scholarly journals Malignant triton tumor of the left thoracic cavity: a case report

2019 ◽  
Vol 2019 (8) ◽  
Author(s):  
Masashi Ishikawa ◽  
Hiroyuki Chou ◽  
Naoto Imamura ◽  
Yumeta Shimazu ◽  
Kazuo Ono
Keyword(s):  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Large Mass ◽  
Surgical Removal ◽  
Thoracic Cavity ◽  
Left Hemithorax ◽  
Malignant Triton Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Failure Investigation

Abstract Malignant triton tumor (MTT) is a rare subtype of malignant peripheral nerve sheath tumors with rhabdomyoblastic differentiation. Although the condition may manifest sporadically, it typically affects adult patients with neurofibromatosis type 1. In this article, an extremely rare case of MTT with chest wall origin, which expanded into the left thoracic cavity, is reported. A 64-year-old male was admitted to the institution with sudden shortness of breath. Radiological examination revealed a large mass with massive pleural effusion occupying the patient’s left hemithorax. A percutaneous needle biopsy was performed and the patient underwent subtotal tumor resection with left pleuropneumonectomy. Immunohistochemical study of postsurgical pathologic specimens confirmed the diagnosis of MTT. Despite extensive surgical removal, tumor recurrence was reported soon after resection, leading to patient’s death 20 days after surgery due to acute respiratory failure. Investigation of rare MTT cases is necessary for understanding this condition.

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

2015 ◽  
Vol 39 (3) ◽  
pp. E17 ◽  
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.

scholarly journals Surgical management of malignant peripheral nerve sheath tumors

2007 ◽  
Vol 22 (6) ◽  
pp. 1-8 ◽  
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.

scholarly journals Oral Metastasis of Metaplastic Breast Carcinoma in a Patient with Neurofibromatosis 1

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Ana Paula Molina Vivas ◽  
Luana Eschholz Bomfin ◽  
Clovis Antonio Lopes Pinto ◽  
Ulisses Ribaldo Nicolau ◽  
Fabio Abreu Alves
Keyword(s):  
Breast Cancer ◽  
Breast Carcinoma ◽  
English Literature ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Increased Risk ◽  
Multiple Metastases ◽  
Metaplastic Breast Carcinoma ◽  
Oral Metastasis

Neurofibromatosis type 1 (NF1) has been associated with an increased risk for development of malignancy, especially malignant peripheral nerve sheath tumors. In addition, recently, literature has demonstrated an increased risk of breast cancer in women with NF1. The present paper shows a 53-year-old woman with NF1 who presented with metaplastic breast carcinoma and developed multiple metastases, including mandible. Furthermore, we reviewed the English literature, found 63 cases showing the association between NF1 and breast cancer, and added one more case. The present study demonstrated an important association between NF1 and breast cancer. Until the present time, there has been only one case of metaplastic breast carcinoma associated with NF1. Curiously, in our case the oral metastasis corresponded to sarcomatous component of metaplastic breast carcinoma.

scholarly journals New Model Systems and the Development of Targeted Therapies for the Treatment of Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors

