Malignant Peripheral Nerve Sheath Tumor of Non-Neurofibromatosis Type I Metastasized to the Cerebrospinal Axis

Abstract

A malignant peripheral nerve sheath tumor (MPNST) is a type of sarcoma that arises from peripheral nerves or cells of the associated nerve sheath. This tumor most commonly metastasizes to the lung and metastases to the spinal cord and brain are very rare. We describe a case of young patient with spinal cord and brain metastases resulting from MPNST. An 18-year-old man presented with a 6-month history of low back pain and radiating pain to his anterior thigh. Magnetic resonance imaging showed a paraspinal mass that extended from the central space of L2 to right psoas muscle through the right L2-3 foraminal space. The patient underwent surgery and the result of the histopathologic study was diagnostic for MPNST. Six months after surgery, follow-up images revealed multiple spinal cord and brain metastases. The patient was managed with chemotherapy, but died several months later. Despite complete surgical excision, the MPNST progressed rapidly and aggressively. Thus, patients with MPNST should be followed carefully to identify local recurrence or metastasis as early as possible.

Central nervous system metastasis of an intradural malignant peripheral nerve sheath tumor in a dog

“…MPNST central nervous system metastases and their features have not been reported in dogs. The radiological description of human MPNST spinal metastases from sporadic case reports is mainly intradural contrast-enhancing lesions (Thomas et al ., 2014) or multiple edematous infiltrations of the spinal cord in T2W images and multiple enhancing point lesions in T1W images (Park et al ., 2013). Another report of human MPNST central nervous system metastasis showed the appearance of extensive leptomeningeal enhancement (Baek et al ., 2005).…”

“…(2003) concluded that the involvement of proximal peripheral neural structures did not allow a total resection and lead to centripetal progression and infiltration of the spinal cord and brain stem. In humans, as in dogs, surgical excision with at least histologically clean margins is the goal of the treatment of MPNST; chemotherapy usefulness in humans is yet to be proven and is reserved for metastatic disease (Baehring et al ., 2003; Park et al ., 2013). A recent study suggests stereotactic radiotherapy as a promising alternative to surgical treatment in dogs, at least for proximal and nerve root location (Dolera et al ., 2016).…”

“…Sites of metastases included lungs, bone, pleura, retroperitoneum, diaphragm, lymph nodes, liver, chest wall, pericardium, thyroid gland, adrenal gland, leptomeninges, and brain. Spinal cord and brain metastases are reported to be very rare (Hruban et al ., 1990; Baehring et al ., 2003; Baek et al ., 2005; Xu et al ., 2012; Park et al ., 2013; Ren et al ., 2013; Thomas et al ., 2014). In cattle, a form of multifocal benign neurofibroma is described involving mainly the brachial plexus, intercostal nerve, and cardiac nerve.…”

Malignant peripheral nerve sheath tumor of thigh with sphenoid and brain metastases: Extremely rare occurrence with dismal prognosis despite significant response to palliative chemoradiotherapy

“…[7] Half the cases of MPNST are associated with NF-1, an autosomal dominant neurocutaneous disorder associated with poor prognosis, and a higher incidence of distant metastases compared to non-NF-1 cases. [3] Lung has been described as the most common site of distant metastases from MPNST[12345678910] followed by bone,[210] liver,[28] LNs,[8] pleura,[10] retroperitoneum,[10] and heart,[3] although this remains a topic of debate and further research. [10] Although bone metastases have been described often in the literature,[2610] the extensive research in Pubmed and Medline revealed no literature on biopsy-proven metastases to sphenoid from MPNST as reported by us.…”

Tumor targeted delivery of doxorubicin in malignant peripheral nerve sheath tumors

“…MPNST is a term coined to represent several tumors including malignant schwannoma, malignant neurilemmoma and neurofibrosarcoma, for the tumors of neurogenic origin. There are certain non NF1 MPNSTs which are equally as aggressive as NF1[ 2 ]. MPNSTs are an invasive form of soft tissue sarcoma which have a metastasizing property[ 3 – 6 ].…”