Peripheral nerve sheath tumours (PNST) with epithelial appearing cells compromise a heterogeneous group of neoplasms that are rare and diagnostically challenging. Of these, malignant PNSTs with epithelioid features (epithelioid MPNST) are commonly described in literature. However benign epithelioid PNSTs are rare and till date about 38 cases have been described in the literature. We report a be...

Malignant peripheral nerve sheath tumors (MPNST) are rarely encountered in the paranasal sinuses and frontal skull base. We present histopathological findings, cytometric DNA measurements, and the preliminary results of comparative genomic hybridization analysis of a 46-year-old male patient with a frontobasal MPNST. The tumor was resected via the endonasal approach. No tumor recurrence was det...

Malignant Peripheral Nerve Sheath Tumor (MPNST) is a rare soft tissue sarcoma that can occur in superficial locations. Histologically it is categorized into two variants: a conventional/spindled and an epithelioid variant. The latter one is very rare and can be confused histologically with malignant melanoma as it is diffusely positive for S100-protein. Herein we present a case that was initial...

A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the...

Neurofibromatosis type 1 (NF1) represents a major risk factor for development of malignancy, particularly malignant peripheral nerve sheath tumors (MPNST), optic gliomas, other gliomas, and leukemias. The oncologist will see NF1 patients referred for treatment of malignancy, and should be alert to the possibility of undiagnosed NF1 among patients with cancer. Brain tumors tend to have a more in...

Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and ...

Even though the diagnosis of schwannoma is usually straightforward in lesions with classic features, tumors with predominantly epithelioid morphology or marked myxoid change may present a diagnostic challenge. There are relatively few reported cases of epithelioid schwannoma entirely lacking classic morphology, and these have occurred in subcutaneous tissues, mucosal surfaces, or as gastrointes...

We present a case of a solitary neurofibroma involving the right posterior shoulder of a 69-year-old man with degeneration into a massive, malignant peripheral nerve sheath tumor measuring more than 3 times the average reported size. The radiographic, magnetic resonance imaging, and computed tomographic features are compared with the gross appearance and pathology.

Isolated nerve sheath tumors of the uterine cervix are very rare entities. This is especially true for benign nerve sheath tumors. We present, to the best of our knowledge, the first case of a benign nerve sheath tumor resected hysteroscopically. Our patient is a 69 year-old white female with a history of post menopausal bleeding. Initial workup included an endometrial biopsy and an ultrasound....