Multinodular/plexiform (multifascicular) schwannomas of major peripheral nerves: an underrecognized part of the spectrum of schwannomas

2010 ◽  
Vol 112 (2) ◽  
pp. 372-382 ◽  
Author(s):  
Marie-Noëlle Hébert-Blouin ◽  
Kimberly K. Amrami ◽  
Bernd W. Scheithauer ◽  
Robert J. Spinner
Keyword(s):  
Peripheral Nerves ◽  
Clinical Presentation ◽  
Imaging Features ◽  
Surgical Results ◽  
Nerve Sheath Tumors ◽  
The Past ◽  
Nerve Sheath ◽  
Imaging Characteristics ◽  
Complex Forms ◽  
Distinguishing Features

Object In clinical practice, schwannomas are among the most common types of nerve sheath tumors. Their clinical presentation, imaging characteristics, and operative features are well known. Over the past 20 years, clinical outcomes have improved due to resection of these tumors at a fascicular level. Despite these advances, a subgroup of patients with schwannomas is associated with a disappointing neurological outcome following resection. The purpose of this study was to correlate the imaging and histological features in this group of patients with more anatomically complex forms of schwannomas. Methods In a retrospective review performed at their institution over a 10-year period, the authors found a subgroup of patients with complex multinodular/plexiform schwannomas affecting major peripheral nerves. Eleven patients were identified, and the clinical, imaging, and pathological features of their disease were reviewed. Results The clinical presentation of multinodular/plexiform schwannomas of major peripheral nerves may be similar to that of conventional schwannomas, but their imaging features, operative appearance, and outcomes differ. Conclusions Preoperatively and intraoperatively, the distinguishing features of multinodular/plexiform schwannomas of major peripheral nerves may be subtle and can easily go unrecognized, thus explaining the often suboptimal surgical results. Familiarity with the imaging and operative features of multinodular/plexiform schwannomas will no doubt alter treatment approaches and improve neurological function in this subgroup of patients.

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

2013 ◽  
Vol 47 (3) ◽  
pp. 230-238 ◽  
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.

Malignant Nerve Sheath Tumor of the Right Cerebral Peduncle: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 54 (2) ◽  
pp. 500-504 ◽  
Author(s):  
Patrick Beauchesne ◽  
Jean-François Mosnier ◽  
Thierry Schmitt ◽  
Jacques Brunon
Keyword(s):  
Stereotactic Biopsy ◽  
Clinical Presentation ◽  
Positive Response ◽  
Cerebral Peduncle ◽  
Nerve Sheath Tumor ◽  
Chemotherapy Treatment ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Right ◽  
First Time

Abstract OBJECTIVE AND IMPORTANCE Schwannomas occurring in the neuraxis are very rare. Usually, these tumors are benign. Primary malignant intracerebral nerve sheath tumors are extremely rare, with only five documented cases in the international literature. We report one case of a primary malignant intracerebral nerve sheath tumor occurring in the right cerebral peduncle of a 35-year-old man. CLINICAL PRESENTATION Magnetic resonance imaging revealed a heterogeneous peripherally enhancing mass of the right cerebral peduncle, surrounded by a small edema. INTERVENTION Unlike the five cases previously reported, this is the first time a stereotactic biopsy has been performed, and this is the only patient who responded to cranial radiation therapy for approximately 2 years. When the tumor recurred, a systemic chemotherapy treatment was prescribed. No positive response was seen, and the patient died 29 months after the initial diagnosis. CONCLUSION An accurate diagnosis and planned aggressive treatment seem to be the key elements in the management of the disease.

Dumbbell Nerve Sheath Tumors

2018 ◽  
Author(s):  
Hussam Abou-Al-Shaar ◽  
Mark A. Mahan
Keyword(s):  
Imaging Modality ◽  
Spinal Nerve ◽  
Surgical Approaches ◽  
Nerve Sheath Tumor ◽  
Imaging Features ◽  
Neural Foramen ◽  
Dumbbell Tumor ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Individual

A dumbbell tumor is a nerve sheath tumor that arises from a spinal nerve in the neural foramen and grows as a dumbbell-shaped mass. The differential diagnosis for a dumbbell tumor includes schwannoma, neurofibroma, malignant peripheral nerve sheath tumor, and metastases, among others. MR imaging is considered the gold-standard imaging modality for diagnosis of dumbbell tumors. Surgical approaches that are tailored to the individual patient’s case can be utilized. The chapter reviews dumbbell tumors, including a case example and covers the incidence, clinical presentation, imaging features, decision-making strategy, surgical approaches, outcomes, and potential complications associated with their management.

