Marc G. Schlatter

Changing Trends in Necrotizing Enterocolitis

Three hundred two infants with necrotizing enterocolitis (NEC) were treated from 1972 to 1990. One hundred eighteen were treated medically while 184 infants required operation. Comparisons were made between two treatment periods, 1972 to 1982 (n = 176) and 1983 to 1990 (n = 126). Infants in the more recent era were of lower birth weight (1505 +/- 853 g versus 1645 +/- 836 g), earlier gestational age (30.4 +/- 4.7 weeks versus 32.4.5 weeks; [p less than 0.01]), had symptom onset at an older age (15.7 +/- 13.9 days versus 10.0 +/- 10.8 days; [p less than 0.001]), and a lower incidence of hyaline membrane disease (p less than 0.001). Fewer patients in the 1983 to 1990 group had acidosis (p less than 0.001) and severe oliguria (p less than 0.001). Operation was performed sooner after diagnosis in the second group (2.6 versus 3.8 days; [p less than 0.001]). Survival was unaffected by sex, maternal complications, or whether infants were inborn or transferred from other facilities. Improved survival (1983 to 1990) was observed in those infants between 24 to 27 weeks gestation (p less than 0.002) and those weighing less than 1000 g (p less than 0.001). Since 1983 portal vein air (PVA) on abdominal radiographs was used as an indicator for operation. Survival in infants with PVA has improved from 29% to 64% (p less than 0.02). Despite patients being more immature and weighing less, the overall survival rate improved from 58% (1972 to 1982) to 82% (1983 to 1990) (p less than 0.001). Operative survival rate improved from 51% to 75% (p less than 0.002). Long-term survival was 75% overall and 65% for surgical infants in the 1983 to 1990 group (p less than 0.05).

Surveillance After Initial Surgery for Pediatric and Adolescent Girls With Stage I Ovarian Germ Cell Tumors: Report From the Children's Oncology Group

PURPOSE To determine whether overall survival (OS) can be preserved for patients with stage I pediatric malignant ovarian germ cell tumor (MOGCT) with an initial strategy of surveillance after surgical resection. PATIENTS AND METHODS Between November 2003 and July 2011, girls age 0 to 16 years with stage I MOGCT were enrolled onto Childrens Oncology Group study AGCT0132. Required histology included yolk sac, embryonal carcinoma, or choriocarcinoma. Surveillance included measurement of serum tumor markers and radiologic imaging at defined intervals. In those with residual or recurrent disease, chemotherapy with compressed PEB (cisplatin, etoposide, and bleomycin) was initiated every 3 weeks for three cycles (cisplatin 33 mg/m(2) on days 1 to 3, etoposide 167 mg/m(2) on days 1 to 3, bleomycin 15 U/m(2) on day 1). Survivor functions for event-free survival (EFS) and OS were estimated using the Kaplan-Meier method. RESULTS Twenty-five girls (median age, 12 years) with stage I MOGCT were enrolled onto AGCT0132. Twenty-three patients had elevated alpha-fetoprotein (AFP) at diagnosis. Predominant histology was yolk sac. After a median follow-up of 42 months, 12 patients had evidence of persistent or recurrent disease (4-year EFS, 52%; 95% CI, 31% to 69%). Median time to recurrence was 2 months. All patients had elevated AFP at recurrence; six had localized disease, two had metastatic disease, and four had tumor marker elevation only. Eleven of 12 patients experiencing relapse received successful salvage chemotherapy (4-year OS, 96%; 95% CI, 74% to 99%). CONCLUSION Fifty percent of patients with stage I pediatric MOGCT can be spared chemotherapy; treatment for those who experience recurrence preserves OS. Further study is needed to identify the factors that predict recurrence and whether this strategy can be extended successfully to older adolescents and young adults.

Gastric duplication cyst : a unique presentation

Duplications of the alimentary tract are rare and occur in 1 of 4500 births (Duplication of the stomach: report of a case and review of the English literature. Arch Surg 1961; 82:634-640). Gastric duplications constitute 8% of these or roughly 17 of every 1,000,000 births (Shew SB, Holcomb GW. Alimentary tract duplications. In: Ashcraft KW, Holcomb GW, Murphy JP, editors. Pediatric surgery. Philadelphia, PA: Elsevier, 2005. p. 543-552). Symptoms often occur by 2 years and can include nausea, vomiting, hematemesis, and vague abdominal pain. Occasionally, a palpable abdominal mass may be identified on physical examination. We offer an unusual and previously unreported presentation of a gastric duplication cyst.

Reduced and compressed cisplatin-based chemotherapy in children and adolescents with intermediate-risk extracranial malignant germ cell tumors: A report from the children's oncology group

Purpose To investigate whether event-free survival (EFS) can be maintained among children and adolescents with intermediate-risk (IR) malignant germ cell tumors (MGCT) if the administration of cisplatin, etoposide, and bleomycin (PEb) is reduced from four to three cycles and compressed from 5 to 3 days per cycle. Patients and Methods In a phase 3, single-arm trial, patients with IR MGCT (stage II-IV testicular, II-III ovarian, I-II extragonadal, or stage I gonadal tumors with subsequent recurrence) received three cycles of PEb. A parametric comparator model specified that the observed EFS rate should not be significantly < 92%. As recommended for trials that test a reduction of therapy, a one-sided P value ≤ .10 was used to indicate statistical significance. In a post hoc analysis, we also compared results to the EFS rate of comparable patients treated with four cycles of PEb in two prior studies. Results Among 210 eligible patients enrolled from 2003 to 2011, 4-year EFS (EFS4) rate was 89% (95% confidence interval, 83% to 92%), which was significantly lower than the 92% threshold of the comparison model ( P = .08). Among 181 newly diagnosed patients, the EFS4 rate was 87%, compared with 92% for 92 comparable children in the historical cohort ( P = .15). The EFS4 rate was significantly associated with stage (stage I, 100%; stage II, 92%; stage III, 85%; and stage IV, 54%; P < .001). Conclusion The EFS rate for children with IR MGCT observed after three cycles of PEb was less than that of a prespecified parametric model, particularly for patients with higher-stage tumors. These data do not support a reduction in the number of cycles of PEb from four to three. However, further investigation of a reduction in the number of cycles for patients with lower-stage tumors is warranted.

Impact of central surgical review in a study of malignant germ cell tumors

BACKGROUND Verification of surgical staging has received little attention in clinical oncology trials. Central surgical review was undertaken during a study of malignant pediatric germ cell tumors. METHODS Childrens Oncology Group study AGCT0132 included central surgical review during the study. Completeness of submitted data and confirmation of assigned stage were assessed. Review responses were: assigned status confirmed, assignment withheld pending review of additional information requested, or institutional assignment of stage disputed with explanation given. Changes in stage assignment were at the discretion of the enrolling institution. RESULTS A total of 206 patients underwent central review. Failure to submit required data elements or need for clarification was noted in 40%. Disagreement with stage assignment occurred in 10% with 17/21 discordant patients reassigned to stage recommended by central review. Four ovarian tumor patients not meeting review criteria for Stage I remained in that stratum by institutional decision. Two-year event free survival in Stage I ovarian patients was 25% for discordant patients compared to 57% for those meeting Stage I criteria by central review. CONCLUSIONS Central review of stage assignment improved complete data collection and assignment of correct tumor stage at study entry, and allowed for prompt initiation of chemotherapy in patients determined not to have Stage I disease.