Perineurioma (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.
The 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.
Expression 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.
Huguet P, De la Torre J, et al. Intraosseous intraneural perineurioma: report of a case with morphological, immunohistochemical and FISH study. Med Oral. 2004;9:64–8. PubMed
Vencio FE, Cheim PA Jr, et al. Perineurioma of the mandibular dental nerve: a case report and review of the literature. Oral Surg. 2009;2:103–7. CrossRef
Hata H, Yamazaki Y, et al. Perineurioma of the inferior alveolar nerve with expansion of the mandibular canal. J Oral Maxillofac Surg. 2011;57:419–23.
Lazarus SS, Trombetta LD. Ultrastructural identification of benign perineurial cell tumor. Cancer. 1978;41:1823–9. CrossRef
Damm D, White D, et al. Intraneural perineurioma—not restricted to major nerves. Oral Surg Oral Med Oral Radiol Endod. 2003;96:192–6. CrossRef
Da Cruz Perez DE, Amanajas de Aguiar FC Jr, et al. Intraneural perineurioma of the tongue: a case report. J Oral Maxillofac Surg. 2006;64:1140–2. CrossRef
Siponen M, Sandor GK, et al. Multiple orofacial intraneural perineuriomas in a patient with hemifacial hyperplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;104:e38–44. CrossRef
Boyanton BL, Jones JK, et al. Intraneural perineurioma. A systematic review with illustrative cases. Arch Pathol Lab Med. 2007;131:1382–92. PubMed
Dundr P, Povysil C, et al. Intraneural perineurioma of the oral mucosa. Br J Oral Maxillofac Surg. 2007;45:503–4. CrossRef
Kang J, Khang SK, Choi J, et al. Intraneural perineurioma in the tongue. Korean J Pathol. 2007;41:51–4.
Tanaka A, Alva PG, Miyazaki Y, et al. Intraneural perineurioma of the tongue: report of a case and review of the literature. Oral Med Pathol. 2009;13:71–4. CrossRef
Rocha LA, SMP L, ARDS S, Lopes MA, Vargas PA. Oral intraneural perineurioma: report of two cases. Clinics. 2009;64:1037–9. CrossRef
Hirano M, Muraki T, Nagayama M, et al. Intraneural perineurioma arising in the lateral border of the tongue. Pathol Int. 2013;63:619–21. CrossRef
Gomes da Silva W, Martinez M, et al. Oral perineurioma: clinicopathologic features from two cases and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol. 2017;123:e91–8. CrossRef
Emory TS, Scheithauer BW, Hirose T, Wood M, Onofrio BM, Jenkins RB. Intraneural perineurioma: a clonal neoplasm associated with abnormalities of chromosome 22. Am J Clin Pathol. 1995;103:696–704. CrossRef
Giannini C, Scheithauer BW, Jenkins RB, et al. Soft-tissue perineurioma. Evidence for an abnormality of chromosome 22, criteria for diagnosis, and review of the literature. Am J Surg Pathol. 1997;21:164–73. CrossRef
Barrett AW, Hopper C, et al. Intra-osseous soft tissue perineurioma of the inferior alveolar nerve. Oral Oncol. 2002;38:793–6. CrossRef
Noonan VL, Greene DJ, et al. Extraneural sclerosing perineurioma of the buccal mucosa: a case report and clinicopathologic review. Head Neck Pathol. 2010;4:169–73. CrossRef
Koutlas IG, Scheithauer BW, et al. Intraoral perineurioma, soft tissue type: report of five cases, including 3 intraosseous examples, and review of the literature. Head Neck Pathol. 2010;4:113–20. CrossRef
Koutlas IG, Scheithauer BW. On pseudo-onion bulb intraneural proliferations of the non-major nerves of the oral mucosa. Head Neck Pathol. 2013;7:334–43. CrossRef
Gong Z, Zheng L, et al. Role of complexity of variant Philadelphia chromosome in chronic myeloid leukemia in the era of tyrosine kinase inhibitor therapy. Ann Hematol. 2017;96:501–4. CrossRef
- 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
- BioMed Central
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