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

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.

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

Cancers ◽

10.3390/cancers13051017 ◽

2021 ◽

Vol 13 (5) ◽

pp. 1017

Author(s):

Julian Zipfel ◽

Meizer Al-Hariri ◽

Isabel Gugel ◽

Alexander Grimm ◽

Volker Steger ◽

...

Keyword(s):

Peripheral Nerve ◽

Clinical Signs ◽

Complete Resection ◽

Adult Patients ◽

Neurological Deficits ◽

Primary Tumors ◽

Surgical Interventions ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

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.

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Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis

Journal of Neurosurgery ◽

10.3171/jns.2000.92.2.0342 ◽

2000 ◽

Vol 92 (2) ◽

pp. 342-346 ◽

Cited By ~ 32

Author(s):

Jacob Schwarz ◽

Allan J. Belzberg

Keyword(s):

Peripheral Nerve ◽

Neurological Impairment ◽

Malignant Degeneration ◽

Surgical Removal ◽

Surgical Indications ◽

Content Type ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Adjacent Structures

✓ Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. Patients with NF5 present with café-au-lait macules, freckles, and/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain, neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.

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The Risk of Peripheral Nerve Tumor Biopsy in Suspected Benign Etiologies

Neurosurgery ◽

10.1093/neuros/nyz549 ◽

2020 ◽

Vol 86 (3) ◽

pp. E326-E332 ◽

Cited By ~ 5

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.

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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

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MAGNETIC RESONANCE IMAGING CHARACTERISTICS OF PERIPHERAL NERVE SHEATH TUMORS OF THE CANINE BRACHIAL PLEXUS IN 18 DOGS

Veterinary Radiology & Ultrasound ◽

10.1111/j.1740-8261.2007.00195.x ◽

2007 ◽

Vol 48 (1) ◽

pp. 1-7 ◽

Cited By ~ 37

Author(s):

SUSAN KRAFT ◽

E. J. EHRHART ◽

DAVID GALL ◽

LISA KLOPP ◽

PATRICK GAVIN ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Peripheral Nerve ◽

Brachial Plexus ◽

Resonance Imaging ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Imaging Characteristics

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Malignant peripheral nerve sheath tumors presenting as spinal dumbbell tumors: clinical outcomes and characteristic imaging features

European Spine Journal ◽

10.1007/s00586-014-3467-8 ◽

2014 ◽

Vol 24 (10) ◽

pp. 2119-2125 ◽

Cited By ~ 6

Author(s):

Yoshihiro Matsumoto ◽

Makoto Endo ◽

Katsumi Harimaya ◽

Mitsumasa Hayashida ◽

Toshio Doi ◽

...

Keyword(s):

Peripheral Nerve ◽

Clinical Outcomes ◽

Imaging Features ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Characteristic Imaging

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

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2017.182 ◽

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.

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