Mitesh V. Shah

Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

PURPOSE To report the results in patients reirradiated with proton therapy for recurrent or progressive chordoma, with or without salvage surgery. METHODS AND MATERIALS A retrospective review of 16 consecutive patients treated from 2005 to 2012 was performed. All patients had received at least 1 prior course of radiation therapy to the same area, and all but 1 patient had at least 1 surgical resection for disease before receiving reirradiation. At the time of recurrence or progression, half of the patients underwent additional salvage surgery before receiving reirradiation. The median prior dose of radiation was 75.2 Gy (range, 40-79.2 Gy). Six patients had received prior proton therapy, and the remainder had received photon radiation. The median gross tumor volume at the time of reirradiation was 71 cm(3) (range, 0-701 cm(3)). Reirradiation occurred at a median interval of 37 months after prior radiation (range, 12-129 months), and the median dose of reirradiation was 75.6 Gy (relative biological effectiveness [RBE]) (range. 71.2-79.2 Gy [RBE]), given in standard daily fractionation (n=14) or hyperfractionation (n=2). RESULTS The median follow-up time was 23 months (range, 6-63 months); it was 26 months in patients alive at the last follow-up visit (range, 12-63 months). The 2-year estimate for local control was 85%, overall survival 80%, chordoma-specific survival 88%, and development of distant metastases 20%. Four patients have had local progression: 3 in-field and 1 marginal. Late toxicity included grade 3 bitemporal lobe radionecrosis in 1 patient that improved with hyperbaric oxygen, a grade 4 cerebrospinal fluid leak with meningitis in 1 patient, and a grade 4 ischemic brainstem stroke (out of radiation field) in 1 patient, with subsequent neurologic recovery. CONCLUSIONS Full-dose proton reirradiation provided encouraging initial disease control and overall survival for patients with recurrent or progressive chordoma, although additional toxicities may develop with longer follow-up times.

Influence of Residual Tumor Volume and Radiation Dose Coverage in Outcomes for Clival Chordoma

PURPOSE The purpose of this study was to evaluate factors associated with tumor control in clival chordomas. METHODS AND MATERIALS A retrospective review of 39 patients treated with surgery and proton therapy for clival chordomas between 2004 and 2014 was performed. The median prescribed dose was 77.4 Gy (relative biological effectiveness [RBE]); range was 70.2-79.2 Gy (RBE). Minimum and median doses to gross tumor volume (GTV), radiation dose received by 1 cm(3) of GTV (D1cm(3)), and the equivalent uniform dose were calculated. Receiver operating characteristics curves evaluated the predictive sensitivity and specificity for local failure of potential cutpoint values for GTV and D1cm(3). RESULTS After a median follow-up of 51 months, the 5-year estimate of local control (LC) was 69.6% (95% confidence interval [CI] 50.0%-89.2%), and overall survival (OS) was 81.4% (95% CI: 65.3%-97.5%). Tumor histology, GTV at the time of radiation, and prescribed radiation dose were significantly associated with local control on multivariate analysis, whereas D1cm(3) was associated with overall survival. Compared to those patients whose conditions remained controlled, patients experiencing tumor failure had statistically significant larger GTVs and lower D1cm(3), and prescribed and median doses to GTV. A subset of 21 patients with GTV of ≤20 cm(3) and D1cm(3) of >67 Gy (RBE) had a median follow-up of 47 months. The 5-year estimate of local control in this subset was 81.1% (95% CI: 61.7%-100%; P=.004, overall comparison by GTV ≤20 cm(3) stratified by D1cm(3)). A D1cm(3) of 74.5 Gy (RBE) had 80% sensitivity for local control and 60% specificity, whereas a GTV of 9.3 cm(3) had 80% sensitivity for local control and 66.7% specificity. CONCLUSIONS Local control of clival chordomas was associated with both smaller size of residual tumor and more complete high-dose coverage of residual tumor. Multidisciplinary care should seek maximal safe surgical resection, particularly to facilitate delivery of high-dose radiation therapy in proximity to critical structures. A D1cm(3) ≥74.5 Gy (RBE) represents a proposed treatment planning objective.

Posterior interhemispheric transfalcine transprecuneus approach for microsurgical resection of periatrial lesions: indications, technique, and outcomes.

