A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

Abstract

Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed.

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 .…”

Oral metastasis intraosseous mimicking periapical lesion: a case report

“…Although squamous cell carcinoma is the fifth-most common nonendodontic periapical lesion [11], intraosseous mesenchymal tumors mimicking periapical lesions have also been reported. Schwannoma in the mandibular alveolar bone [12] and malignant peripheral nerve sheath tumors in the maxilla [13], both mimicking an inflammatory periapical lesion, have also been reported.…”