scholarly journals Diffuse Neurofibroma in a Patient with Unknown History of NF1

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Mahmoud A. K. Ebrahim ◽  
Mishal M. AlMutairi ◽  
Khaled M. Hindi ◽  
Jassem M. Bastaki
Keyword(s):  
Parotid Gland ◽  
Previous History ◽  
Neurofibromatosis Type ◽  
Needle Aspiration ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
History Of ◽  
Laboratory Investigations ◽  
Magnetic Resonance Imaging Mri

Malignant peripheral nerve sheath tumor (MPNST) is a rare disease in the parotid gland with a poor prognosis in most cases. The disease most often develops in the setting of neurofibromatosis type 1 (NF1) but can also occur sporadically. Herein, we report a rare case of MPNST in the parotid gland, in a patient with no previous history of NF1. Initial investigations of the patient, which consisted of laboratory investigations, ultrasound imaging of the swelling, fine-needle aspiration (FNA), computed tomography (CT) scan, and magnetic resonance imaging (MRI) of the neck and swelling, were compatible with a benign pleomorphic adenoma of the parotid gland. However, intraoperatively, the dissection was challenging as the tumor was adherent to the neighboring tissue. A diagnosis of MPNST arising from a diffuse neurofibroma was established based on clinicopathologic features of the disease. The patient, who exhibited clinical features compatible with (NF1), proceeded for radiotherapy following surgery to continue his treatment.

Malignant Peripheral Nerve Sheath Tumor Arising from a Solitary Neurofibroma in a Patient without Neurofibromatosis Type 1

2016 ◽  
Vol 78 (5) ◽  
pp. 516-521
Author(s):  
Fumihisa SAWADA ◽  
Eiichi MAKINO ◽  
Takenobu YAMAMOTO ◽  
Ryo TANAKA ◽  
Yutaka FUJIWARA ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Solitary Neurofibroma

scholarly journals Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

2017 ◽  
Vol 75 (6) ◽  
pp. 366-371 ◽  
Author(s):  
Roberto André Torres de Vasconcelos ◽  
Pedro Guimarães Coscarelli ◽  
Regina Papais Alvarenga ◽  
Marcus André Acioly
Keyword(s):  
Overall Survival ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Tumor Size ◽  
Retrospective Chart Review ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population.

Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

2006 ◽  
Vol 47 (5) ◽  
pp. 636-638 ◽  
Author(s):  
Samar A. Muwakkit ◽  
Carlos Rodriguez-Galindo ◽  
Ahmad I. EL Samra ◽  
Ruby Khoury ◽  
Samir R. Akel ◽  
...  
Keyword(s):  
Young Child ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

Intracerebral malignant peripheral nerve sheath tumor in a child with neurofibromatosis Type 1 and middle cerebral artery aneurysm treated with endovascular coil embolization

2011 ◽  
Vol 8 (4) ◽  
pp. 346-352 ◽  
Author(s):  
Michael J. Ellis ◽  
Samuel Cheshier ◽  
Sunjay Sharma ◽  
Derek Armstrong ◽  
Cynthia Hawkins ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Middle Cerebral Artery ◽  
Cerebral Artery ◽  
Neurofibromatosis Type 1 ◽  
Coil Embolization ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Endovascular Coil Embolization

Among the neoplastic conditions that affect patients with neurofibromatosis Type 1 (NF1) are malignant peripheral nerve sheath tumors (MPNSTs), which typically arise from peripheral nerves of the limbs, trunk, and lumbar and brachial plexuses. Ionizing radiation is an established risk factor for MPNST development, especially in susceptible patients such as those with NF1. Patients with NF1 are also at risk for intracranial aneurysms, which are increasingly being successfully managed with endovascular therapies. The authors describe the case of a 9-year-old, previously healthy girl who presented in extremis with a right frontal intracerebral hemorrhage resulting from a ruptured right middle cerebral artery (MCA) trifurcation aneurysm. Following urgent decompressive craniectomy, the patient underwent endovascular coil embolization of the MCA aneurysm without complication. Given her mother's history of NF1, the child underwent genetic testing, which disclosed signs positive for NF1. The patient recovered well, but follow-up MR imaging and MR angiography performed at 14 months demonstrated a large frontotemporal mass encasing the right MCA trifurcation. The patient underwent frontotemporal craniotomy and subtotal resection of the mass, which was histologically found to be an intracranial MPNST. The patient received chemotherapy and focal radiation therapy and remains alive at 6 months postresection. To the authors' knowledge, this represents the only known case of intracranial neoplasm arising in the region of an intracranial aneurysm repaired by endovascular coil embolization. While patients with NF1 represent a population with genetic susceptibility to radiation-induced tumors, the pathogenesis of intracerebral MPNSTs remains poorly understood.

scholarly journals Special Issue: “Genomics and Models of Nerve Sheath Tumors”

Genes ◽  
2020 ◽  
Vol 11 (9) ◽  
pp. 1024
Author(s):  
Angela C. Hirbe ◽  
Rebecca D. Dodd ◽  
Christine A. Pratilas
Keyword(s):  
Soft Tissue ◽  
Neurofibromatosis Type ◽  
Benign Tumors ◽  
Nerve Sheath Tumor ◽  
Special Issue ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Biological Potential

Nerve sheath tumors arising in the context of neurofibromatosis type 1 (NF1) include benign tumors such as cutaneous, diffuse and plexiform neurofibromas; atypical neurofibromas or atypical neurofibromatosis neoplasms of uncertain biological potential (ANNUBP); and the aggressive soft tissue sarcoma, the malignant peripheral nerve sheath tumor (MPNST) [...]

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