Bilateral Inferior Turbinate Flap for Repair of Skull Base Dehiscence after Proton Beam Radiotherapy for Clival Chordoma

2021 ◽  
Author(s):  
Kayva L. Crawford ◽  
Arvin R. Wali ◽  
Adam S. DeConde ◽  
Thomas L. Beaumont
Keyword(s):  
Skull Base ◽  
Proton Beam ◽  
Inferior Turbinate ◽  
Proton Beam Radiotherapy ◽  
Clival Chordoma

scholarly journals Metastatic clival chordoma: a case report of multiple extraneural metastases following resection and proton beam radiotherapy in a 5-year old boy

2017 ◽  
Vol 19 (5) ◽  
pp. 531-537 ◽  
Author(s):  
Martin J. Rutkowski ◽  
Harjus S. Birk ◽  
Matthew D. Wood ◽  
Arie Perry ◽  
Theodore Nicolaides ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Proton Beam ◽  
Distant Metastases ◽  
Csf Leak ◽  
Communicating Hydrocephalus ◽  
Lung Mass ◽  
Proton Beam Radiotherapy ◽  
Extraneural Metastases ◽  
Clival Chordoma ◽  
Disseminated Disease

The authors report the case of a 5-year-old boy in whom extraneural metastases developed 5 years after he underwent an occipitocervical fusion and transoral approach to treat a clival chordoma without local recurrence. Following primary resection, the patient's postoperative course was complicated by recurrent meningitis secondary to CSF leak, which responded to antibiotics, and communicating hydrocephalus, for which a ventriculoperitoneal shunt was placed. The patient then underwent postoperative proton beam radiotherapy. Five years following his initial presentation, surveillance imaging revealed a new asymptomatic lung mass for which the patient underwent thoracotomy and resection of the mass. Histological examination of the lung mass revealed findings consistent with a de-differentiated chordoma, confirming extraneural metastasis from the original tumor without evidence of local recurrence. Chest wall and scalp metastases subsequently developed, and the patient was started on an adjuvant chemotherapy regimen that included imatinib and rapamycin followed by subsequent nivolumab and an EZH2 inhibitor for recurrent, disseminated disease. Despite this patient's remote and distant metastases, primary gross-total resection for chordoma remains a critical treatment objective, followed by proton beam radiotherapy. This case illustrates the importance of interval posttreatment imaging and the emerging potential to treat chordoma with molecularly targeted therapies.

scholarly journals Numerous Severe Long-Term Sequelae of Skull Base Proton Beam Radiation for Infantile Clival Chordoma: An Illustrative Case Report

2018 ◽  
Vol 79 (S 01) ◽  
pp. S1-S188
Author(s):  
John Hanks ◽  
Kevin Kovatch ◽  
Syed Ali ◽  
Emily Stucken ◽  
Matthew Spector ◽  
...  
Keyword(s):  
Case Report ◽  
Skull Base ◽  
Proton Beam ◽  
Illustrative Case ◽  
Beam Radiation ◽  
Clival Chordoma ◽  
Proton Beam Radiation

Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law

Neurosurgery ◽  
2017 ◽  
Vol 82 (5) ◽  
pp. 652-660 ◽  
Author(s):  
Marcio S Rassi ◽  
M Maher Hulou ◽  
Kaith Almefty ◽  
Wenya Linda Bi ◽  
Svetlana Pravdenkova ◽  
...  
Keyword(s):  
Overall Survival ◽  
Skull Base ◽  
Proton Beam ◽  
Statistical Significance ◽  
Progression Free Survival ◽  
Gross Total Resection ◽  
Total Resection ◽  
Free Survival ◽  
Proton Beam Radiotherapy ◽  
Skull Base Chordomas

Abstract BACKGROUND Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. OBJECTIVE To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors. METHODS Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). RESULTS Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. CONCLUSION Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

scholarly journals Review of skull base chordomas: prognostic factors and long-term results of proton-beam radiotherapy

2001 ◽  
Vol 10 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Eugen B. Hug
Keyword(s):  
Prognostic Factors ◽  
Skull Base ◽  
Proton Beam ◽  
The United States ◽  
Long Term Results ◽  
Severe Toxicity ◽  
Long Term Outcome ◽  
Proton Beam Radiotherapy ◽  
Skull Base Chordomas

Fractionated proton beam radiotherapy has been used for skull base tumors in the United States since the mid-70s, and more than 300 patients in whom diagnosis of chordoma of the skull base has been made have been treated. The ability to achieve high degrees of radiation dose conformity by using protons has resulted in higher radiation doses than can be delivered with conventional radiotherapy in the base of skull. High target volume doses have led to improved tumor control and patient survival. Side effects such as severe toxicity are acceptable considering the alternatives of uncontrolled tumor growth. The authors of various analyses have identified prognostic factors that can be used to predict a patient's chance of treatment success. On the horizon are important technical developments that will further increase dose conformity and increase target doses. In this paper the author reviews long-term outcome data and prognostic predictors for survival of patients with skull base chordomas based on the largest worldwide patient series.

Proton beam radiotherapy: report of the first ten patients treated at the “Centro Nazionale di Adroterapia Oncologica (CNAO)” for skull base and spine tumours

2013 ◽  
Vol 119 (4) ◽  
pp. 277-282 ◽  
Author(s):  
Roberto Orecchia ◽  
Viviana Vitolo ◽  
Maria Rosaria Fiore ◽  
Piero Fossati ◽  
Alberto Iannalfi ◽  
...  
Keyword(s):  
Skull Base ◽  
Proton Beam ◽  
Proton Beam Radiotherapy

scholarly journals Preliminary Result of Hyperfractionated High-Dose Proton Beam Radiotherapy for Pediatric Skull Base Chordomas

2017 ◽  
Vol 08 (04) ◽  
pp. 327-332
Author(s):  
Masashi Mizumoto ◽  
Hiroyoshi Akutsu ◽  
Tetsuya Yamamoto ◽  
Takashi Fukushima ◽  
Yoshiko Oshiro ◽  
...  
Keyword(s):  
Skull Base ◽  
Proton Beam ◽  
High Dose ◽  
Proton Beam Radiotherapy ◽  
Skull Base Chordomas

Complications after Proton Beam Therapy to Skull Base Tumors: Case Series

2019 ◽  
Author(s):  
Gustavo Rangel ◽  
Mostafa Shahein ◽  
Thiago Felicio ◽  
Guilhermo Malave ◽  
Nyall London ◽  
...  
Keyword(s):  
Skull Base ◽  
Proton Beam ◽  
Case Series ◽  
Proton Beam Therapy ◽  
Skull Base Tumors

Proton Beam Therapy for Unresectable Adenoid Cystic Carcinoma of the Skull Base

Skull Base ◽  
2008 ◽  
Vol 18 (S 01) ◽  
Author(s):  
Annie Chan ◽  
Paul Busse ◽  
Urmila Kamat ◽  
Derrick Lin ◽  
Norbert Liebsch
Keyword(s):  
Adenoid Cystic Carcinoma ◽  
Skull Base ◽  
Proton Beam ◽  
Proton Beam Therapy ◽  
Adenoid Cystic
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