Surgical Management of Sporadic Peripheral Nerve Schwannomas in Adults: Indications and Outcome in a Single Center Cohort

Most sporadic peripheral nerve sheath tumors in adults are schwannomas. These tumors usually present with significant pain but can also cause neurological deficits. Symptomatology is diverse, and successful surgical interventions demand interdisciplinarity. We retrospectively reviewed 414 patients treated between 2006 and 2017 for peripheral nerve sheath tumors. We analyzed clinical signs, symptoms, histology, and neurological function in the cohort of adult patients with schwannomas without a neurocutaneous syndrome. In 144 patients, 147 surgical interventions were performed. Mean follow-up was 3.1 years. The indication for surgery was pain (66.0%), neurological deficits (23.8%), significant tumor growth (8.8%), and suspected malignancy (1.4%). Complete tumor resection was achieved on 136/147 occasions (92.5%). The most common location of the tumors was intraspinal (49.0%), within the cervical neurovascular bundles (19.7%), and lower extremities (10.9%). Pain and neurological deficits improved significantly (p ≤ 0.003) after 131/147 interventions (89.1%). One patient had a persistent decrease in motor function after surgery. Complete resection was possible in 67% of recurrent tumors, compared to 94% of primary tumors. There was a significantly lower chance of complete resection for schwannomas of the cervical neurovascular bundle as compared to other locations. The surgical outcome of sporadic schwannoma surgery within the peripheral nervous system is very favorable in experienced peripheral nerve surgery centers. Surgery is safe and effective and needs a multidisciplinary setting. Early surgical resection in adult patients with peripheral nerve sheath tumors with significant growth, pain, neurological deficit, or suspected malignancy is thus recommended.

Download Full-text

Clinical Signs of Neurofibromatosis Impact on the Outcome of Malignant Peripheral Nerve Sheath Tumors

Oncology ◽

10.1159/000357137 ◽

2014 ◽

Vol 86 (2) ◽

pp. 122-126 ◽

Cited By ~ 3

Author(s):

W. Lamm ◽

S. Schur ◽

W.J. Köstler ◽

P. Funovics ◽

R. Windhager ◽

...

Keyword(s):

Peripheral Nerve ◽

Clinical Signs ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Download Full-text

Primary pediatric intraspinal sarcomas

Journal of Neurosurgery Pediatrics ◽

10.3171/2009.3.peds08272 ◽

2009 ◽

Vol 4 (3) ◽

pp. 222-229 ◽

Cited By ~ 17

Author(s):

Paul Klimo ◽

Patrick J. Codd ◽

Holcombe Grier ◽

Liliana C. Goumnerova

Keyword(s):

Peripheral Nerve ◽

Ewing Sarcoma ◽

Pediatric Population ◽

Spinal Nerve ◽

Neurological Deficits ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Lumbar Spinal ◽

Extraosseous Ewing Sarcoma

Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery. Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.

Download Full-text

A retrospective evaluation of 51 cases of peripheral nerve sheath tumors in the dog

Journal of the American Animal Hospital Association ◽

10.5326/15473317-31-4-349 ◽

1995 ◽

Vol 31 (4) ◽

pp. 349-359 ◽

Cited By ~ 86

Author(s):

DM Brehm ◽

CH Vite ◽

HS Steinberg ◽

J Haviland ◽

T van Winkle

Keyword(s):

Peripheral Nerve ◽

Clinical Signs ◽

Diagnostic Procedures ◽

Lumbosacral Plexus ◽

Survival Times ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Significant Difference ◽

Root Group

Fifty-one cases of canine peripheral nerve sheath tumors were reviewed. Signalment, presenting clinical signs, duration of clinical signs, physical and neurological examination findings, results of diagnostic procedures, type of surgery performed, tumor location, relapse-free intervals and survival times, and causes of death were evaluated. Tumors were divided into three anatomical groups: tumors involving nerves distal to the brachial or lumbosacral plexus (Peripheral Group), tumors involving nerves of the brachial or lumbosacral plexus (Plexus Group), and tumors involving the vertebral canal (Root Group). The most common clinical findings were unilateral forelimb lameness and muscle atrophy. The most useful diagnostic tests were myelography and electromyography. Although there was no significant difference, dogs in the Root Group tended to have shorter relapse-free intervals and survival times than dogs in the Plexus Group. The overall prognosis for surgical management of peripheral nerve sheath tumors is guarded to poor.

