Charles G. Fisher, Tony Goldschlager, Stefano Boriani, Peter Paul Varga, Laurence D. Rhines, Michael G. Fehlings, Alessandro Luzzati, Mark B. Dekutoski, Jeremy J. Reynolds, Dean Chou, Sigurd H. Berven, Richard P. Williams, Nasir A. Quraishi, Chetan Bettegowda and Ziya L. Gokaslan
The National Institutes of Health recommends strategies to obtain evidence for the treatment of rare conditions such as primary tumors of the spine (PTSs). These tumors have a low incidence and are pathologically heterogeneous, and treatment approaches are diverse. Appropriate evidence-based care is imperative. Failure to follow validated oncological principles may lead to unnecessary mortality and profound morbidity. This paper outlines a scientific model that provides significant evidence guiding the treatment of PTSs.
A four-stage approach was used: 1) planning: data from large-volume centers were reviewed to provide insight; 2) recruitment: centers were enrolled and provided the necessary infrastructure; 3) retrospective stage: existing medical records were reviewed and completed with survival data; and 4) prospective stage: prospective data collection has been implemented. The AOSpine Knowledge Forum Tumor designed six modules: demographic, clinical, diagnostic, therapeutic, local recurrence, survival, and perioperative morbidity data fields and provided funding.
It took 18 months to implement Stages 1–3, while Stage 4 is ongoing. A total of 1495 tumor cases were captured and diagnosed as one of 18 PTS histotypes. In addition, a PTS biobank network has been created to link clinical data with tumor pathology and molecular analysis.
This scientific model has not only aggregated a large amount of PTS data, but has also established an international collaborative network of spine oncology centers. Access to large volumes of data will generate further research to guide and enhance PTS clinical management. This model could be applied to other rare neoplastic conditions. Clinical trial registration no.: NCT01643174 (ClinicalTrials.gov).
Chetan Bettegowda, Owoicho Adogwa, Vivek Mehta, Kaisorn L. Chaichana, Jon Weingart, Benjamin S. Carson, George I. Jallo and Edward S. Ahn
Choroid plexus tumors (CPTs) are rare intracranial neoplasms that constitute approximately 2%–5% of all pediatric brain tumors. Most of these tumors present with severe hydrocephalus. The optimal perioperative management and oncological care remain a matter of debate. The authors present the epidemiological and clinical features of CPTs from a 20-year single-institutional experience.
A total of 39 consecutive patients with pathologically proven CPTs (31 choroid plexus papillomas [CPPs] and 8 choroid plexus carcinomas [CPCs]) were included in this series. Patient demographics, clinical presentation, comorbidities, indications for surgery, radiological studies, tumor location, and all operative variables were reviewed for each case. Multivariate regression analysis was performed to identify independent predictors of tumor recurrence and survival.
The overall mean age (± SD) was 13.13 ± 19.59 years (15.27 ± 21.10 years in the CPP group and 3.66 ± 3.59 years in the CPC group). Hydrocephalus was noted at presentation in 34% of patients. The most common presenting symptoms were headache (32%) and nausea/vomiting (26%). Gross-total resection (GTR) was achieved in 86% of CPPs and in 71% of CPCs (p = 0.57). There was 100% survival in patients with CPPs observed at the 5- and 10-year follow-up and 71% survival in patients with CPCs at the 5-year follow-up. In a multivariate regression analysis, a diagnosis of papilloma, preoperative vision changes, or hydrocephalus; right ventricle tumor location; and GTR were all independently associated with a decreased likelihood of tumor recurrence at last follow-up.
The authors' study suggests that patients with CPCs are more likely to experience local recurrence and metastasis; hence, GTR with chemotherapy and radiotherapy, particularly for CPCs, is pivotal in preventing recurrence and prolonging survival. While GTR was important for local control following resection of CPPs, it had a minimal effect on prolonging survival in this patient cohort.
Vivek A. Mehta, Chetan Bettegowda, George I. Jallo and Edward S. Ahn
Craniosynostosis, the premature closure of cranial sutures, has been known to exist for centuries, but modern surgical management has only emerged and evolved over the past 100 years. The success of surgery for this condition has been based on the recognition of scientific principles that dictate brain and cranial growth in early infancy and childhood. The evolution of strip craniectomies and suturectomies to extensive calvarial remodeling and endoscopic suturectomies has been driven by a growing understanding of how a prematurely fused cranial suture can affect the growth and shape of the entire skull. In this review, the authors discuss the early descriptions of craniosynostosis, describe the scientific principles upon which surgical intervention was based, and briefly summarize the eras of surgical management and their evolution to present day.
Vivek A. Mehta, Chetan Bettegowda, Sebastian A. Ahmadi, Petra Berenberg, Ulrich-Wilhelm Thomale, Ernst-Johannes Haberl, George I. Jallo and Edward S. Ahn
Symptom response to spinal cord untethering, and the impact of duraplasty and scoliosis on retethering, are poorly understood in tethering after myelomeningocele (MMC) repair. In this retrospective study, the authors examined the outcomes of children who developed first-time spinal cord tethering following MMC repair. The response of symptoms to untethering and the role of duraplasty and scoliosis in retethering are explored.
The authors performed a review of 54 children with first-time symptomatic spinal cord tethering following MMC repair to determine the impact of untethering on symptoms, the impact of dural repair type on retethering, and the role of scoliosis on the prevalence and time to retethering.
The average patient age was 10.3 ± 4.9 years, and 44% were males. The most common presenting symptoms of tethered cord syndrome were urinary (87%), motor (80%), gait (78%), and sensory (61%) dysfunction. The average postoperative time to symptom improvement was 2.02 months for sensory symptoms, 3.21 months for pain, 3.50 months for urinary symptoms, and 4.48 months for motor symptoms, with sensory improvement occurring significantly earlier than motor improvement (p = 0.02). At last follow-up (an average of 47 months), motor symptoms were improved in 26%, maintained in 62%, and worsened in 11%; for sensory symptoms, these rates were 26%, 71%, and 3%, respectively; for pain, 28%, 65%, and 7%, respectively; and for urinary symptoms, 17%, 76%, and 7%, respectively. There was no difference in symptom response with type of dural repair (primary closure vs duraplasty). Symptomatic retethering occurred in 17 (31%) of 54 patients, but duration of symptoms, age at surgery, and type of dural repair were not associated with retethering. Scoliosis was not associated with an increased prevalence of retethering, but was associated with significantly earlier retethering (32.5 vs 61.1 months; p = 0.042) in patients who underwent additional untethering operations.
Symptomatic retethering is a common event after MMC repair. In the authors' experience, sensory improvements occur sooner than motor improvements following initial untethering. Symptom response rates were not altered by type of dural closure. Scoliosis was associated with significantly earlier retethering and should be kept in mind when caring for individuals who have had previous MMC repair.
Vivek A. Mehta, Chetan Bettegowda and George I. Jallo
Tumoral calcinosis (TC), a calcium hydroxyapatite–based mass, is common in the extremities and hips, but has rarely been reported in the spine, and has never been reported within the spinal cord. It may occur sporadically, in familial form, or as a consequence of disorders that promote soft-tissue calcification. Gross-total resection appears to be curative, but the diagnosis of TC is rarely considered prior to surgery. In this report, the authors describe the management of the first case of intramedullary TC located at the T-5 level in a 20-month-old boy who presented with lower-extremity spasticity. Additionally, salient features of the TC diagnosis, radiological patterns, histological findings, treatment, and outcomes are discussed.