Texas Children’s Hospital opened its doors in 1954, and since that time the institution has remained dedicated to a three-part mission: patient care, education, and research. Dr. William R. Cheek developed an early interest in pediatric neurosurgery, which led to his efforts in building and developing a service at Texas Children’s Hospital at a time when the field was just emerging. His work with other early pioneers in the field led to the establishment of organized societies, educational texts, and governing bodies that have led to significant advances in the field over the past 50 years.
Nisha Gadgil, Ganesh Rao, Raymond Sawaya, Daniel Yoshor, Lucia Ruggieri, Natalie Cormier, Daniel J. Curry, William E. Whitehead, Guillermo Aldave, David F. Bauer, Samuel McClugage III, and Howard L. Weiner
Vijay M. Ravindra, M. Fatih Okcu, Lucia Ruggieri, Thomas S. Frank, Arnold C. Paulino, Susan L. McGovern, Vincent E. Horne, Robert C. Dauser, William E. Whitehead, and Guillermo Aldave
The authors compared survival and multiple comorbidities in children diagnosed with craniopharyngioma who underwent gross-total resection (GTR) versus subtotal resection (STR) with radiation therapy (RT), either intensity-modulated radiation therapy (IMRT) or proton beam therapy (PBT). The authors hypothesized that there are differences between multimodal treatment methods with respect to morbidity and progression-free survival (PFS).
The medical records of children diagnosed with craniopharyngioma and treated surgically between February 1997 and December 2018 at Texas Children’s Hospital were reviewed. Surgical treatment was stratified as GTR or STR + RT. RT was further stratified as PBT or IMRT; PBT was stratified as STR + PBT versus cyst decompression (CD) + PBT. The authors used Kaplan-Meier analysis to compare PFS and overall survival, and chi-square analysis to compare rates for hypopituitarism, vision loss, and hypothalamic obesity (HyOb).
Sixty-three children were included in the analysis; 49% were female. The mean age was 8.16 years (95% CI 7.08–9.27). Twelve of 14 children in the IMRT cohort underwent CD. The 5-year PFS rates were as follows: 73% for GTR (n = 31), 54% for IMRT (n = 14), 100% for STR + PBT (n = 7), and 77% for CD + PBT (n = 11; p = 0.202). The overall survival rates were similar in all groups. Rates of hypopituitarism (96% GTR vs 75% IMRT vs 100% STR + PBT, 50% CD + PBT; p = 0.023) and diabetes insipidus (DI) (90% GTR vs 61% IMRT vs 85% STR + PBT, 20% CD + PBT; p = 0.004) were significantly higher in the GTR group. There was no significant difference in the HyOb or vision loss at the end of study follow-up among the different groups. Within the PBT group, 2 patients presented a progressive vasculopathy with subsequent strokes. One patient experienced a PBT-induced tumor.
GTR and CD + PBT presented similar rates of 5-year PFS. Hypopituitarism and DI rates were higher with GTR, but the rate of HyOb was similar among different treatment modalities. PBT may reduce the burden of hypopituitarism and DI, although radiation carries a risk of potential serious complications, including progressive vasculopathy and secondary malignancy. Further prospective study comparing neurocognitive outcomes is necessary.
Vijay M. Ravindra, Lucia Ruggieri, Sanjeev A. Vasudevan, M. John Hicks, Jodi A. Muscal, Norma M. Quintanilla, Deborah A. Schady, and Guillermo Aldave
Pediatric germ cell tumors (GCTs) are neoplasms that originate from primordial germ cells and, according to their site of presentation, are classified as gonadal or extragonadal. The most common site of extragonadal GCTs in children is the sacrococcygeal region, and the standard management is multimodal with a focus on chemotherapy. In selected instances, sacrococcygeal resection is performed. Herein, the authors report on 2 patients who presented with presacral yolk sac tumors managed with multimodal treatment. Both patients underwent salvage sacrococcygeal resection for oncological control and surgical removal of the sacral vertebral elements: a 27-month-old girl with a recurrent sacrococcygeal yolk sac tumor following chemotherapy and initial resection and a 24-month-old boy in whom a primary sacrococcygeal yolk sac tumor was resected following chemotherapy. These 2 cases illustrate the complexity in the management of these unusual tumors and will help neurosurgeons with the understanding of yolk sac tumors in the sacrococcygeal region.