Comparison of Clinical, Histopathological, and Genomic Features Between Malignant Peripheral Nerve Sheath Tumors and Cellular Schwannomas of the Eighth Cranial Nerve: A Case Series

2019 ◽  
Vol 122 ◽  
pp. e487-e497
Author(s):  
Fu Zhao ◽  
Shun Zhang ◽  
Jiang Du ◽  
Yang Chen ◽  
Bo Wang ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Case Series ◽  
Eighth Cranial Nerve ◽  
Genomic Features ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation

2016 ◽  
Vol 125 (5) ◽  
pp. 1120-1129 ◽  
Author(s):  
Matthew L. Carlson ◽  
Jeffrey T. Jacob ◽  
Elizabeth B. Habermann ◽  
Amy E. Glasgow ◽  
Aditya Raghunathan ◽  
...  
Keyword(s):  
Systematic Review ◽  
Peripheral Nerve ◽  
Cranial Nerve ◽  
English Literature ◽  
Seer Database ◽  
Eighth Cranial Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented. METHODS The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented. RESULTS The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000–0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process. Treatment included microsurgery alone, microsurgery with adjuvant radiation, or microsurgery with chemoradiation. Sixty-one percent of patients receiving treatment experienced recurrence, 22% of which were diagnosed with drop metastases to the spine. Ultimately, 13 patients (54%) died of progressive disease at a median of 3 months following diagnosis. The ability to achieve gross-total resection was the only feature that was associated with improved disease-specific survival. CONCLUSIONS MPNSTs of the eighth CN are extremely rare and portend a poor prognosis. Nearly half of patients initially present with findings consistent with a benign VS, often making an early diagnosis challenging. In light of these data, early radiological and clinical follow-up should be considered in those who elect nonoperative treatment, particularly in patients with a short duration of symptoms or atypical presentation. These data also provide a baseline rate of malignancy that should be considered when estimating the risk of malignant transformation following stereotactic radiosurgery for VS.

Malignant Peripheral Nerve Sheath Tumors of the 8th Cranial Nerve Arising without Prior Irradiation

2016 ◽  
Vol 77 (S 01) ◽  
Author(s):  
Matthew Carlson ◽  
Jeffrey Jacob ◽  
Elizabeth Habermann ◽  
Amy Wagie ◽  
Aditya Raghunathan ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

scholarly journals P.098 Evaluation and surgical management of pelvic peripheral nerve sheath tumors: the University of Toronto experience and review of literature

2017 ◽  
Vol 44 (S2) ◽  
pp. S38-S38
Author(s):  
S Wang ◽  
Z Merali ◽  
F Pirouzmand
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Case Series ◽  
Tumor Location ◽  
Presenting Symptoms ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Imaging Characteristics ◽  
Surgical Technical

Background: Pelvic peripheral nerve sheath tumors (PNST), which includes neurofibroma, schwannoma, and MPNST, are rare tumors located in the retroperitoneum. Methods: The case records of a prospectively maintained database at Sunnybrook Health Sciences Center (SHSC) were reviewed to identify patients with pelvic PNST, managed between 2006 - 2016. Medical records were retrospectively reviewed for patient demographics, presentation, tumor location, symptoms, imaging characteristics, management, and outcome. The surgical technical caveats were described. An English language literature review was performed to describe previously published experiences. Results: The series consisted of 7 patients, ranging from 22 - 74 years of age at presentation. These lesions tend to be large at the time of diagnosis, and presenting symptoms include abdominal, flank, or back pain, as well as leg edema or hydronephrosis from local compression. Most patients in this cohort were managed surgically with midline abdominal transperitoneal exposures. Lastly, 5 tumors were benign schwannomas managed with gross total resection or debulking, while 2 patients had MPNSTs managed with biopsy followed by adjuvant chemoradiation therapy. Conclusions: In this case series, we describe the characteristics, evaluation, and management of 7 patients with pelvic PNST at a major healthcare institution in Toronto, Canada, highlighting the technical aspects of managing this rare and challenging entity.

scholarly journals Malignant Peripheral Nerve Sheath Tumors of the Head and Neck: A Case Series and Literature Review

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Brandon T. Mullins ◽  
Trevor Hackman
Keyword(s):  
Peripheral Nerve ◽  
Head And Neck ◽  
Adjuvant Radiotherapy ◽  
Case Series ◽  
Data Series ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Modality Approach ◽  
Poor Outcomes

Background. Malignant peripheral nerve sheath tumors (MPNSTs) of the head and neck are rare aggressive neoplasms with a poor prognosis. This study describes the management and outcomes of 3 of our patients with MPNSTs of the head and neck.Methods. We identified 3 patients presenting with MPNST of the head and neck and treated at the University of North Carolina. We compared our results to the literature from 1963 to 2014.Results. Mean follow-up was 31 months. Average age at diagnosis was 44.7 years of age. All patients received wide-local excision and adjuvant radiotherapy. No patients recurred during the series. Recurrence-free survival time for the patients was 45, 37, and 3 months, respectively.Conclusions. Our data series confirms that a combined-modality approach with complete surgical resection and adjuvant radiotherapy leads to improved outcomes in MPNSTs of the head and neck. Nonetheless, due to historically poor outcomes, continued research into newer therapies needs to be explored.

MR imaging of benign peripheral nerve sheath tumors

1994 ◽  
Vol 35 (3) ◽  
pp. 282-286 ◽  
Author(s):  
Veli Soderlund ◽  
H. Goranson ◽  
H. C. F. Bauer
Keyword(s):  
Mr Imaging ◽  
Peripheral Nerve ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath
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