Malignant peripheral nerve sheath tumour (MPNST) is a very rare tumour with an incidence of one per 100,000 and constitutes between 3 to 10% of all soft tissue sarcomas. Most of the sarcoma involve the extremities and retroperitoneal regions. However, this case presented with mass in left inguinal region and then spread rapidly to omentum, assuming the appearance of an omental cake. Mass respon...

We report a patient with multiple endocrine neoplasia type 1 (MEN-1) and an aggressive malignant peripheral nerve sheath tumor (MPNST) arising from a ganglioneuroma of the adrenal gland. Patients with MEN-1 require careful consideration of other tumor associations, including MPNST, as it can portend a poor prognosis. MEN-1 and MPNST have not been reported. We report a patient with multiple endo...

Patients with NF1 microdeletion develop more neurofibromas at a younger age, and have an increased risk of malignant peripheral nerve sheath tumors (MPNSTs). We postulated that the increased risk of malignancy could be due to inactivation, in addition to NF1, of a second tumor suppressor gene located in the typical 1.4-Mb microdeletion found in most of the microdeleted patients. We investigated...

A 63-year-old African American female with history of bilateral breast cancer status after lumpectomy and radiation therapy presented with right hand, wrist, and arm pain. She was found to have a right axillary mass and a large lesion in the right brachial plexus. A biopsy of the brachial plexus mass came back as a malignant peripheral nerve sheath tumor. This case report illustrates the critic...

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,...

Malignant peripheral nerve sheath tumors originating from the parotid gland are extremely rare. A 76-year-old male patient underwent an incisional biopsy for an ulcerated mass in the anteroinferior aspect of the left auricle. The diagnosis was made as malignant mesenchymal tissue sarcoma, but the patient refused treatment. Upon progressive growth of the mass within two months, he underwent a pa...

Aneuploidy is a hallmark of human cancers, but most mouse cancer models lack the extensive aneuploidy seen in many human tumors. The zebrafish is becoming an increasingly popular model for studying cancer. Here we report that malignant peripheral nerve sheath tumors (MPNSTs) that arise in zebrafish as a result of mutations in either ribosomal protein (rp) genes or in p53 are highly aneuploid. K...

Malignant peripheral nerve sheath tumors (MPNST) are incurable tumors of the Schwann cell lineage that progress unpredictably from benign plexiform neurofibromas. In this issue of Cancer Discovery, Watson and colleagues use an insertional mutagenesis screen combined with network analysis to identify the canonical WNT signaling pathway as an important potential biomarker of tumor progression and...

Objective To present a case of successful repair of a spontaneous cerebrospinal fluid leak in a previously unreported anatomic site. Methods Retrospective chart review. Results A 48-year-old woman developed a spontaneous cerebrospinal fluid leak from the optic nerve sheath and underwent a multilayer endoscopic closure with no damage to the optic nerve. Conclusion Endoscopic surgeons can s...

The optic nerves are well demonstrated by high-resolution computed tomography. Involvement of the optic nerve by optic gliomas and optic nerve sheath meningiomas is well known. However, nonneoplastic processes such as increased intracranial pressure, optic neuritis, Grave ophthalmopathy, and orbital pseudotumor may also alter the appearance of the optic nerve/sheath on computed tomography. Cert...