Malignant peripheral nerve sheath tumor: A rarity

Abstract

Malignant peripheral nerve sheath tumor (MPNST) of the mandible is an uncommon tumor that develops either from a preexisting neurofibroma or de novo. MPNSTs are sarcomas that originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells or from fibroblasts. Because MPNSTs can arise from multiple cell types, the overall appearance can vary greatly from one case to the next. A case of MPNST of the right side of the mandible in a 23-year-old female is reported.

Malignant peripheral nerve sheath tumour of the oral and maxillofacial region—A systematic review

“…Thirty articles fulfilled the eligibility criteria, and 20 articles were identified in the hand search process. A total of 50 studies reporting 57 cases of MPNST in the oral and maxillofacial region were included in this systematic review (Ali et al, 2011; Arruda et al, 2016; Bagan et al, 2005; Bavitz & Chewning, 1990; Can et al, 1999; Chao et al, 2007; Chao, Levine, et al, 2007; Che et al, 2006; Chitale & Dickersin, 1983; Colmenero et al, 1991; David et al, 1978; Devore & Waldron, 1961; DiCerbo et al, 1992; do Amaral et al, 2016; Fernandes et al, 2006; Gangothri et al, 2011; Grätz et al, 1991; Grevers & Röcken, 1995; Guglielmotti et al, 1987; Habal, 1997; Haisa et al, 1996; Hamakawa et al, 1998; Hammond & Calderwood, 1969; Hoffmann et al, 1988; Hujala et al, 1993; Imamura et al, 2003; James et al, 2003; Janardhanan et al, 2011; Kameyama et al, 1987; Kenali et al, 1999; Lee et al, 2019; Majumdar et al, 2016; Martinez Devesa et al, 2006; Muraki et al, 1999; Olivier, 2017; Oztürk & Tutkun, 2012; Paeng et al, 2006; Patel et al, 2015; Probst et al, 2018; Ramalingam et al, 2012; Raveh et al, 1995; Roy et al, 2017; Sempere‐Ortega & Martinez‐San‐Millan, 2008; Seyhan et al, 2012; Sham et al, 2010; Shotton et al, 1988; Victoria et al, 1999; Yaga et al, 2015; Yamazaki et al, 2005; Zakhary et al, 2011). A flowchart of the selection process of the studies is illustrated in Figure .…”

“…MPNST may arise sporadically in the setting of previous radiation, from pre‐existing neoplasms, including neurofibromas and neurilemmomas, or as part of neurofibromatosis type 1 (NF1) (Farid et al, 2014; Meyer, 2020). In general, sarcoma can be considered an MPNST if one of the following criteria can be established: (I) origin of the tumour from a peripheral nerve; (II) origin from a pre‐existing benign lesion of the nerve sheath; and (III) microscopically, the tumour reveals differentiation of Schwann cells (Janardhanan et al, 2011; Schaefer & Fletcher, 2015; Yaga et al, 2015).…”

Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

“…In summary, several questions remain unanswered, and some of them may ultimately converge on the exact identity of the cell of origin of MPNSTs. It is assumed that most MPNSTs originate from the Schwann cell lineage, although some may have a fibroblastic origin [23][24][25]. The origin of epithelioid MPNST is unclear.…”