The differential diagnosis for lesions causing neural foraminal widening is vast. The majority are solitary benign peripheral nerve sheath tumours, such as neurofibromas or schwannomas. We present a case of a rare cause of neural foraminal expansion secondary to a posterior thoracic extradural angiolipoma. We describe the presence of chemical shift artefact on post gadolinium T1-weighted imagin...

Malignant degeneration of neurogenic tumors has been reported to occur in 1-25% of patients with neurofibromatosis-I, and is the leading cause of cancer-related death in these patients. We report a case of multidisciplinary management of a giant malignant endothoracic nerve sheath tumor leading to histologically proven remission.

Nerve sheath myxoma is a rare benign lesion that has a strong predilection to the extremities. In addition to the extremities, nerve sheath myxoma had been reported in scalp, back, the neck, lateral chest wall, mandible and gingiva. Histologically it has a polylobated appearance and pale staining round nests of spindled and stellate cells separated by thin collagenous septa. The tumor cells sho...

Rhabdomyoblastic differentiation in a malignant peripheral nerve sheath tumor (MPNST) is termed malignant triton tumor (MTT), a rare neoplasm that poses a diagnostic dilemma in the differential diagnosis of neck masses and portends poor prognosis. We report a sporadic case of MTT of the neck in a 23-year-old female. We present the pathological findings. Immunohistochemistry confirmed the neurog...

OBJECTIVES This study focuses on the adjacent venous structures of tumors and their treatment in patients with second cervical (C2) dumbbell-shaped peripheral nerve sheath tumors (PNSTs). PATIENTS AND METHODS The authors retrospectively analyzed the clinical outcome and the venous structures involved with tumors in 16 patients with C2 dumbbell-shaped PNSTs treated surgically between 2008 and ...

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in children and adolescents but occur more frequently in NF1 patients. Angiosarcomatous differentiation in MPNSTs is a rare entity with poor prognosis. We report on a 13-year-old boy with intrathoracic angiosarcoma arising in MPNST associated with NF1.

Malignant peripheral nerve sheath tumor is a rare tumor occurring in 5%-10% of all malignant soft tissues sarcomas and triton tumor arising from neurofibromatosis type 1 (NF-1) is even rarer with associated high rate of mortality. No case of triton tumor has been reported in Nigeria to the best of our knowledge. We seek to report a case of lately detected retroperitoneal triton tumor presenting...

Zebrafish carrying heterozygous mutations for 17 different ribosomal protein (rp) genes are prone to developing malignant peripheral nerve sheath tumors (MPNSTs), a tumor type that is seldom seen in laboratory strains of zebrafish. Interestingly, the same rare tumor type arises in zebrafish that are homozygous for a loss-of-function point mutation in the tumor suppressor gene p53. For these rea...

This article reviews the typical and atypical locations, imaging findings, local recurrence, and metastatic pattern of malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are rare soft tissue sarcomas, commonly occur in extremities, and are often associated with neurofibromatosis. Distinction between benign and malignant tumors can be challenging on imaging. MPNSTs have a poor prognosis; ...