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The clinical course and role of surgery in pediatric malignant peripheral nerve sheath tumors: a database study

Victor M. Lu, Shelly Wang, David J. Daniels, Robert J. Spinner, Allan D. Levi, and Toba N. Niazi

OBJECTIVE

Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors found throughout the body, with their clinical course in children still not completely understood. Correspondingly, this study aimed to determine survival outcomes and specific clinical predictors of survival in this population from a large national database.

METHODS

All patients with MPNSTs aged ≤ 18 years in the US National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized, and overall survival was modeled using Kaplan-Meier and Cox regression analyses.

RESULTS

A total of 251 pediatric patients with MPNSTs (132 [53%] females and 119 [47%] males) were identified; the mean age at diagnosis was 13.1 years (range 1–18 years). There were 84 (33%) MPNSTs located in the extremities, 127 (51%) were smaller than 1 cm, and 22 (9%) had metastasis at the time of diagnosis. In terms of treatment, surgery was pursued in 187 patients (74%), chemotherapy in 116 patients (46%), and radiation therapy in 129 patients (61%). The 5-year overall survival rate was estimated at 52% (95% CI 45%–59%), with a median survival of 64 months (range 36–136 months). Multivariate regression revealed that older age (HR 1.10, p < 0.01), metastases at the time of diagnosis (HR 2.14, p = 0.01), and undergoing biopsy only (HR 2.98, p < 0.01) significantly and independently predicted a shorter overall survival. Chemotherapy and radiation therapy were not statistically significant.

CONCLUSIONS

In this study, the authors found that older patient age, tumor metastases at the time of diagnosis, and undergoing only biopsy significantly and independently predicted poorer outcomes. Only approximately half of patients survived to 5 years. These results have shown a clear survival benefit in pursuing maximal safe resection in pediatric patients with MPNSTs. As such, judicious workup with meticulous resection by an expert team should be considered the standard of care for these tumors in children.

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Triceps motor branch transfer for isolated traumatic pediatric axillary nerve injuries

Harvey Chim, Michelle F. Kircher, Robert J. Spinner, Allen T. Bishop, and Alexander Y. Shin

OBJECT

Transfer of the triceps motor branch has been used for treatment of isolated axillary nerve palsy in the adult population. However, there are no published data on the effectiveness of this procedure in the pediatric population with traumatic injuries. The authors reviewed demographics and outcomes in their series of pediatric patients who underwent this procedure.

METHODS

Six patients ranging in age from 10 to 17 years underwent triceps motor branch transfer for the treatment of isolated axillary nerve injuries between 4 and 8 months after the inciting injury. Deltoid muscle strength was evaluated using the modified British Medical Research Council (MRC) grading system. Shoulder abduction at last follow-up was measured.

RESULTS

The mean duration of follow-up was 38 months. The average postoperative MRC grading of deltoid muscle strength was 3.6 ± 1.3. The median MRC grade was 4. One patient who did not achieve an MRC grade of 3 suffered multiple injuries from high-velocity trauma. Unlike in the adult population, age, body mass index of the patient, and delay from injury to surgery were not significant factors affecting the outcome of the procedure.

CONCLUSIONS

In the pediatric population with traumatic injuries, isolated axillary nerve injury treated with triceps motor branch transfer can result in good outcomes.

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Traumatic peripheral nerve injuries in children: epidemiology and socioeconomics

Clinical article

Symeon Missios, Kimon Bekelis, and Robert J. Spinner

Object

Despite the negative effects of peripheral nerve injuries (PNIs) on long-term population health, their true prevalence among pediatric trauma patients is under debate. The authors investigated the prevalence of PNIs among children involved in trauma and investigated associations between PNIs and several patient characteristics.

Methods

The authors performed a retrospective cohort study of pediatric trauma patients who were registered in the National Trauma Data Bank from 2009 through 2011 and who fulfilled the study inclusion criteria. They used regression techniques to investigate the association of demographic and socioeconomic factors with the rate of PNIs among these patients.

Results

Of the 245,470 study patients, 50,211 were involved in motor vehicle crashes, 3380 in motorcycle crashes, 20,491 in bicycle crashes, 18,262 in pedestrian accidents, 26,294 in other crashes (mainly involving all-terrain vehicles and snowmobiles), and 126,832 in falls. The respective prevalence of PNIs was 0.66% for motor vehicle crashes, 1% for motorcycle crashes, 0.38% for bicycle crashes, 0.42% for pedestrian accidents, 0.79% for other crashes, and 0.52% for falls. Multivariate logistic regression analysis demonstrated that the following were associated with an increased incidence of PNIs: increased patient age (OR 1.10, 95% CI 1.01–1.20), higher Injury Severity Score (OR 1.10, 95% CI 1.01–1.20), elevated systolic blood pressure at arrival at the emergency room (OR 1.10, 95% CI 1.01–1.20), and increased number of trauma surgeons at the institution (OR 1.10, 95% CI 1.01–1.20). The following were associated with lower incidence of PNIs: female sex (OR 0.94, 95% CI 0.87–1.02), rural hospitals (OR 0.94, 95% CI 0.87–1.02), and urban nonteaching hospitals (OR 0.94, 95% CI 0.87–1.02).

Conclusions

PNIs are more common than previously identified for the pediatric trauma population. These injuries are associated with older age and increased severity of the overall injury.