Type I and Ir pleuropulmonary blastoma (PPB): A report from the International PPB/DICER1 Registry.

BACKGROUND: Pleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood. Type I PPB is a purely cystic lesion that has a microscopic population of primitive small cells with or without rhabdomyoblastic features and may progress to type II or III PPB, whereas type Ir lacks primitive small cells. METHODS: Children with suspected PPB were enrolled in the International PPB/DICER1 Registry. Pathology was centrally reviewed, and follow-up was ascertained annually. RESULTS: Between 2006 and 2022, 205 children had centrally reviewed type I or Ir PPB; 39% of children with type I and 5% of children with type Ir PPB received chemotherapy. Outcomes were favorable, although 11 children (nine with type I and two with type Ir PPB) experienced progression to type II/III (n = 8) or regrowth of type I PPB at the surgical site (n = 3), none of whom received chemotherapy before progression. Age and cyst size in combination were more suitable than either factor alone in predicting whether a particular lesion was type I or Ir PPB. CONCLUSIONS: For young children with type I PPB, outcomes are favorable, but complete resection is indicated because of the risk for progression. Chemotherapy may be useful in a subset of children at increased risk for recurrence/progression. Efforts to risk stratify children with type I PPB to optimize outcomes while reducing treatment-related side effects are underway.

Fetal Non-Ovarian Abdominopelvic Cystic Lesions: A Single-Center Report.

INTRODUCTION: There is a paucity of reports describing the clinical course and likely postnatal outcomes of prenatally identified simple cystic abdominopelvic lesions which are not associated with the ovary. OBJECTIVE: The aim of this study was to describe the natural history and postnatal outcomes of prenatally discovered abdominopelvic cystic lesions seen at our center. METHODS: This study is a retrospective review of all newborns with prenatally discovered non-ovarian simple cystic abdominal or pelvic lesions (September 2012-December 2018). Prenatal solid organ involvement, lesion size, and postnatal clinical outcomes are described. RESULTS: Sixty-six patients with 68 cystic lesions were identified; 22 patients with 24 lesions met the defined study criteria and were included. Eleven (46%) resolved prenatally, while 5 (21%) resolved by 18 months of age. Of the 10 lesions associated with an organ, 4 (40%) resolved prenatally. Of the remaining 14 lesions not associated with a solid organ, 7 (50%) resolved prenatally. Seven lesions (29%) required postnatal surgical intervention. Larger maximum prenatal lesions tended toward postnatal surgical intervention (one-way ANOVA: p = 0.072). CONCLUSIONS: The majority of simple non-ovarian cystic abdominopelvic lesions at our center resolved in the perinatal period. Due to the low frequency of these lesions at fetal centers, a larger multicenter study based on a consistent monitoring protocol should be undertaken to better describe the resolution patterns of simple non-ovarian cystic lesions for improved prenatal counseling.

Peritoneal dialysis catheter placement, outcomes and complications.

PURPOSE: Peritoneal dialysis (PD) is a commonly used method for renal support in pediatric patients and can be associated with the risk of post-surgical complications. We evaluated method of placement of PD catheters with regard to post-surgical complications. METHODS: PD catheters placed at two institutions between 2005 and 2017 were reviewed. Complication rates were evaluated based on method of placement, delayed usage, omentectomy, and patient age using Fisher's exact test, two-sided, with significance set at 0.05. Factors influencing complication were evaluated with multivariate logistic regression and Kaplan-Meier survival analysis. RESULTS: There were 130 patients with 157 catheters placed, ranging in age from 1 day to 23 years. There was no significant difference in complication rate by method of placement or delayed usage. Infants were significantly more likely to experience leakage (21% vs 8%, p 0.036) and hernias (15% vs 5%, p 0.030). Patients that underwent an omentectomy were less likely to require a catheter replacement (7% vs 27%, p 0.004), and the catheters had a significantly higher survival rate (p 0.009). We found that laparoscopic intervention resulted in catheter salvage. Lateral exit sites may be a risk factor for catheter migration in some patients. CONCLUSIONS: Omentectomy is associated with longer PD catheter survival. Laparoscopic salvage of dysfunctional catheters may be a valuable adjunct in management.

Surgical experience in patients with cystic fibrosis: a 25-year perspective.

The spectrum of surgical diseases in patients with cystic fibrosis (CF) has not been comprehensively studied. A retrospective review of 792 consecutive patients with CF presenting over a 25 year period (1970-1994) was made to determine the incidence of operations, procedures performed, complications encountered, and impact on physical development and pulmonary function tests (PFTs). A total of 191 operations were performed on 130 (16%) of the 792 patients; 98 operations (51%) were abdominal, 58 (30%) thoracic, and 31 (16%) hernias; 64 were male, and 66 female; average age was 14 +/- 10 years. Complications occurred in 12 (16%); 9 deaths were from progressive respiratory failure, 2 from superficial wound infections, and 1 from an episode of line sepsis. In the first 15 years, 9 complications occurred in 126 operations vs. 3 in 73 operations during the last 10 years. Operations were classified as emergent, urgent, or elective. Of the 9 deaths, 8 occurred after emergent or urgent operations (4 abdominal and 4 thoracic), while 1 death occurred following elective herniorrhaphy. For each subgroup, (abdominal, thoracic, and hernia), there was no difference in height/weight indicies, peak flow, forced vital capacity (FVC), forced expired volume in 1 sec (FEV(1)), or FEV(1)/FVC ratio when comparing 1 year preoperation and 1 year postoperation. In conclusion, patients in this high-risk population were operated on with few complications, but when a complication occurred it tended to be pulmonary and fatal (4.7% of all operations). Furthermore, operations did not cause significant deteriorations in PFTs and they did not cause these children to fall off their expected age-adjusted growth curves.

