Intraosseous intraneural perineurioma derived from the inferior alveolar nerve with an abnormality of chromosome 22 and expression of the BCR-ABL fusion gene: report of a case and review of recent literature

Abstract

BackgroundPerineurioma (PN) is a peripheral nerve disease that primarily develops in the limbs and trunk and very rarely occurs in the oral cavity. PN is classified into two types: intraneural perineurioma (INPN) and soft tissue perineurioma (extraneural perineurioma, ENPN). In this article, we report a patient with mandibular body INPN derived from the perineurium of the inferior alveolar nerve.Case presentationThe patient was a 43-year-old male. He consulted our department for a detailed examination of the right mandibular body. A biopsy was performed at another hospital and he was diagnosed with a schwannoma. At his first visit, hypesthesia extending from the right lower lip to the mental region was recognized and enlargement of the right mandibular canal was confirmed with X-ray CT and MRI. Considering the possibility of future tumor growth, we extirpated the tumor under general anesthesia. Cystic tumor was seen continuously in the inferior alveolar nerve. Immunohistologically, the tumor cells were positive for Glut-1, weakly positive for EMA, and weakly positive for Claudin-1, and the histopathological diagnosis was INPN. In addition, absence of the BCR region of chromosome 22 and expression of the BCR-ABL fusion gene were observed by fluorescent in situ hybridization (FISH), and a chromosome 22 abnormality was confirmed. These findings indicated that the disease was a neoplastic lesion.ConclusionExpression of the BCR-ABL fusion gene in INPN that develops in the oral cavity is thought to be very rare, and to the best of our knowledge, ours is the first case to be reported in the literature. About three postoperative years have passed, but findings suggestive of recurrence have not been observed.

An Unusual Representation of an Odontogenic Cyst Engulfing the Inferior Alveolar Nerve

“…The sensory function of the IAN and the morphology of the MC have been reported to be affected by neural origin lesions such as intraosseous neurofibroma [ 17 ] and intraosseous intraneural perineurioma [ 8 ]. Conversely, IAN-derived schwannomas were reported to be asymptomatic; however, the borders of the MC were not detectable within the region of the lesion on the OPG [ 18 , 19 ], which was also mutual with our case.…”

“…Furthermore, mandibular intraosseous pathology that is associated with the inferior alveolar nerve (IAN) is infrequently seen; nevertheless, some cases have been reported. These may include developmental cysts [ 4 ], benign osteogenic tumors [ 5 ], benign neural-derived tumors such as schwannomas [ 6 , 7 ], and perineuriomas [ 8 , 9 ].…”