Download Full-text

Malignant Peripheral Nerve Sheath Tumors of the Bilateral Adrenal Glands: Are They Metachronous Primary Tumors - A Case Report -

The Korean Journal of Pathology ◽

10.4132/koreanjpathol.2009.43.5.471 ◽

2009 ◽

Vol 43 (5) ◽

pp. 471

Author(s):

Jae Hong Park ◽

Seung Yeon Ha ◽

Hyun Yee Cho

Keyword(s):

Case Report ◽

Peripheral Nerve ◽

Adrenal Glands ◽

Primary Tumors ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Download Full-text

Palliative Epineurotomy for Focal Radial Malignant Peripheral Nerve Sheath Tumor in a Dog

Journal of the American Animal Hospital Association ◽

10.5326/jaaha-ms-6336 ◽

2016 ◽

Vol 52 (5) ◽

pp. 330-334 ◽

Cited By ~ 2

Author(s):

Andrew David Gibson ◽

Emma Davies ◽

Ana Lara-Garcia ◽

Pilar Lafuente

Keyword(s):

Peripheral Nerve ◽

Radial Nerve ◽

Clinical Signs ◽

Histopathological Analysis ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Deep Branch ◽

Nerve Sheath

ABSTRACT This case report describes the diagnosis of a peripheral nerve sheath tumor of the deep branch of the radial nerve distal to the elbow in a dog. The lesion was identified using computed tomography and ultrasonography and confirmed as sarcoma on histopathological analysis of incisional biopsies. Clinical signs dramatically improved following surgical biopsy before recurring three months later. Repeat epineurotomy of the deep branch of the radial nerve resulted in clinical improvement for a further month before signs once again returned. Epineurotomy as a palliative treatment for peripheral nerve sheath tumors has not been previously described, but may have a place in palliation of clinical signs in specific cases of peripheral nerve sheath tumors in which limb amputation is not an option.

Download Full-text

Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management

Radiology and Oncology ◽

10.2478/raon-2013-0047 ◽

2013 ◽

Vol 47 (3) ◽

pp. 230-238 ◽

Cited By ~ 13

Author(s):

Sophia Constance Kamran ◽

Atul Bhanudas Shinagare ◽

Stephanie Anne Holler Howard ◽

Mizuki Nishino ◽

Jason Laurence Hornick ◽

...

Keyword(s):

Peripheral Nerve ◽

Chest Wall ◽

Standardized Uptake Value ◽

Imaging Features ◽

Primary Tumors ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Imaging Characteristics ◽

Adjacent Structures

Abstract Background. The aim of the study was to analyze the clinical and imaging characteristics of primary intrathoracic malignant peripheral nerve sheath tumors (MPNSTs). Patients and methods. In this institutional review board (IRB)-approved retrospective study, clinical and imaging features of 15 patients (eight men; mean age 50 years [range 18-83)] with pathologically proven malignant peripheral nerve sheath tumors seen from January 1999 to December 2011 were analyzed. Imaging features (CT in 15, MRI in 5 and PET/CT in 4) of primary tumors were evaluated by three radiologists and correlated with clinical management. Results. Of the 15 tumors, six were located in the mediastinum (two each in anterior, middle and posterior mediastinum), four in chest wall, two were paraspinal, and three in the lung. Four patients had neurofibromatosis-1 (NF1); four tumors had heterologous rhabdomyoblastic differentiation (malignant triton tumor). Masses typically were elongated along the direction of nerves, with mean size of 11 cm. The masses were hypo- or isodense to muscles on CT, isointense on T1-weighted images, hyperintense on T2-weighted images and intensely fluorodeoxyglucose (FDG) avid (mean standardized uptake value [SUV]max of 10.5 [range 4.4-23.6]). Necrosis and calcification was seen in four tumors each. Finding of invasion of adjacent structures on imaging led to change in management in seven patients; patients with invasion received chemoradiation. Conclusions. Intrathoracic MPNSTs appear as large elongated masses involving mediastinum, lung or chest wall. Radiological identification of invasion of adjacent structures is crucial and alters therapy, with patients with invasion receiving neoadjuvant or adjuvant chemoradiation.

Download Full-text