit to determine the effect of case status on survey score. Odds ratios for the risk of disordered eating behaviors (EAT-26 score ≥ 20) in cases versus controls were also determined. RESULTS: Ninety-six subjects with macromastia and 103 control subjects participated in this study. Age was similar between groups, but subjects with macromastia had a higher BMI (P = .02). Adolescents with macromastia had lower Short-Form 36v2 domain, Rosenberg Self-Esteem Scale, and Breast-Related Symptoms Questionnaire scores and higher EAT-26 scores compared with controls. Macromastia was also associated with a higher risk of disordered eating behaviors. In almost all cases, the impact of macromastia was independent of BMI category. CONCLUSIONS: Macromastia has a substantial negative impact on health-related quality of life, self-esteem, physical symptoms, and eating behaviors in adolescents with this condition. These observations were largely independent of BMI category. Health care providers should be aware of these important negative health outcomes that are associated with macromastia and consider early evaluation for adolescents with this condition.

Exclusion and micro-rejection: event-related potential response predicts mitigated distress.

We studied time-based neural activity with event-related potentials (ERPs) in young adults during a computer-simulated ball-toss game. Experiencing fair play initially, participants were ultimately excluded by other players. Dense-array ERPs showed time-dependent associations between slow-wave activity (580-900 ms) in left prefrontal/medial frontal cortical regions for exclusion events and self-reported distress. More subtle 'micro-rejections' during fair play showed a similar distress to ERP association (420-580 ms). In both cases, greater positive amplitude neural activity was associated with less post-exclusion distress. Findings suggest that rapidly occurring neural responses to social exclusion events are linked to individual differences in ostracism-related distress. Relations emerged even during fair play, providing a window into the neural basis of more subtle social-cognitive perceptual processes.