In our opinion, PB have a poor prognosis and are very aggressive, especially in small children. Survival rate at 1 and 5 years in the present series is 66.6% (8/12) and 50% (6/12), respectively. We propose an algorithm for the treatment of pediatric patients with PB.

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Introduction: This study presents the incidence of gastrointestinal stromal tumors (GISTs) in an Italian region of over 4 million inhabitants monitored for 10 years and is the largest incidence study of this type of cancer conducted so far in Italy. Methods: In order to ensure the registration of all GISTs, including those with nonmalignant behavior, a cancer list was integrated with the cases found through an ad hoc data mining process that covered all the pathologic reports of Puglia. Case distributions by sex, age groups, site, and prognostic groups according to Miettinem and Lasota classification and crude and age-standardized incidence rates were produced. Results: In the 10-year period 2006 to 2015, 708 cases of GIST were recorded in Puglia. The average crude incidence rate was 1.7 per 100,000 person-years and the age-standardized incidence rate, using 2013 European standard population, was 1.8 per 100,000 person-years (95% confidence interval [CI], 1.6–1.9). Incidence was higher in men than in women: crude incidence rate was 2.0 per 100,000 person-years and age-standardized incidence rate 2.2 per 100,000 person-years (95% CI, 2.0–2.4) in men and 1.5 per 100,000 person-years and 1.4 per 100,000 person-years (95% CI, 1.2–1.6) in women. Discussion: Our incidence rates are comparable with those of other international studies and they are located in the medium to high end of the range. The comparisons are affected by a different capacity of the cancer registries to intercept and record GISTs with nonmalignant behavior. Distribution of cases for sex, age groups, sites, and prognostic risk groups are consistent with previous results.

Objective To evaluate maternal and perinatal outcomes of late open fetal repair for open spina bifida (OSB) between 26+0–27+6 weeks. Methods A cohort of fetuses with OSB who underwent open surgery in two fetal surgery centers (Argentina and Mexico). Two groups were defined based on the gestational age (GA) at intervention: Management of Myelomeningocele Study (MOMS) time window group: GA 19+0–25+6, and late intervention group: GA 26+0–27+6. Results Intrauterine OSB repair was successfully performed in 140 cases, either before (n = 57) or after (n = 83) 26 weeks, at on average 25+0 (22+6–25+6) and 26+5 (26+0–27+6) weeks + days, respectively. There were no significant differences in the rate of premature rupture of membranes, chorioamnionitis, oligohydramnios, preterm delivery, perinatal death and maternal complications. The late intervention group showed a significantly lower surgical times (112.6 vs. 124.2 min, p = 0.01), lower interval between fetal surgery and delivery (7.9 vs. 9.2 weeks, p < 0.01) and similar rate of hydrocephalus requiring treatment (30.6% vs. 23.3%, p = 0.44) than the MOMS time window group. Conclusion Late fetal surgery for OSB repair between 26+0–27+6 weeks is feasible and was associated with similar outcomes than that performed before 26 weeks. These findings may allow an extension of the proposed time window for cases with late diagnosis or referral.