Atypical teratoid rhabdoid tumors (ATRTs) are rare malignant central nervous system tumors, commonly occurring before 3 years of age. Median overall survival (OS) of patients with these tumors is about 1 year, despite aggressive multimodal therapy. Pediatric primary spinal ATRTs are even more rare, with fewer than 50 cases reported. The authors present a series of four patients who were treated at Ann and Robert H. Lurie Children’s Hospital of Chicago in the period from 1996 to 2017. These patients, with ages 2–11 years, presented with pain and a decline in motor functions. They were found to have lesions in the lumbar, thoracic, and/or cervical spine. One patient’s tumor was intramedullary with exophytic components, while another patient’s tumor had both intra- and extradural components. All patients underwent resection followed by chemotherapy (systemic and intrathecal). Two patients had fractionated radiation therapy and one had an autologous stem cell transplant. Three patients are known to be deceased (OS 8.5–45 months). The fourth patient was in remission 19 years after her initial diagnosis. To the authors’ knowledge, this is the largest series of pediatric primary spinal ATRTs documented at a single institution. These cases illustrate a variety of presentations of spinal ATRT and add to the body of literature on this aggressive pathology. A systematic MEDLINE search was also conducted using the keywords “atypical teratoid rhabdoid tumor,” “pediatric spinal rhabdoid tumor,” and “malignant rhabdoid tumor spine.” Reports were included for patients younger than 21 years, without evidence of intracranial or systemic disease at the time of diagnosis. Clinical characteristics and outcomes of the four institutional cases were compared to those in the literature. This review yielded an additional 48 cases of primary pediatric spinal ATRTs reported in the English-language literature. Patients (ages 2 months to 19 years) presented with symptoms of pain, regression of motor function, and spinal cord compression. The majority of tumors were intradural (14 extramedullary, 8 intramedullary, 1 both). Eleven cases in the literature described tumors limited to extradural structures, while 10 tumors involved the intra- and extradural spine. Four reports did not specify tumor location. Although rare, spinal ATRT should be considered in the differential diagnosis of pediatric patients presenting with a new spinal mass.

Cerebral arteriovenous malformations (AVMs) have been long thought to be a congenital anomaly of vasculogenesis in which arteries and veins form direct connections forming a vascular nidus without an intervening capillary bed or neural tissue. Scattered case reports have described that AVMs may form de novo suggesting they can become an acquired lesion. The current case report describes a patient who presented with new-onset seizures with an initial negative magnetic resonance imaging (MRI) of the brain and subsequently developed an AVM on a MRI 9 years later. This case joins a small, but growing body of literature that challenges the notion that all AVMs are congenital.

Introduction Higher surgical volumes correlate with superior patient outcomes for various surgical pathologies, including transnasal transsphenoidal (TNTS) pituitary tumor resection. With the introduction of endoscopic approaches, there have been nationwide shifts in technique with relative declines in microsurgery. We examined the volume-outcome relationship (VOR) for TNTS pituitary tumor surgery in an era of increasingly prevalent endoscopic approaches. Methods Patients who underwent TNTS pituitary tumor resection between 2009 and 2011 were retrospectively identified in the State Inpatient Database subset of the Healthcare Cost and Utilization Project. Generalized linear mixed-effect models were used to assess odds of various outcome measures. Institutions were grouped into quartiles by case volume for analysis. Results A total of 6,727 patients underwent TNTS pituitary tumor resection between 2009 and 2011. White or Asian American patients and those with private insurance were more likely to receive care at higher volume centers (HVC). Patients treated at HVC (>60 cases/year) were less likely to have nonroutine discharges (3.9 vs. 1.9%; p = 0.002) and had shorter length of stay (LOS; 4 vs. 2 days; p = 0.001). Overall, care at HVC trended toward lower rates of postoperative complications, for example, a 10-case/year increase correlated with a 10% decrease in the rate of iatrogenic panhypopituitarism (odds ratio [OR] = 0.90, 95% confidence interval [CI]: 0.81–0.99; p = 0.04) and 5% decrease in likelihood of diabetes insipidus (OR = 0.95, 95% CI: 0.90–0.99; p = 0.04) on multivariable analysis. Conclusions Our analysis shows that increased case volume is related to superior perioperative outcomes for TNTS pituitary tumor resections. Despite the recent adoption of newer endoscopic techniques and concerns of technical learning curves, this VOR remains undisturbed.