Analysis of Clinical Features, Pathological Features and Misdiagnosis of Pulmonary Lymphoma

2021 ◽  
Vol 11 (7) ◽  
pp. 1877-1886
Author(s):  
Guobin Zhang ◽  
Shuang Song ◽  
Yue Yang ◽  
Qin Huang
Keyword(s):  
Clinicopathological Characteristics ◽  
Imaging Features ◽  
Imaging Findings ◽  
Pathological Features ◽  
Imaging Diagnosis ◽  
The Past ◽  
Primary Pulmonary Lymphoma ◽  
Imaging Characteristics ◽  
Pulmonary Lymphoma ◽  
Leaf Distribution

Primary pulmonary lymphoma is a relatively rare extranodal lymphoma, and the incidence rate has increased in recent years. In the past, the disease mainly relied on surgery to obtain the pathological basis, so the clinical misdiagnosis rate was high. How to improve its early diagnosis and treatment has attracted much attention. By exploring the imaging manifestations of primary pulmonary lymphoma, we can further understand and improve the imaging diagnosis level of primary pulmonary lymphoma. This paper discusses the classification, imaging manifestations, diagnosis and identification of pulmonary lymphoma. The clinical data and imaging findings of primary pulmonary lymphoma diagnosed in a hospital were retrospectively summarized, and their imaging features were analyzed. We observe the clinicopathological characteristics and immunohistochemical phenotypes of multiple masses with cavitation type primary lung lymphoma, and analyze the virus and imaging characteristics of hybridization. The results of the study show that the CT (Computed Tomography) manifestations of primary lung lymphoma are diverse. The characteristics of cross-leaf distribution are more characteristic, and enhanced scanning lesions are usually mild to moderate. In the case of simultaneous masses and pneumonialike consolidation in the lungs, this disease needs to be considered.

Pictorial Essay: Imaging of Peripheral Nerve Sheath Tumours

2011 ◽  
Vol 62 (3) ◽  
pp. 176-182 ◽  
Author(s):  
Daniel W.Y. Chee ◽  
Wilfred C.G. Peh ◽  
Tony W.H. Shek
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Peripheral Nerve ◽  
Imaging Features ◽  
Resonance Imaging ◽  
Target Sign ◽  
Nerve Sheath ◽  
Pictorial Essay ◽  
Characteristic Imaging ◽  
Distinguishing Features

Peripheral nerve sheath tumours (PNST) may be benign or malignant. Benign PNSTs include neurofibroma and schwannoma. Neurogenic tumours share certain characteristic imaging features, suggested by a fusiform-shaped mass with tapered ends, the “split-fat” sign, atrophy of the muscles supplied by the involved nerve, the “fascicular sign,” and the “target sign”; these imaging features are best demonstrated on magnetic resonance imaging. This pictorial essay emphasizes the characteristic signs and distinguishing features of PNSTs on imaging.

The Risk of Peripheral Nerve Tumor Biopsy in Suspected Benign Etiologies

Neurosurgery ◽  
2020 ◽  
Vol 86 (3) ◽  
pp. E326-E332 ◽  
Author(s):  
Roberto J Perez-Roman ◽  
S Shelby Burks ◽  
Luca Debs ◽  
Iahn Cajigas ◽  
Allan D Levi
Keyword(s):  
Peripheral Nerve ◽  
Neurological Deficit ◽  
High Rate ◽  
Tumor Type ◽  
Imaging Features ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Preoperative Biopsy ◽  
Postoperative Deficit

Abstract BACKGROUND Peripheral nerve sheath tumors (PNSTs) are tumors with unique clinical and imaging features that present to a variety of physicians. These lesions are often referred for biopsy, which can put nerve fascicles at risk. Preoperative biopsy may cause distortion of normal anatomic planes, making definitive resection difficult. OBJECTIVE To evaluate the neurological risks of preoperative biopsy in benign PNSTs. METHODS Surgical cases collected retrospectively using a prospectively established database of PNSTs treated by a single surgeon between 1997 and 2019. Patients were dichotomized depending on preoperative biopsy. The effects of biopsy were assessed via history and physical examination both pre- and postdefinitive resection. RESULTS A total of 151 cases were included. Only 23.2% (35) of patients underwent preoperative biopsy, but 42.9% of these experienced new or worsening neurological examination immediately following biopsy. After definitive resection, the rate of neurological deficit was significantly different between the 2 groups with 60% of biopsy patients and 19% of those patients not biopsied experiencing decline in examination (F = 25.72, P < .001). Odds ratio for any postoperative deficit for biopsy was 6.40 (CI [2.8, 14.55], P < .001). Univariate logistic regression of neurological deficit with patient age, sex, tumor type, and biopsy status showed that only biopsy was associated with the occurrence of any postoperative deficit. CONCLUSION Biopsy of benign PNSTs is associated with a high rate of neurological deficit both immediately following the procedure and after definitive resection. Careful selection is imperative prior to proceeding with biopsy of nerve sheath tumors exhibiting benign features given the unacceptably high rate of neurological decline.