OBJECT Surgical exposure of the peritrigonal or periatrial region has been challenging due to the depth of the region and overlying important functional cortices and white matter tracts. The authors demonstrate the operative feasibility of a contralateral posterior interhemispheric transfalcine transprecuneus approach (PITTA) to this region and present a series of patients treated via this operative route. METHODS Fourteen consecutive patients underwent the PITTA and were included in this study. Pre- and postoperative clinical and radiological data points were retrospectively collected. Complications and extent of resection were reviewed. RESULTS The mean age of patients at the time of surgery was 39 years (range 11-64 years). Six of the 14 patients were female. The mean duration of follow-up was 4.6 months (range 0.5-19.6 months). Pathology included 6 arteriovenous malformations, 4 gliomas, 2 meningiomas, 1 metastatic lesion, and 1 gray matter heterotopia. Based on the results shown on postoperative MRI, 1 lesion (7%) was intentionally subtotally resected, but ≥ 95% resection was achieved in all others (93%) and gross-total resection was accomplished in 7 (54%) of 13. One patient (7%) experienced a temporary approach-related complication. At last follow-up, 1 patient (7%) had died due to complications of his underlying malignancy unrelated to his cranial surgery, 2 (14%) demonstrated a Glasgow Outcome Scale (GOS) score of 4, and 11 (79%) manifested a GOS score of 5. CONCLUSIONS Based on this patient series, the contralateral PITTA potentially offers numerous advantages, including a wider, safer operative corridor, minimal need for ipsilateral brain manipulation, and better intraoperative navigation and working angles.

Accuracy of contrast-enhanced MDCT and MRI for identifying the severity and cause of neural foraminal stenosis in cervical radiculopathy: A prospective study

OBJECTIVE The purpose of this study was to determine the accuracy of IV contrast-enhanced MDCT and MRI for evaluation of the severity and cause of neural foraminal stenosis in patients with cervical radiculopathy. SUBJECTS AND METHODS Eighteen patients with cervical radiculopathy prospectively underwent contrast-enhanced MDCT and MRI. Contrast-enhanced MDCT scans were acquired at 1-mm thickness and reconstructed in oblique axial (parallel to disk) and sagittal (perpendicular to neural foramen) 2-mm sections without a gap. The MRI sequences used were sagittal T1-weighted, fast spin-echo T2-weighted, 3D fast spin-echo T2-weighted, axial T2-weighted, and 3D gradient-recalled echo. Three neuroradiologists independently and blindly rated the severity and cause of neural foraminal stenosis on a 4-point scale. Using the same scale at surgery, one of three surgeons rated the severity and cause of neural foraminal stenosis, and the results were used as the reference standard. Interobserver and intraobserver agreement (kappa) was calculated. RESULTS For severity of neural foraminal stenosis, the sensitivities of contrast-enhanced MDCT (50/55, 91%) and MRI (55/57, 96%) were similar, as were their specificities (contrast-enhanced MDCT, 13/24, 54%; MRI, 11/24, 46%). For cause of neural foraminal stenosis, the accuracies of contrast-enhanced MDCT (46/54, 85%) and MRI (45/57, 79%) were similar. Interobserver agreement on severity of neural foraminal stenosis was moderate to almost perfect for contrast-enhanced MDCT (kappa=0.50-1.00) and MRI (kappa=0.43-1.00). For cause of neural foraminal stenosis, interobserver agreement was moderate to substantial for contrast-enhanced MDCT (kappa=0.52-0.76) but only fair for MRI (kappa=0.23-0.39). Intra observer agreement was very high for severity of neural foraminal stenosis (contrast-enhanced MDCT, kappa=0.85; MRI, kappa=0.80) and cause of neural foraminal stenosis (contrast-enhanced MDCT, kappa=0.86; MRI, kappa=1.00). CONCLUSION Contrast-enhanced MDCT is as accurate as MRI in evaluation of the severity and cause of neural foraminal stenosis and may have better interobserver agreement.

Malignant familial glomus jugulare and contralateral carotid body tumor.

Paragangliomas originate from neural crestderived tissue located throughout the body.’ Overall, the most common site for a paraganglioma is the adrenal medulla (pheochromocytoma). In the head and neck, paragangliomas occur most frequently in the carotid body which, along with the carotid sinus, serves as a chemoreceptor and baroreceptor.2 Paraganglia tissue is found in other head and neck locations including the larynx, orbit and paranasal sinuses, and in the jugulotympanic area along cranial nerves IX and X.3 Paragangliomas arising in the jugulotympanic region are termed glomus (from the Latin glomer: a ball) tumors and are classified by their site of origin into glomus jugulare

Spinal surgery following organ transplantation.