Mycotic aneurysm of the descending thoracic aorta caused by Pseudomonas aeruginosa in a solid organ transplant recipient: case report and review.

BACKGROUND: Pseudomonas aeruginosa is a rare cause of aortic mycotic aneurysms. Optimal treatment, including reconstructive graft material and appropriate length of antibiotic therapy, is being debated. METHODS: We describe a 26-year-old kidney-pancreas recipient who developed an aneurysm of the descending thoracic aorta caused by P. aeruginosa. RESULTS: After surgical debridement and cryopreserved allograft reconstruction, parenteral antibiotics were continued for 12 months, at which time the patient was converted to oral antibiotic therapy. Within 6 months, he redeveloped a thoracic aortic aneurysm, necessitating reoperation and lifelong parenteral antibiotic therapy. CONCLUSION: Herein we review and discuss the relevant literature concerning surgical and antibiotic treatment of mycotic thoracic aneurysms.

Multimodal therapy in the treatment of a venolymphatic malformation of the axilla and chest wall in an infant.

We report our staged multimodal treatment of a female infant with a very large complex venolymphatic malformation of the axilla and chest wall. We successfully managed the patient's severely restricted arm mobility and consumptive coagulopathy with surgical debulking followed by medical therapy with the mammalian target of rapamycin inhibitor sirolimus. The diseased burden reduced in size throughout therapy, and hematologic parameters reached and maintained normal levels. Normal health and limb functionality were restored with no observed adverse side effects of medical therapy. This case presents a previously unreported and potentially promising method to treat severe vascular malformations.

Attenuated Salmonella typhimurium prevents the establishment of unresectable hepatic metastases and improves survival in a murine model.

PURPOSE: The authors investigated the utility of attenuated Salmonella typhimurium for preventing the establishment of hepatic metastases in a murine model. METHODS: A single, oral 10(8) cfu dose of attenuated S typhimurium was given 8 days before the establishment of a model of unresectable hepatic metastases. Animals were assessed for hepatic tumor number and volume, hepatic lymphocyte population analysis, and survival. RESULTS: Pretreatment with Salmonella provided a 10-fold reduction in hepatic tumor burden compared with saline-treated controls. The antitumor effect is associated with markedly elevated natural killer (NK), CD8+ and CD4+ hepatic lymphocytes. Pretreatment with Salmonella provided a 90-day survival rate of 30%, whereas control animals were dead by 30 days. All long-term survivors were devoid of hepatic tumor. CONCLUSIONS: Attenuated S typhimurium effectively prevents the establishment of hepatic metastases in a murine model, providing a clear survival benefit. Thus, it may represent a novel form of in vivo immunotherapy for the prevention of hepatic metastases for patients with locally advanced colorectal cancer.

Cyclooxygenase-2 inhibition augments the hepatic antitumor effect of oral Salmonella typhimurium in a model of mouse metastatic colon cancer.

INTRODUCTION: Oral inoculation with a nontoxic, attenuated strain of Salmonella typhimurium reduces tumor burden and improves survival in a mouse model of metastatic colon cancer. These effects are likely mediated by S. typhimurium-induced increases in hepatic natural killer leukocytes. Cyclooxygenase-2 inhibitors may mediate antitumor effects through antiangiogenic, immune, or proapoptotic pathways. We hypothesized that cyclooxygenase-2 inhibitors would act synergistically with S. typhimurium, resulting in additional antitumor effects. METHODS: Four groups of mice were studied: control, S. typhimurium alone, cyclooxygenase-2 inhibitor alone, and S. typhimurium plus cyclooxygenase-2 inhibitor. Mice were given normal drinking water (control, S. typhimurium alone) or water with 1,600 parts per million cyclooxygenase-2 inhibitor (cyclooxygenase-2 inhibitor alone, and S. typhimurium plus cyclooxygenase-2 inhibitor) and orally inoculated with saline (control, cyclooxygenase-2 inhibitor alone) or 10(9) S. typhimurium (S. typhimurium alone, S. typhimurium plus cyclooxygenase-2 inhibitor). Twenty-four hours later, all mice underwent laparotomy, and 5 x 10(4) MCA38 murine adenocarcinoma cells were injected into the spleen. On Day 14, hepatic tumor number and tumor volume was quantitated and hepatic leukocytes were analyzed by flow cytometry. RESULTS: Compared with control mice orally inoculated with saline, S. typhimurium-treated mice had fewer and smaller tumors; mice treated with cyclooxygenase-2 inhibitor alone had tumor burden similar to control mice, and mice treated with S. typhimurium plus cyclooxygenase-2 inhibitor had fewer and smaller tumors compared with all other groups. Increased liver natural killer cells and decreased CD4+ and CD8+ T cells were observed in both S. typhimurium-treated groups. No alterations in hepatic leukocyte phenotype were observed in mice receiving cyclooxygenase-2 inhibitor alone. CONCLUSION: Oral cyclooxygenase-2 inhibitor appeared to act synergistically with S. typhimurium to reduce tumor burden. This combination therapy may have clinical application in the treatment or prevention of hepatic metastases associated with colorectal cancer.