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Kristin Huntoon, Edward H. Oldfield and Russell R. Lonser

Arvid Lindau, MD, PhD, consolidated the disparate array of benign and malignant visceral and nervous system lesions into the neoplastic syndrome known as von Hippel-Lindau (VHL) disease. Based on this pioneering work, Dr. Lindau was awarded both a Rockefeller fellowship to work in Dr. Harvey Cushing's laboratory in Boston, Massachusetts, and the Lennmalm Prize. While working in Dr. Cushing's laboratory, Dr. Lindau continued his study of CNS hemangioblastomas. His work with Dr. Cushing led to their lifelong friendship and scientific collaboration. In this paper the authors describe Arvid Lindau's pioneering work in nervous system tumor pathology, his relationship to Dr. Cushing, and his role in advancing neurological surgery and research in Europe.

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David Dornbos III, Christy Monson, CNP, Andrew Look, Kristin Huntoon, Luke G. F. Smith, Jeffrey R. Leonard, Sanjay S. Dhall and Eric A. Sribnick

OBJECTIVE

While the Glasgow Coma Scale (GCS) has been effective in describing severity in traumatic brain injury (TBI), there is no current method for communicating the possible need for surgical intervention. This study utilizes a recently developed scoring system, the Surgical Intervention for Traumatic Injury (SITI) scale, which was developed to efficiently communicate the potential need for surgical decompression in adult patients with TBI. The objective of this study was to apply the SITI scale to a pediatric population to provide a tool to increase communication of possible surgical urgency.

METHODS

The SITI scale uses both radiographic and clinical findings, including the GCS score on presentation, pupillary examination, and CT findings. To examine the scale in pediatric TBI, a neurotrauma database at a level 1 pediatric trauma center was retrospectively evaluated, and the SITI score for all patients with an admission diagnosis of TBI between 2010 and 2015 was calculated. The primary endpoint was operative intervention, defined as a craniotomy or craniectomy for decompression, performed within the first 24 hours of admission.

RESULTS

A total of 1524 patients met inclusion criteria for the study during the 5-year span: 1469 (96.4%) were managed nonoperatively and 55 (3.6%) patients underwent emergent operative intervention. The mean SITI score was 4.98 ± 0.31 for patients undergoing surgical intervention and 0.41 ± 0.02 for patients treated nonoperatively (p < 0.0001). The area under the receiver operating characteristic (AUROC) curve was used to examine the diagnostic accuracy of the SITI scale in this pediatric population and was found to be 0.98. Further evaluation of patients presenting with moderate to severe TBI revealed a mean SITI score of 5.51 ± 0.31 in 40 (15.3%) operative patients and 1.55 ± 0.02 in 221 (84.7%) nonoperative patients, with an AUROC curve of 0.95.

CONCLUSIONS

The SITI scale was designed to be a simple, objective communication tool regarding the potential need for surgical decompression after TBI. Application of this scale to a pediatric population reveals that the score correlated with the perceived need for emergent surgical intervention, further suggesting its potential utility in clinical practice.

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Kristin Huntoon, Charles P. Pluto, Lynne Ruess, Daniel R. Boué, Christopher R. Pierson, Jerome A. Rusin and Jeffrey Leonard

OBJECTIVE

Sporadic meningiomas have been classified in many different ways. Radiographically, these lesions can be described as occurring in either typical or atypical locations. The purpose of this study was to determine if there are any histopathological differences between sporadic meningiomas that arise in these varying locations in children.

METHODS

The neuroimaging, histopathological findings, and clinical records in patients with sporadic pediatric meningiomas not associated with neurofibromatosis Type 2 or prior radiation therapy were retrospectively reviewed. Tumors were classified by radiological findings as either typical or atypical, and they were categorized histopathologically by using the latest WHO nomenclature and grading criteria.

RESULTS

Fifteen sporadic meningiomas in pediatric patients were biopsied or resected at the authors’ institution between 1989 and 2013. Five (33%) were typical in radiographic appearance and/or location and 10 (67%) were atypical. Four (80%) typical meningiomas were WHO Grade I tumors. Most (60%) of the atypical meningiomas were WHO Grade II or III.

CONCLUSIONS

This study is the largest series of sporadic pediatric meningiomas in atypical locations to date. Although sporadic meningiomas are relatively infrequent in children, those with atypical imaging, specifically those with apparently intraparenchymal and intraosseous locations, may be more common than previously recognized. In this study, pediatric sporadic meningiomas arising in atypical locations, in particular intraparenchymal meningiomas, may be of higher histopathological grade. The authors’ findings should alert clinicians to the potential for more aggressive clinical behavior in these tumors.

