Incidence and management of pleural effusions in patients with Wilms tumor: A Pediatric Surgical Oncology Research Collaborative study.

Wilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi-institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P < .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event-free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage-specific treatment regimens.

Surgical management of myelocystocele: a single-center experience with long-term functional outcomes.

OBJECTIVE: Myelocystocele (MCC) is an uncommon form of skin-covered spinal dysraphism. The authors aimed to present long-term functional outcomes of patients treated for MCC with and without associated abnormalities of cloacal development (ACD). METHODS: All patients with MCC and at least one tethered cord release (TCR) treated at a single institution between 1982 and 2019 were retrospectively reviewed. Demographic, operative, and functional outcome data were analyzed. RESULTS: Of 51 children with MCC, 30 (58.8%) had MCC only and 21 (41.2%) had associated ACD (MCC/ACD). Thirty-two patients (62.7%) had undergone one TCR, while 19 patients (37.3%) had multiple TCRs. Urinary continence assessment was possible in 41 patients (80.4%), and bowel continence assessment was possible in 43 patients (84.3%) who were either older than 6 years or toilet trained. Although patients with MCC only were more likely to void volitionally (p = 0.0001), there was no difference in overall bladder continence based on the presence of ACD (p = 0.15) or the need for additional untethering procedures (p = 0.15). Those with MCC only were more likely to have overall bowel continence (p = 0.0001) and not require any management (p = 0.002), while those with MCC/ACD were more likely to have an ileostomy (p = 0.01). Of the 30 patients with MCC only, 29 (96.7%) were able to ambulate in the community. Of 21 patients with MCC/ACD, 14 (66.7%) were able to ambulate in the community, 5 (23.8%) were not ambulating, and 2 (9.5%) were therapeutic ambulators. A greater proportion of children in the MCC cohort were ambulating in the community (p = 0.01). There was no difference in community ambulation based on the number of TCRs (p > 0.99), but those with multiple TCRs were more likely to use braces (p = 0.01) and require lower-extremity orthopedic surgery (p = 0.01). CONCLUSIONS: Patients born with an MCC, with or without an associated ACD, attained long-term favorable outcomes in bladder and bowel continence and ambulation.