Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma with limited therapeutic options and a poor prognosis. Although neurofibromatosis type 1 (NF1) and radiation exposure have been identified as risk factors for MPNST, the genetic and molecular mechanisms underlying MPNST pathogenesis have only lately been roughly elucidated. Plexiform neurofibroma (PN) and atypical neurofibromatous neoplasm of unknown biological potential (ANNUBP) are novel concepts of MPNST precancerous lesions, which revealed sequential mutations in MPNST development. This review summarized the current understanding of MPNST and the latest consensus from its diagnosis to treatment, with highlights on molecular biomarkers and targeted therapies. Additionally, we discussed the current challenges and prospects for MPNST management.

Relationship between magnetic resonance imaging findings and histological grade in spinal peripheral nerve sheath tumors in dogs

“…12 In people, the only effective treatment for MPNST is complete surgical resection to obtain negative surgical margins; patients with negative margins have a relatively higher survival rate than those with positive margins. 13 Previous reports described the imaging findings and outcome of PNSTs in small cohorts of dogs. 14,15 Despite accurate spatial resolution, imaging techniques cannot definitively support a clinical diagnosis of PNST.…”

“…Given that MPNSTs commonly invade and breach the epineurium of the affected nerves, 10,11 a higher recurrence rate after surgical resection is expected in dogs because of incomplete resection 12 . In people, the only effective treatment for MPNST is complete surgical resection to obtain negative surgical margins; patients with negative margins have a relatively higher survival rate than those with positive margins 13 …”

Interdisziplinäre Behandlung von Raumforderungen in Assoziation zu peripheren Nerven: Tumore und tumorähnliche Läsionen

“…Die bei diesem Krankheitsbild auftretenden plexiformen Neurofibrome (PN) und atypische neurofibromatöse Neoplasien mit unklarem biologischem Potential (ANNUBP) sind primär benigne Läsionen ohne intrinsisches Metastasierungspotential und geringer Rezidivrate. Sie stellen jedoch potentielle Vorgängerläsionen eines MPNSTs mit entsprechenden Entartungspotential dar[41]. Dies erklärt das gehäufte und frühe Auftreten von MPNSTs in diesem Patientenkollektiv.An einen malignen Nerventumor sollte gedacht werden, bei Vorliegen von Ruheschmerz, fehlender Gewebeverschieblichkeit, zügiger Wachstumsdynamik, motorischem Defizit sowie bei größeren und in der Bildgebung infiltrativ imponierenden Tumoren[7].…”

Magnetic Resonance Imaging Features for Differentiating Low-Grade and High-Grade Malignant Peripheral Nerve Sheath Tumors

“…1 Approximately 50% of patients with MPNST had neurofibromatosis type 1 (NF1), 10% had a history of radiation exposure, and the rest were sporadic. 1 The lifetime risk of developing MPNST in NF1 patients is estimated to be 8% to 16%. 2 Malignant peripheral nerve sheath tumor can occur at any age and has no gender preference.…”

“…Malignant peripheral nerve sheath tumor (MPNST) is a relatively rare soft tissue sarcoma that accounts for 5% to 10% of soft tissue sarcomas 1 . Approximately 50% of patients with MPNST had neurofibromatosis type 1 (NF1), 10% had a history of radiation exposure, and the rest were sporadic 1 . The lifetime risk of developing MPNST in NF1 patients is estimated to be 8% to 16% 2 .…”