Intracranial ependymomas: The role of advanced neuroimaging in diagnosis and management

2021 ◽  
Vol 34 (2) ◽  
pp. 80-92
Author(s):  
Marco Varrassi ◽  
Flavia Cobianchi Bellisari ◽  
Maria Carmela De Donato ◽  
Emanuele Tommasino ◽  
Alessandra Di Sibio ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Scientific Literature ◽  
Illustrative Case ◽  
Imaging Features ◽  
Resonance Imaging ◽  
Anaplastic Ependymoma ◽  
Rare Tumour ◽  
Imaging Characteristics ◽  
Recent Classification

Intracranial ependymomas represent a rare subgroup of glial tumours, showing a wide variety of imaging characteristics, often representing a challenging diagnosis for neuroradiologists. Here, we review the most recent scientific Literature on intracranial ependymomas, highlighting the most characteristic computed tomography and magnetic resonance imaging features of these neoplasms, along with epidemiologic data, recent classification aspects, clinical presentation and conventional therapeutic strategies. In addition, we report an illustrative case of an 18-year-old girl presenting with an intracranial supratentorial, anaplastic ependymoma, with the aim of contributing to the existing knowledge and comprehension of this rare tumour.

scholarly journals Targeted Therapies for the Neurofibromatoses

Cancers ◽  
2021 ◽  
Vol 13 (23) ◽  
pp. 6032
Author(s):  
Lauren D. Sanchez ◽  
Ashley Bui ◽  
Laura J. Klesse
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
The Past ◽  
Therapeutic Trials ◽  
Nerve Sheath

Over the past several years, management of the tumors associated with the neurofibromatoses has been recognized to often require approaches that are distinct from their spontaneous counterparts. Focus has shifted to therapy aimed at minimizing symptoms given the risks of persistent, multiple tumors and new tumor growth. In this review, we will highlight the translation of preclinical data to therapeutic trials for patients with neurofibromatosis, particularly neurofibromatosis type 1 and neurofibromatosis type 2. Successful inhibition of MEK for patients with neurofibromatosis type 1 and progressive optic pathway gliomas or plexiform neurofibromas has been a significant advancement in patient care. Similar success for the malignant NF1 tumors, such as high-grade gliomas and malignant peripheral nerve sheath tumors, has not yet been achieved; nor has significant progress been made for patients with either neurofibromatosis type 2 or schwannomatosis, although efforts are ongoing.

Rare Primary Embryonal Carcinoma in the Brachial Plexus: A Case Report and Literature Review

Neurosurgery ◽  
2019 ◽  
Vol 87 (2) ◽  
pp. E152-E155 ◽  
Author(s):  
Huihao Chen ◽  
Gang Yin ◽  
Na Cui ◽  
Haodong Lin
Keyword(s):  
Brachial Plexus ◽  
Embryonal Carcinoma ◽  
Clinical Presentation ◽  
Middle Part ◽  
Resonance Imaging ◽  
Primary Tumors ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of

Abstract BACKGROUND AND IMPORTANCE Primary tumors of the brachial plexus are rare. Most are benign and characterized as Schwannoma and neurofibroma, whereas malignant peripheral nerve sheath tumors are less common. Here, we report a rare case of primary embryonal carcinoma in the brachial plexus. CLINICAL PRESENTATION A 17-yr-old male presented with a 3-mo history of a mass growing in the left supraclavicular region over the middle part of the clavicle. Magnetic resonance imaging revealed a well-defined mass (diameter 2.5 cm) straddling the brachial plexus. After surgical resection, and the mass was histologically confirmed to be an embryonal carcinoma. CONCLUSION Primary embryonal carcinoma in the brachial plexus has not been reported previously. This case highlights the importance of considering the possibility that some primary brachial plexus tumors may be malignant and should be treated promptly.

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