OBJECT Organ transplantation for renal, liver, cardiac, and pulmonary failure has become more common in recent years, and patients are living longer as a result of improved organ preservation methods, immunosuppressive regimens, and general posttransplant care. Some of these patients undergo spine fusion surgery following organ transplantation, and there is little available information concerning outcomes. The authors report on their experience with and the outcomes of spine fusion in this rare and unique immunosuppressed patient group. METHODS Using the Current Procedural Terminology and ICD-9 codes for solid organ transplants, bone marrow transplantations (BMTs), and spine fusion surgeries, the authors searched their patient database between 1997 and 2008. Data points of interest included primary diagnosis, type of organ transplant, immunosuppressant drug therapy, complications from spine surgery, and radiographic analysis of spine fusion. Spine fusion was assessed with CT or radiography at the latest follow-up. RESULTS The database search results revealed 5999 patients who underwent heart, lung, liver, kidney, pancreas, intestine, or bone marrow transplant between 1997 and 2008. Eighteen of the 5999 patients underwent a spine fusion surgery while receiving immunosuppressive therapy. Organ transplants included kidney, liver, heart, pancreas, and allogenic BMT. There were 3 deaths unrelated to spine fusion within 1 year of the surgery and 1 death immediately after spine surgery. Graft-versus-host disease developed in 1 patient when prednisone was stopped prior to the spine surgery. Thirteen patients underwent follow-up radiographic imaging at an average of 25 months after spine surgery; 12 demonstrated radiographic fusion. CONCLUSIONS The results suggest that spine fusion rates are adequate despite immunosuppressive therapy in patients undergoing spinal fusion after transplant procedures. The data also illustrate the high morbidity and mortality rates found in the organ transplant patient population.

Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review.

Background: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures. There are only reports of these tumors presenting acutely with intracranial hemorrhage in adult patients. Case Description: A 10-year-old boy presented with acute onset of headache, emesis, and diplopia. Head computed tomography and magnetic resonance imaging of brain were suspicious for a hemorrhagic mass located in the left petroclival region, compressing the ventral pons. The mass was surgically resected and demonstrated acute intratumoral hemorrhage. Pathologic examination was consistent with chordoma. Conclusion: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors’ knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.

Metastasis of soft-tissue myoepithelial carcinoma to clivus

The differential diagnosis for masses involving the clivus is broad. The authors present a case of myoepithelial carcinoma metastatic to the clivus, a lesion that has not been reported to their knowledge. This 14-year-old girl with a history of myoepithelial carcinoma originating in the soft tissues of the left hip and metastatic to the lung presented with left lateral gaze palsy. Imaging demonstrated a 3 × 3-cm osteolytic mass in the clivus. Microscopic transsphenoidal resection with endoscopic assistance was performed. Pathological findings were consistent with the previously diagnosed myoepithelial carcinoma. Within 4 weeks postoperatively and 2 weeks into a chemotherapeutic regimen, the tumor exhibited progression. Radiation therapy was started and growth of the tumor was halted. Myoepithelial carcinoma should be included in the differential diagnosis for clival masses, especially in patients with previously diagnosed myoepithelial carcinoma. The primary management of this tumor should be with chemotherapy and radiation, with surgery serving only for decompression.

Ciliochoroidal ganglioneuroma in neurofibromatosis type 1: Report of a case and review of the literature.

Orbitofacial neurofibromatosis (OFNF) is considered a variant of neurofibromatosis type 1 (NF1). OFNF most often affects the eye, orbit and one side of the face. It is characterized by the development of relatively aggressive and disfiguring lesions, including plexiform and diffuse neurofibromas. Ciliochoroidal ganglioneuromas have not been previously reported in patients with this syndrome. We report the case of a 50‐year‐old man with OFNF, ciliochoroidal ganglioneuroma and a large ipsilateral frontoethmoidal encephalocele.

Technology that achieves the Triple Aim: an economic analysis of the BrainPath TM approach in neurosurgery

Background The Triple Aim is defined as: improving the patient experience of care, improving the health of populations, and reducing the per capita cost of health care. The purpose of this analysis was to evaluate the economic value of a new neurosurgical technique, the BrainPath™ approach, for use in patients with subcortical tumors and intracerebral hemorrhage (ICH). Methods Inpatient length of stay (LOS) data were collected for ICH and brain tumor surgical patient cases between August 2013 and November 2015. Patient cases were separated into two groups; BrainPath approach (n = 28) and conventional techniques, such as craniotomy, (n = 208). The average intensive care unit (ICU) LOS was calculated for each group by diagnosis-related group and compared between groups. Results The new surgical technology resulted in surgical intervention in 14 ICH cases which otherwise would have been medically managed due to the hemorrhage location or size of the ICH. A reduction in ICU LOS was seen in this group. Based on the variable direct cost per day in the neuro critical care unit at this academic medical center, 14 patient cases incurred ~ US