Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis

✓ 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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Diffusion tensor tractography for the surgical management of peripheral nerve sheath tumors

Neurosurgical FOCUS ◽

10.3171/2015.6.focus15228 ◽

2015 ◽

Vol 39 (3) ◽

pp. E17 ◽

Cited By ~ 14

Author(s):

Manfred Schmidt ◽

Gregor Kasprian ◽

Gabriele Amann ◽

Dominik Duscher ◽

Oskar C. Aszmann

Keyword(s):

Peripheral Nerve ◽

Diffusion Tensor ◽

Tumor Resection ◽

Significant Risk ◽

Diffusion Tensor Tractography ◽

Surgical Removal ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Mri Scans

OBJECT Peripheral nerve sheath tumors (PNSTs) are uncommon but bear a significant risk of malignancy. High-resolution MRI is the standard technique for characterizing PNSTs. However, planning the appropriate extent of resection and subsequent reconstructive strategies is highly dependent on the intraoperative findings because preoperative MRI evaluation can be insufficient. Diffusion tensor tractography (DTT) represents a recently developed advanced MRI technique that reveals the microstructure of tissues based on monitoring the random movement of water molecules. DTT has the potential to provide diagnostic insights beyond conventional MRI techniques due to its mapping of specific fibrillar nerve structures. Here, DTT was applied to evaluate PNSTs and to examine the usefulness of this method for the correct delineation of tumor and healthy nerve tissue and the value of this information in the preoperative planning of surgical interventions. METHODS In this prospective study, patients with the clinical symptoms of a PNST were investigated using DTT 3-Tesla MRI scans. Image data processing and tractography were performed using the FACT (fiber assessment by continuous tracking) algorithm and multiple-regions-of-interest approach. The surgical findings were then compared with the results of the DTT MRI scans. Preoperative fascicle visualization and the correlation with the intraoperative findings were graded. RESULTS In a 21-month period, 12 patients with PNSTs were investigated (7 female and 5 male patients with a mean age of 46.2 ± 19.2 years). All patients underwent surgical removal of the tumor. Schwannoma was the most common benign histopathological finding (n = 7), whereas 2 malignant lesions were detected. In 10 of 12 patients, good preoperative nerve fascicle visualization was achieved using DTT scans. In 9 of 10 patients with good preoperative fascicle visualization, good intraoperative correlation between the DTT scans and surgical anatomy was found. CONCLUSIONS DTT properly visualizes the peripheral nerve fascicles and their correct anatomical relation to PNST. DTT represents a promising new method for the preinterventional planning of nerve tumor resection.

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The Lipid Asset Is Unbalanced in Peripheral Nerve Sheath Tumors

International Journal of Molecular Sciences ◽

10.3390/ijms23010061 ◽

2021 ◽

Vol 23 (1) ◽

pp. 61

Author(s):

Ignazio G. Vetrano ◽

Michele Dei Cas ◽

Vittoria Nazzi ◽

Marica Eoli ◽

Niccolò Innocenti ◽

...

Keyword(s):

Peripheral Nerve ◽

Biological Behavior ◽

Malignant Degeneration ◽

Benign Tumors ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Molecular Features ◽

Nerve Sheath ◽

Inflammatory Processes ◽

Oxidized Triacylglycerols

Peripheral nerve sheath tumors (PNSTs) include schwannomas, neurofibromas (NFs), and plexiform neurofibromas (PNFs), among others. While they are benign tumors, according to their biological behavior, some have the potential for malignant degeneration, mainly PNFs. The specific factors contributing to the more aggressive behavior of some PNSTs compared to others are not precisely known. Considering that lipid homeostasis plays a crucial role in fibrotic/inflammatory processes and in several cancers, we hypothesized that the lipid asset was also unbalanced in this group of nerve tumors. Through untargeted lipidomics, NFs presented a significant increase in ceramide, phosphatidylcholine, and Vitamin A ester. PNFs displayed a marked decrease in 34 out of 50 lipid class analyzed. An increased level of ether- and oxidized-triacylglycerols was observed; phosphatidylcholines were reduced. After sphingolipidomic analysis, we observed six sphingolipid classes. Ceramide and dihydroceramides were statistically increased in NFs. All the glycosylated species appeared reduced in NFs, but increased in PNFs. Our findings suggested that different subtypes of PNSTs presented a specific modulation in the lipidic profile. The untargeted and targeted lipidomic approaches, which were not applied until now, contribute to better clarifying bioactive lipid roles in PNS natural history to highlight disease molecular features and pathogenesis.

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Surgical management of malignant peripheral nerve sheath tumors

Neurosurgical FOCUS ◽

10.3171/foc.2007.22.6.14 ◽

2007 ◽

Vol 22 (6) ◽

pp. 1-8 ◽

Cited By ~ 20

Author(s):

John Gachiani ◽

Daniel Kim ◽

Adriane Nelson ◽

David Kline

Keyword(s):

Soft Tissue ◽

Peripheral Nerve ◽

Surgical Techniques ◽

Soft Tissue Sarcomas ◽

Neurofibromatosis Type ◽

Surgical Removal ◽

Complete Excision ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Object The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment. Methods The authors retrospectively analyzed data on 43 surgically treated soft tissue sarcomas involving the nerves, 34 (79%) of which were MPNSTs. Tumor classifications are presented, together with patient numbers, locations of MPNSTs, surgical techniques, and adjunctive treatments. Results The 34 MPNSTs were surgically treated during a period of 40 years. Most of these lesions (19 MPNSTs [56%]) were located in the brachial plexus, whereas the rest were located on other major nerves. Neurofibromatosis Type 1–associated tumors (12 lesions) represented 35% of the total number of MPNSTs. Although the main goal of surgery was complete excision, it was successful in only 16 patients (47%). Of the available records, 18 patients (53%) died due to disease progression. Conclusions Malignant peripheral nerve sheath tumors are rare. Surgical removal with margins when possible coupled by adjuvant radiotherapy offers the best chance of survival. The role of chemotherapy is still being defined.

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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.

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