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Russell R. Lonser, John A. Butman, Kristin Huntoon, Ashok R. Asthagiri, Tianxia Wu, Kamran D. Bakhtian, Emily Y. Chew, Zhengping Zhuang, W. Marston Linehan and Edward H. Oldfield

Object

The tumors most frequently associated with von Hippel-Lindau (VHL) disease are hemangioblastomas. While they are associated with significant neurological impairment and mortality, their natural history and optimal management have not been fully defined.

Methods

Patients with VHL were enrolled in a prospective study designed to define the natural history of CNS hemangioblastomas. In the present analysis, serial imaging, laboratory, genetic, and clinical data were evaluated in those with at least 2 years of follow-up data.

Results

At study entrance 225 patients (111 males, 114 females) harbored 1921 CNS hemangioblastomas in the supratentorial compartment (21 tumors [1%]), cerebellum (865 [45%]), brainstem (129 [7%]), spinal cord (689 [36%]), cauda equina (212 [11%]), and nerve roots (5 [0.3%]; follow-up 15,819 hemangioblastoma-years). Increased tumor burden was associated with partial deletions in the VHL gene (p = 0.005) and male sex (p = 0.002). Hemangioblastoma development (median 0.3 new tumors/year) was associated with younger age (p < 0.0001) and more tumors at study entrance (p < 0.0001). While 1278 hemangioblastomas (51%) did not grow, 1227 hemangioblastomas (49%) grew in a saltatory (886 [72%]), linear (76 [6%]), or exponential (264 [22%]) pattern. Faster tumor growth was associated with male sex (p = 0.001), symptomatic tumors (p < 0.0001), and tumors associated with cysts (p < 0.0001). Location-dependent tumor size was the primary predictor of eventual symptom formation (159 symptomatic tumors [6.3%]; area under the curve > 0.9).

Conclusions

Central nervous system hemangioblastoma burden in VHL is associated with partial germline deletions and male sex. Unpredictable growth of hemangioblastomas compromises assessment of nonsurgical therapies. The judicious treatment of symptom-producing hemangioblastomas, while avoiding unnecessary treatment of asymptomatic tumors that may not progress, can provide clinical stability. Clinical trial registration no.: NCT00005902 (ClinicalTrials.gov).

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Kristin Huntoon, Tianxia Wu, J. Bradley Elder, John A. Butman, Emily Y. Chew, W. Marston Linehan, Edward H. Oldfield and Russell R. Lonser

OBJECT

Peritumoral cysts are frequently associated with CNS hemangioblastomas and often underlie neurological morbidity and mortality. To determine their natural history and clinical impact, the authors prospectively analyzed hemangioblastoma-associated peritumoral cysts in patients with von Hippel-Lindau (VHL) disease.

METHODS

Patients with VHL disease who had 2 or more years of follow-up and who were enrolled in a prospective study at the National Institutes of Health were included. Serial prospectively acquired laboratory, genetic, imaging, and clinical data were analyzed.

RESULTS

One hundred thirty-two patients (of 225 in the VHL study with at least 2 years of follow-up) had peritumoral cysts that were followed for more than 2 years (total of 292 CNS peritumoral cysts). The mean age at study entrance was 37.4 ± 13.1 years ([mean ± SD], median 37.9, range 12.3–65.1 years). The mean follow-up was 7.0 ± 1.7 years (median 7.3, range 2.1–9.0 years). Over the study period, 121 of the 292 peritumoral cysts (41.4%) became symptomatic. Development of new cysts was associated with a larger number cysts at study enrollment (p = 0.002) and younger age (p < 0.0001). Cyst growth rate was associated with anatomical location (cerebellum cysts grew faster than spine and brainstem cysts; p = 0.0002 and p = 0.0008), younger age (< 35 years of age; p = 0.0006), and development of new neurological symptoms (p < 0.0001). Cyst size at symptom production depended on anatomical location (p < 0.0001; largest to smallest were found, successively, in the cerebellum, spinal cord, and brainstem). The most common location for peritumoral cysts was the cerebellum (184 cysts [63%]; p < 0.0001).

CONCLUSIONS

Peritumoral cysts frequently underlie symptom formation that requires surgical intervention in patients with VHL disease. Development of new cysts was associated with a larger number of cysts at study enrollment and younger age. Total peritumoral cyst burden was associated with germline partial deletion of the VHL gene.