Genes ◽  
2020 ◽  
Vol 11 (5) ◽  
pp. 477 ◽  
Author(s):  
Kyle B. Williams ◽  
David A. Largaespada
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Single Agent ◽  
Neurofibromatosis Type ◽  
Mek Inhibitors ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Neurofibromatosis Type 1 (NF1) is a common genetic disorder and cancer predisposition syndrome (1:3000 births) caused by mutations in the tumor suppressor gene NF1. NF1 encodes neurofibromin, a negative regulator of the Ras signaling pathway. Individuals with NF1 often develop benign tumors of the peripheral nervous system (neurofibromas), originating from the Schwann cell linage, some of which progress further to malignant peripheral nerve sheath tumors (MPNSTs). Treatment options for neurofibromas and MPNSTs are extremely limited, relying largely on surgical resection and cytotoxic chemotherapy. Identification of novel therapeutic targets in both benign neurofibromas and MPNSTs is critical for improved patient outcomes and quality of life. Recent clinical trials conducted in patients with NF1 for the treatment of symptomatic plexiform neurofibromas using inhibitors of the mitogen-activated protein kinase (MEK) have shown very promising results. However, MEK inhibitors do not work in all patients and have significant side effects. In addition, preliminary evidence suggests single agent use of MEK inhibitors for MPNST treatment will fail. Here, we describe the preclinical efforts that led to the identification of MEK inhibitors as promising therapeutics for the treatment of NF1-related neoplasia and possible reasons they lack single agent efficacy in the treatment of MPNSTs. In addition, we describe work to find targets other than MEK for treatment of MPNST. These have come from studies of RAS biochemistry, in vitro drug screening, forward genetic screens for Schwann cell tumors, and synthetic lethal screens in cells with oncogenic RAS gene mutations. Lastly, we discuss new approaches to exploit drug screening and synthetic lethality with NF1 loss of function mutations in human Schwann cells using CRISPR/Cas9 technology.

Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1

Neurology ◽  
2019 ◽  
Vol 93 (11) ◽  
pp. e1076-e1084 ◽  
Author(s):  
Shivani Ahlawat ◽  
Jaishri O. Blakeley ◽  
Fausto J. Rodriguez ◽  
Laura M. Fayad
Keyword(s):  
Fdg Pet ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Pet Ct ◽  
Adc Values ◽  
Adc Mapping ◽  
Fdg Pet Ct

ObjectiveTo determine the utility of quantitative metrics obtained from fMRI using diffusion-weighted imaging (DWI)/apparent diffusion coefficient (ADC) mapping compared with metabolic (18F-fluorodeoxyglucose [FDG]-PET/CT) imaging in patients with neurofibromatosis type 1 (NF1) for the characterization of peripheral nerve sheath tumors (PNSTs) as benign or malignant.MethodsThis Institutional Review Board–approved, Health Insurance Portability and Accountability Act–compliant study retrospectively reviewed imaging of 55 PNSTs in 21 patients with NF1. Imaging included anatomic (unenhanced T1, fluid-sensitive, contrast-enhanced T1-weighted), functional DWI (b = 50, 400, 800 s/mm2) and ADC mapping, magnetic resonance sequences, and FDG-PET/CT imaging. Anatomic (size), functional (minimum ADC values), and metabolic (maximum standardized uptake values [SUVmax]) imaging characteristics were recorded. ADC values were correlated with SUVmax. With histologic correlation for all malignant PNSTs (MPNSTs) or clinical or imaging stability (>12 months) for benign lesions used as reference standards, diagnostic accuracy was calculated.ResultsOf 55 PNSTs, there were 19 (35%) malignant and 36 (65%) benign PNSTs. Benign PNSTs were overall smaller than MPNSTs (largest diameter 4.3 ± 1.3 vs 8.2 ± 3.3 cm, respectively, p = 0.014). Benign PNSTs had higher ADCmin (×10−3 mm2/s) than MPNSTs (1.6 ± 0.4 vs 0.6 ± 0.2, respectively, p < 0.0001) and lower SUVmax than MPNSTs (3.2 ± 1.8 vs 8 ± 3.9, p < 0.0001, respectively). ADCmin correlated inversely with SUVmax (correlation coefficient r = −0.0.58, p < 0.0001). Maintaining a sensitivity of 100% with threshold values of ADCmin ≤1 or SUVmax >3.2, DWI yielded a specificity of 94% while FDG-PET/CT offered a specificity of 83%.ConclusionsBoth quantitative metabolic imaging and functional imaging offer high sensitivity for the characterization of PNSTs in NF1; however, DWI/ADC mapping offers increased specificity and may be a more useful modality.Classification of evidenceThis study provides Class II evidence that for patients with NF1, MRI using DWI/ADC mapping accurately distinguishes malignant and benign PNSTs.

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